The Unofficial Guide to Paediatrics is unique in being the only textbook to cover core subject summaries, examination practice, practical skills, career opportunities, OSCE practice and clinical cases all in one place. It does this through 1000+ colour images and illustrations, 200+ multiple-choice questions, and 60+ real-life clinical cases bringing paediatrics to life. The contributors range from professors of paediatrics to medical students, teachers, nurses and midwives, meaning the book has influences from a truly multidisciplinary team at all stages of training. This book has relevance beyond examinations for postgraduate education and as a day-to-day reference for professionals. Extensive collaboration with renowned academics and specialists ensures the content is reliable and based on up-to-date evidence. This book is relevant for: • Paediatric nurses
• Junior doctors
• Physician associates
• Healthcare visitors
• Paediatric trainees
• General practitioners
• Medical students
Reviews Professor Anthony Costello, Director, Department of Maternal, Newborn, Child and Adolescent Health, World Health Organisation. The layout and attention to detail in this unofficial guide is a tremendous asset for aspiring paediatricians as well as for those professionals interested in community child health.
Dr Andrew Long, Vice President (Education), RCPCH, Consultant Paediatrician and Associate Medical Director, Great Ormond Street Hospital The Unofficial Guide to Paediatrics is a wonderful compilation of a systems-based approach to a challenging subject matter, with something of interest for everyone. From the ‘sharp end’ of the complexities of adolescent medicine to the pragmatism of pursuing a career in paediatrics, this volume is beautifully illustrated and well referenced, and contains a wealth of knowledge and experience from a range of contributors who clearly appreciate what life is like on the frontline of paediatric practice.
Professor David Osrin, Professor of Global Health, UCL Institute for Global Health, UK, Honorary Consultant Paediatrician, Great Ormond Street Hospital, UK It’s always difficult to grasp what’s important when you’re approaching a subject like paediatrics. The Unofficial Guide to Paediatrics is smart because it tries to combine speciality-specific knowledge with the experience of practitioners at all levels and - most importantly - the duty of care and advocacy for children.
Dr Simon Broughton, Consultant Paediatrician, King’s College NHS Foundation Trust, UK Congratulations to Zeshan and his colleagues on producing The Unofficial Guide to Paediatrics. It is a huge piece of work by trainees and experts for anybody who has an interest in paediatrics, from medical students to established consultants and anybody interested in caring for children.
In the wonderful world of paediatrics, this is a wonderful resource for students, junior doctors, and paediatric trainees alike - or anyone looking for a simple and reliable complement to learning from the literature and clinical encounter. The development and success of this has been no child’s play - one might even say, it’s a milestone of an achievement!
Dr Dan Magnus, Consultant in Paediatric Emergency Medicine, Bristol Royal Hospital for Children, UK The first edition of The Unofficial Guide to Paediatrics is a delight. It is a new kind of paediatrics textbook for a new generation of students and professionals learning about child health and clinical paediatrics. Its quality will ensure its position alongside the very best resources available for supporting those trying to improve healthcare for children worldwide.
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Paed_cover_FINAL.indd All Pages
ISBN-13: 978-0957149953
9
780957
149953
Zeshan Qureshi
Beryl Lin, President, University of New South Wales Medical Society, Australia
The Unofficial Guide to PAEDIATRICS
The Unofficial Guide to Paediatrics
The Unofficial Guide to
PAEDIATRICS Zeshan Qureshi
P P P P
Core paediatric curriculum OSCE, clinical examination and practical skills 60+ clinical cases with 200+ MCQs to test yourself 1000+ high definition, colour clinical photographs and illustrations
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THE UNOFFICIAL GUIDE TO
PAEDIATRICS fIrst edItIon Zeshan Qureshi BM BSc (Hons) MSc MRCPCH
Academic Clinical Fellow, Great Ormond Street Hospital, UK and Institute of Global Health, UCL, UK Paediatric Registrar, London Deanery, UK
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ISBN 978-0957149953 Text, design and illustration © Zeshan Qureshi 2017 Edited by Zeshan Qureshi. Published by Zeshan Qureshi. First published 2017. All rights reserved; no part of this publication may be reproduced, stored in a retrieval system, transmitted in any form, or by any means, electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the publishers. Original design Anne Bonson-Johnson and Zeshan Qureshi. Page make-up by Amnet-systems. Illustrated by Anchorprint Group Limited. Clinical Photography Steven Kenny, Medical Photographer, Lewisham and Greenwich NHS Trust – 3.2 and 3.4. Modelling by Amy Moran, Angad Singh Kooner, Paarus Kaur Johal, Ishminder K Johal, Sorcha Mullen, Sion Santos, Annabelle Santos, Rachel Luke, Micah Ayos Mahinga, Marie Jasim, Ilyaas Jasim and Terouz Pasha. Medical illustrations Caitlin Monney, and Emily McDougall. 1.2: Fig 2-4, 1.3: Fig 1-6, 1.6: Fig 2, 4, 5, 7, 11, 12, 14, 15-7, Table 11, 1.7: Fig 1-2, 4-6, 1.8: Fig 13, 1.9: Fig 1-4, 1.10: Fig 1-2, 1:12: Fig 2, 1:14: Fig 4, 1.16: Fig 3-10, 14-26, Table 7, 1.17: Table 5, Fig 1-2, 1.18: Fig 5, 7. 1.21: Fig 1-4, 6-7. 1.22: Fig 2-6, 8. 1.23: Fig 1, 8. 1.23: Fig 2, 5, 1.25: Fig 1-3, 1.26: Fig 3, 5-9, 11, 3.2: Fig 5, 7-24, 26, 33, 40, 66, 73-4, 78, 86, 109, 110, 123, 126, 136-7, 153, 155, 157. 3.4: Fig 2, 34, 40, 74. Peter Gardiner. 1.3: Fig 7-11, 1.4: Fig 1-4. 1.5: Fig 1-5, 1.11: Fig 2, 4-6, 1.15: Fig 2-5, 22-25, 29, 31, 46, 1.18: Fig 1-3, 1.19: Table 1, 1.22: Fig 1. 3.2: Fig 6, 154 Clinical photographs Alex Rothman. 1.5: Fig 6, 1.6: Fig 10, 1.8: Fig 14, 1.9: Fig 5, 1.15: Fig 8, 1.16: Fig 26. Centers for Disease Control and Prevention Image Library. 1.8: Fig 1 – 2631, Fig 15 – 15408/15403, 1:15: Fig 20 – 2632, 32 – 15115. D@nderm. 1.11: Fig 1, 3, 1.15: Fig 8, 1.19: Fig 12. 1.21: Fig 5. 1.23: Fig 2-7, 9-14, 1.26: Fig 15. Auckland district health board – adhb.govt.nz. 1.15: Fig 6-15, 17-19, 21. NHS Fife. 3.2: Fig 72. John Offenbach. David Osrin review photo.
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X Rays Mark Rodrigues. 1.7: Fig 3, 1.18: Fig 9, 1.19: Fig 2-11, 13. 1.22: Fig 7, 10. Radiopedia. 1.15: Fig 37, 1.16: Fig 11-13, 3.4: Fig 56. A catalogue record for this book is available from the British Library. Zeshan Qureshi’s Acknowledgements I would like to thank my colleagues, mentors, friends, and most of all, my parents for their unwavering support through the years, without which none of this would have been possible. I have been inspired and trained by Paediatricians throughout the years, all of which cannot be named but particularly Ewen Johnston, Julie-Clare Becher, Jason Gane, Shahid Karim, Ella Aidoo, Susanna Sakonidou, Grant Marais, Tobias Hunt, Chris Harris, Simon Broughton, Kamal Ali, Terrence Stephenson, and Ildilko Schuller. Although we have tried to trace and contact copyright holders before publication, in some cases this may not have been possible. If contacted we will be pleased to rectify any errors or omissions at the earliest opportunity. Knowledge and best practice in this field are constantly changing. As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary. Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein. In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility. With respect to any drug or pharmaceutical products identified, readers are advised to check the most current information provided – i) on procedures featured or – ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications. It is the responsibility of practitioners, relying on their own experience and knowledge of their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions. To the fullest extent of the law, neither the publisher nor the authors, contributors, or editors, assume any liability for any injury and/or damage to persons or property that may occur as a result of any person acting or not acting based on information contained in this book. Printed and bound by Cambrian Printers in UK
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Introduction It has been a privilege to work with so many Paediatricians, and to serve as part of the big teams that deliver the excellent care that every child deserves. Whilst my career to date has involved challenging situations, I have invariably been able to unite with colleagues and parents around the fact that above anything else, the wellbeing of a child should not be compromised. It has been a privilege to work with so many Paediatricians, and to serve as part of the big teams that deliver the excellent care that every child deserves. Whilst my career to date has involved challenging situations, I have invariably been able to unite with colleagues and parents around the fact that above anything else, the wellbeing of a child should not be compromised. Editing this book, and working closely with my professional colleagues has really made me reflect on what the true definition of a Paediatrician might be. I’m on a Paediatric training programme, but I don’t think this is necessary to be a Paediatrician. I am privileged to say that I have passed the MRCPCH membership examinations, but again don’t think that is necessary to be a Paediatrician. I am now delighted to say that I’ve edited a Paediatrics textbook. But this doesn’t qualify me as a Paediatrician. So what is the core essence of this profession? Who can be a Paediatrician, in the true spirit of the word? And who should decide? In my humble opinion, it comes down to one simple litmus test. Can you do what is necessary, within the limitations of your knowledge, to be an advocate for a potentially sick child? Are you willing to try your utmost to communicate with a child and family to identify what their possible concerns are, and tease out any relevant pathology? If something goes wrong, or you are unhappy with something that is done regarding a child’s care, regardless of any contextual factors, will you speak up on behalf of the child? There is no substitute for clinical experience. Reading this book will inform you about Paediatrics. But to me, the most important thing in Paediatrics comes down to caring for the child, and when it comes down to this there should be no hierarchy: be tactful, use the appropriate channels, but never hesitate to speak up whenever you are worried that patient care is being compromised, regardless of who it might offend. Anyone can be a Paediatrician. A medical student on a Paediatric rotation; the student will often take the opportunity
to spend more time listening to the patient then any healthcare professional that day: and I’m always grateful when a student comes up to me relaying valuable patient concerns and diagnostic information: they are a Paediatrician. The primary care physician that follows a child from womb to adulthood: they are a Paediatrician. Their knowledge of the family and the
'...the most important thing in Paediatrics comes down to caring for the child, and when it comes down to this there should be no hierarchy' child throughout their life course is indispensable in identifying when things might go wrong in advance. The academics that improve the evidence on which care can be delivered: they are Paediatricians. The managers and policy makers that turn ideas into a reality: they are Paediatricians. And the Emergency Medicine doctor that sees a frightened parent and sick child for the first time, the ENT surgeon, the orthopaedic surgeon, the paediatric surgeon, the geneticist, the immunologist, the physiotherapist, the art therapist, the play specialist, the nurse, the dietician, the pharmacist, the social worker, the teacher, the police, every specialist, every person, every advocate that helps identify and address concerns and potential concerns to a child’s wellbeing: they are all Paediatricians. I am indebted to all their guidance and help in helping me provide care to children that I cannot fully provide on my own. It’s up to you to decide what a Paediatrician is. But in my humble opinion, you can all be a Paediatrician today.
Zeshan Qureshi Chief Editor Unofficial Guide to Paediatrics
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The Unofficial Guide to Medicine Project Additionally, we want you to get involved. This textbook has mainly been written by junior doctors and students just like you because we believe: ...that fresh graduates have a unique perspective on what works for students. We have tried to capture the insight of students and recent graduates to make the language we use to discuss this complex material more digestible for students. ...that texts are in constant need of being updated. Every student has the potential to contribute to the education of others by innovative ways of thinking and learning. This book is an open collaboration with you. You have the power to contribute something valuable to medicine; we welcome your suggestions and would love for you to get in touch.
Please get in touch and be part of the medical education project
[email protected] @DrZeshanQureshi Unofficial Guide to Medicine www.unofficialguidetomedicine.com
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Foreword Dr Simon Broughton PhD FRCPCH ■ Consultant Paediatrician, King’s College Hospital NHS Foundation Trust ■ Senior Lecturer, King’s College London – Course Director, MSc in Advanced Paediatrics ■ Training Programme Director
Congratulations to Zeshan and his colleagues on producing ‘The Unofficial Guide to Paediatrics’. It is a huge piece of work by trainees and experts for anybody who has an interest in paediatrics, from medical students to established consultants and anybody interested in caring for children. This book covers paediatrics in a traditional system based approach, but also has sections on the expanding speciality of adolescent healthcare, child health and the law, and public health. In addition, with sections on undergraduate and postgraduate assessments, starting out as a junior doctor and career sections, it provides useful advice to medical students and junior doctors wherever they are in their own career. So, why do we need another textbook on paediatrics? There are already plenty of excellent texts on this subject, however none have created a book like this. The inspiration behind this book is the working together of junior doctors, medical students, and experts to pull together a textbook that is accessible to all types of learners. We now live in a world where knowledge is so widely and freely available, that simply reprinting knowledge is becoming unnecessary. If knowledge is to be pulled together in a textbook, then every effort should be made to make that knowledge as relevant and accessible to the reader as possible, and that is what the Unofficial Guide to Paediatrics achieves. Every effort has been made to make this textbook as up to date as possible. However, inevitably, new research and guidance will be published. The genius behind this book however, is in empowering readers or users of this book to write to Zeshan with updates and suggestions for future editions. Being a Paediatrician is an absolute privilege, caring for children and young people and their families at very difficult times in their lives is an unbelievably rewarding challenge. One of the challenges that busy paediatricians struggle with, is keeping themselves up to date in all areas of paediatrics. The Unofficial Guide to Paediatrics will help with that, providing guidance to paediatricians of the future and assist in providing excellent care to children, young people and their families.
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Beryl Lin ■ President, University of New South Wales Medical Society ■ Co-chair, University of New South Wales Paediatrics Special Interest Group
Paediatrics is a ‘big’ topic about ‘little’ people. It is intellectually challenging and an exciting field for research and learning, but it can also be daunting when medical school is often set up to focus on adult medicine. Caring for children is different – both in a purely scientific sense, but also the way a sick child and their family should be approached, the dynamic of the hospital and multidisciplinary team, the ethical and sociocultural considerations involved, and even the career pathways that present are unique – all of which are covered in this book. The Unofficial Guide to Paediatrics features an easy-toread overview of paediatrics, broken down by systems. Each chapter describes core conditions by beginning with aetiology and clinical features, and progresses through investigations, differential diagnoses, management, complications, and finally prognosis. Furthermore, this book covers history taking, examination, communication, and practical skills – all supplemented with clinical cases, labelled diagrams, and information about common examinations and assessment criteria. The authors have also provided illustrations of common procedures and medical devices in clinical practice. As a book produced and written by trainees for other trainees, it captures key information in a digestible manner. With extensive collaboration from renowned academics and specialists, the content is reliable and based on up-to-date evidence. This textbook is part of an international medical education project, which embodies a passion for peer teaching, and the empowerment of young people who are making a positive impact. Congratulations to Zeshan’s team for this awardwinning series of textbooks that will help others in their medical journey. In the wonderful world of paediatrics, this is a wonderful resource for students, junior doctors, and paediatric trainees alike - or anyone looking for a simple and reliable complement to learning from the literature and clinical encounter. The development and success of this has been no child’s play - one might even say, it’s a milestone of an achievement!
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03
Abbreviations 4C compound 4-carbon compound 5C compound 5-carbon compound 17OHP 17-Hydroxyprogesterone A Airway ABG Arterial blood gas ABPA Allergic bronchopulmonary aspergillosis ACE Angiotensin-converting enzyme ACE Antegrade colonic enema Acetyl CoA Acetyl coenzyme A Ach Acetylcholine ACTH Adrenocorticotropic hormone ADA Adenosine deaminase ADEM Acute disseminated encephalomyelitis ADH Antidiuretic hormone ADHD Attention Deficit Hyperactivity Disorder AED Anti-epileptic drugs AFP Alpha fetoprotein AI Adrenal insufficiency AIDS Acquired immunodeficiency syndrome AKI Acute kidney inury ALL Acute lymphoblastic leukaemia ALP Alkaline phosphatase AMH Anti-Mullerian hormone AML Acute myeloid leukaemia ANA Antinuclear antibody ANCA Antineutrophil cytoplasmic antibody AP Anteroposterior APLS Advanced Paediatric Life Support APS1 Autoimmune polyendocrine syndrome 1 APTT Activated partial thromboplastin time ART Antiretroviral therapy AS Aortic stenosis ASD Atrial septal defect ASD Autism spectrum disorder ASOT Antistreptolysin O antibody titres ATG Anti-thymocyte globulin ATP Adenosine triphosphate AV Arteriovenous AVP Arginine vasopressin AVPU Alert, voice, pain, unresponsive AVSD Atrioventricular septal defect B Breathing BC Blood culture BCG Bacillus Calmette-Guérin BiPAP Bi-level Positive Airway Pressure BMA Bone marrow aspirate BMD Becker’s muscular dystrophy BMI Body mass index BNF British National Formulary BNFc British National Formulary for Children BP Blood pressure BPM Beats per minute BTS British Thoracic Society BVM Bag-valve mask BXO Balanitis xerotica obliterans C Circulation CAFCASS Children and Family Court Advisory and Support Service CAH Congenital adrenal hyperplasia CAKUT Congeital anomalies of the kidney and urinary tract CAMHS Child and Adolescent Mental Health Service
CA-MRSA Community-associated methicillin resistant Staphylococcus aureus CBA Collagen binding activity CBD Case-based discussion CBS Cystathionine beta synthase CBT Cognitive behavioural therapy CCAM Congenital cystic adenomatoid malformation CD Crohn’s disease CDC Centers for Disease Control and Prevention CDH Congenital dislocation of the hip CDGP Constitutional delay of growth and puberty CEX Clinical evaluation exercise CF Cystic fibrosis CFAM Cerebral function analysing monitor CFTR Cystic fibrosis transmembrane conductance regulator CFRD Cystic fibrosis related diabetes CGD Chronic granulomatous disease CGH Comparative genomic hybridization CHARGE syndrome Coloboma of the eye, heart defect, atresia of the choanae, retarded growth and/or development, genital and/ or urinary abnormality, ear abnormality and deafness CHD Congenital heart defect CHL Conductive hearing loss CK Creatine kinase CKD Chronic kidney disease CLL Chronic lymphoblastic leukaemia CMP Cow’s milk protein CMV Cytomegalovirus CMV Controlled mandatory ventilation CNS Central nervous system Carbon dioxide CO2 CP Cerebral palsy CPAP Continuous positive airway pressure CPR Cardiopulmonary resuscitation CRH Corticotropin releasing hormone CRP C-reactive protein CSF Cerebrospinal fluid CT Computed tomography CTG Cardiotocography CVID Common variable immunodeficiency CVL Central venous line CVS Chorionic villous sampling CXR Chest X-ray CYP450 Cytochrome P450 enzyme D Disability DAT Direct antigen test DDAVP Desmopressin DDH Developmental dysplasia of the hip DEJ Dermo-epidermal junction DEXA Dual energy X-ray absorptiometry DI Diabetes insipidus DIC Disseminated intravascular coagulation DIDMOAD Diabetes insipidus, diabetes mellitus, optic atrophy and deafness DIOS Distal intestinal obstruction syndrome
DHEAS Dehydroepiandrosterone sulphate DHT Dihydrotesterone DKA Diabetic ketoacidosis DM Diabetes mellitus DMARD Disease-modifying antirheumatic drug DMD Duchenne muscular dystrophy DMSA Dimercaptosuccinic acid DNA Deoxyribonucleic acid DOPA Dihydroxyphenylalanine DOPS Direct observation of procedural skills DRESS Drug Reaction (or Rash) with Eosinophilia and Systemic Symptoms DSD Disorder of sexual development dsDNA Double stranded DNA DVT Deep Vein Thrombosis E Exposure EBV Epstein-Barr virus ECG Electrocardiogram ECMO Extracorporeal membrane oxygenation ED Emergency department EDH Extradural haemorrhage EEG Electroencephalogram EHEC Enterohaemorrhagic Escherichia coli EIEC Enteroinvasive Escherichia coli EMA Endomysial antibody EMG Electromyography ENaC Epithelial sodium channel ENT Ear, nose and throat EO Eosinophilic oesophagitis EPEC Enteropathogenic Escherichia coli EPLS European Paediatric Life Support ERT Enzyme replacement therapy ESR Erythrocyte sedimentation rate ESWL Extracorporeal shock wave lithotripsy ET Endotracheal ETC Electron transport chain ETEC Enterotoxigenic Escherichia coli EVD Extraventricular drain FAH Fumarylacetoacetase FBC Full blood count FEV1 Forced expiratory volume in one second FFP Fresh frozen plasma FGFR Fibroblast growth factor receptor FiO2 Fraction of inspired oxygen FISH Fluorescence in situ hybridisation fMRI Functional magnetic resonance imaging FODMAP Fermentable Oligo-Di-Monosaccharides and Polyols FPG Fasting plasma glucose FSH Follicle stimulating hormone FUO Fever of unknown origin FVC Forced vital capacity G6PD Glucose-6-phosphate dehydrogenase GA1 Glutaric aciduria type 1 GABA Gamma-aminobutyric acid GAD Glutamic acid decarboxylase GAGs Glycosaminoglycans GALT Gut-associated lymphoid tissue GALT Galactose-1-phosphate uridyl transferase GARS-2 Gilliam Autism Rating Scale
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GAVI Global Alliance for Vaccines and Immunisation GBM Glomerular basement membrane GBS Group B streptococcus GBS Guillain-Barré syndrome GCS Glasgow coma scale GFR Glomerular filtration rate GH Growth hormone GHD Growth hormone deficiency GLUT-1 Glucose transporter 1 GOR Gastro-oesophageal reflux GORD Gastro-oesophageal reflux disease GP General practitioner GnRH Gonadotropin-releasing hormone GSDs Glycogen storage disorders GvHD Graft-versus-host disease HAART Highly active antiretroviral therapy Hb Haemoglobin HbA1c Glycated haemoglobin HbS Sickle cell haemoglobin HcG Human chorionic gonadotropin HD Hodgkin’s disease HDU High dependency unit HFNC High flow nasal cannulae HFOV High-frequency oscillatory ventilation HH Hypogonadotropic hypogonadism HiB Haemophilus influenza type B HIE Hypoxic-ischaemic encephalopathy HIV Human Immunodeficiency Virus HLA Human leucocyte antigen HLH Haemophagocytic lymphohistiocytosis HPA Hypothalamic-pituitary-adrenal HPV Human papilloma virus HPG Hypothalamic-pituitarygonadal HR Heart rate HRCT High resolution computed tomography HS Hereditary spherocytosis HSCT Haemopoietic stem cell transplantation HSP Henoch Schönlein Purpura HSV Herpes simplex virus HUS Haemolytic uraemic syndrome HVA Homovanillic acid IBD Inflammatory bowel disease IBS Irritable bowel syndrome IC Indeterminate colitis ICD Implantable cardioverter defibrillator ICP Intracranial pressure ICU Intensive care unit Ig Immunoglobulin IgA Immunoglobulin A IGF1 Insulin-like growth factor 1 IGF2 Insulin-like growth factor 2 IL-2 Interleukin-2 IL-4 Interleukin-4 IL-5 Interleukin-5 IL-13 Interleukin-13 ILAE International League Against Epilepsy IIH Idiopathic Intracranial hypertension IM Intramuscular INR International normalised ratio IO Intraosseous IPS Infantile pyloric stenosis IQ Intelligence quotient IRT Immunoreactive trypsinogen ISS Idiopathic short stature ITP Immune thrombocytopenia
ITP Idiopathic thrombocytopenic purpura IUD Intrauterine device IUGR Intrauterine growth restriction IUS Intrauterine system IV Intravenous IVA Isovaleric acidaemia IVH Intraventricular haemorrhage IVIG Intravenous immunoglobulin JIA Juvenile idiopathic arthritis JVP Jugular venous pressure KDOQI Kidney Disease Outcomes Quality Initiative KUB Kidney-ureter-bladder LARC Long acting reversible contraception LCH Langerhans cell histiocytosis LDH Lactate dehydrogenase LFT Liver function test LH Luteinising hormone LP Lumbar puncture LTOT Long term oxygen therapy MAG3 Mercaptoacetyltriglycine MAP Mean arterial pressure MAP Mean airway pressure MCAD Medium-chain acyl-CoA dehydrogenase MCADD Medium-chain acyl-CoA dehydrogenase deficiency MC&S Microscopy, culture and sensitivity MCUG Micturating cystourethrogram MCV Mean cell volume MCV Molluscum contagiosum virus MDGs Millennium Development Goals MDI Metered-dose inhaler MDR Multi-drug resistant MDT Multidisciplinary team MEN Multiple endocrine neoplasia MenC Meningococcus C MG Myasthenia gravis MIBG Metaiodobenzylguanidine MIS Müllerian-inhibiting substance MMA Methylmalonic acidaemia MMR Measles, mumps and rubella MODY Maturity onset diabetes of the young MPS Mucopolysaccharidoses MRCPCH Membership of the Royal College of Paediatrics and Child Health MRI Magnetic resonance imaging MRSA Methicillin resistant Staphylococcus aureus MSUD Maple syrup urine disease NAD Nicotinamide adenine dinucleotide NAI Non-accidental injury NBM Nil by mouth NEC Necrotising enterocolitis NF Neurofibromatosis NF1/2 Neurofibromatosis type 1 or 2 NG Nasogastric NHL Non-Hodgkin lymphoma NICE National Institute for Health and Care Excellence NICU Neonatal intensive care unit NIPE Newborn and infant physical examination NIPPV Non-Invasive Positive Pressure Ventilation NKF National Kidney Foundation NLS Newborn life support NMDA N-methyl-D-aspartate NPA Nasopharyngeal aspirate NSAIDS Non-steroidal antiinflammatory drugs NSPCC National Society for the Prevention of Cruelty to Children NTD Neural tube defect O2 Oxygen OA Osteoarthritis OAE Oto-acoustic emissions
OGTT Oral glucose tolerance test OME Otitis media with effusion OP Oropharyngeal ORS Oral rehydration solution OSCE Objective structured clinical examination OT Occupational therapy PA Propionic acidaemia PALS Patient advice and liaison service PANDAS Paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections PAPP-A Pregnancy-associated plasma protein-A PCD Primary ciliary dyskinesia PCDAI Paediatric Crohn’s Disease Activity Index PCP Pneumocystis carinii (jirovecii) pneumonia PCR Polymerase chain reaction PCV Packed cell volume PDA Patent ductus arteriosus PE Pulmonary Embolism PEEP Positive end expiratory pressure PEF Peak expiratory flow PEG Percutaneous endoscopic gastrostomy PEG-J Percutaneous endoscopic gastro-jejunostomy PET Positron emission tomography PG Plasma glucose PHEX Phosphate regulating endopeptidase homolog PICC Peripherally inserted central catheter PICU Paediatric intensive care unit PIP Peak inspiratory pressure PKU Phenylketonuria PMTCT Prevention of mother-to-child transmission PNET Primitive neuroectodermal tumour PORCN Porcupine homolog PP Precocious puberty PPHN Persistent pulmonary hypertension of the newborn PPV Patent processus vaginalis PR Per rectum PRO Parental Responsibility Order PROM Premature rupture of the membranes PRN Pro re nata i.e. as required PS Pulmonary stenosis PT Prothrombin time PTV Patient triggered ventilation PTH Parathyroid hormone PT Physiotherapy PTSD Post-traumatic stress disorder PUCAI Paediatric Ulcerative Colitis Activity Index PUJ Pelvi-ureteric junction PUV Posterior urethral valve PVL Periventricular leukomalacia PWS Prader-Willi syndrome RBC Red blood cell REM Rapid eye movement ReSoMal Rehydration solution for malnutrition RF Rheumatoid factor RiCOF Ristocetin cofactor RIF Right iliac fossa RNA Ribonucleic acid RR Respiratory rate RSV Respiratory syncytial virus RUTF Ready-to-use therapeutic food SAH Subarachnoid haemorrhage SALT Speech and language therapy SBAR Situation, background, assessment and recommendation SBR Serum bilirubin SC Sickle cell anaemia
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03 SCAD Short-chain acyl-CoA dehydrogenase SCBU Special care baby unit SCID Severe combined immunodeficiency SD Standard deviation SDGs Sustainable Development Goals SDH Subdural haemorrhage SEDU Specialist eating disorders unit SENCO Special Educational Needs Coordinator SGA Small for gestational age SHOX Short Stature Homeobox gene SIADH Syndrome of inappropriate antidiuretic hormone secretion SIDS Sudden infant death syndrome SIGN Scottish Intercollegiate Guidelines Network SIMV Synchronised intermittent mandatory ventilation SLE Systemic lupus erythematosus SMA Spinal muscular atrophy SNHL Sensorineural hearing loss SPECT Single-photon emission computed tomography SpO2 Oxygen saturation SROM Spontaneous rupture of membranes SRS Social Responsiveness Scale SSPE Subacute sclerosing panencephalitis SSRI Selective serotonin reuptake inhibitor STEC Shiga Toxin-Producing E. coli SUDEP Sudden unexpected death in epilepsy SUDI Sudden unexpected death in infancy
SUFE Slipped upper femoral epiphysis SVCO Superior vena cava obstruction SVD Spontaneous vaginal delivery SVT Supraventricular tachycardia SWAN Syndrome without a name SWS Sturge-Weber syndrome T1DM Type 1 diabetes mellitus T2DM Type 2 diabetes mellitus Te Expiratory Time Ti Inspiratory Time TAC Team around the child TAPVD Total anomalous pulmonary venous drainage TB Tuberculosis TCA Tricarboxylic acid TDS Ter die sumendum ie. Three times a day TFT Thyroid function test Tg Thyroglobulin tTGA Tissue transglutaminase antibody Th2 Type 2 Helper T-Cell Thal Thalassaemia TIA Transient ischaemic attack TIBC Total iron binding capacity TLS Tumour lysis syndrome TNF Tumour necrosis factor TORCH Toxoplasmosis, Other, Rubella, CMV, Herpes TPO Thyroperoxidase TPMT Thiopurine methyltransferase TPN Total parenteral nutrition TRH Thyrotropin-releasing hormone TSH Thyroid-stimulating hormone TST Tuberculin skin test tTG Tissue transglutaminase TTN Transient tachypnoea of the newborn
UAC Umbilical artery catheter UC Ulcerative colitis UDT Undescended testes U&E Urea and electrolytes UO Ureteric orifice UN United Nations URTI Upper respiratory tract infection USS Ultrasound scan UTI Urinary tract infection UV Ultraviolet UVC Umbilical vein catheter VACTERL Vertebral defects, anal association atresia, cardiac defects, tracheo-oesophageal fistula, renal anomalies and limb abnormalities VHL Von Hippel-Lindau disease VLCAD Very-long-chain acyl-CoA dehydrogenase VMA Vanillylmandelic acid VMAT Vesicular monoamine transporter VP Ventriculoperitoneal VSD Ventricular septal defect VT Ventricular tachycardia VTEC Verotoxin producing Escherichia coli VUJ Vesicoureteric junction VUR Vesico-ureteric reflux VZV Varicella zoster virus WAS Wiskott-Aldrich syndrome vWD von Willebrand disease vWF von Willebrand factor WCC White cell count WHO World Health Organisation
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Contributors Chief Editor
Associate Editor
Zeshan Qureshi BM MSc BSc (Hons) MRCPCH Academic Clinical Fellow, Great Ormond Street Hospital, and Institute of Global Health, UCL, UK
Tina Sajjanhar MBBS DRCOG DCH FRCPCH FCEM Consultant in Paediatric Emergency Medicine, Divisional Director for Children and Young People Services, Lewisham and Greenwich NHS Trust, UK
John Jungpa Park MB ChB MTh BMedSci (Hons) Junior Doctor, North Central Thames Foundation School, UK
Maxine Wilkie Medical Student, Keele University, UK
Chi Hau Tan MBBS (Hons) GDipSurgAnat Neurosurgery Registrar, Monash Health, Melbourne, Australia
Anna Capsomidis BSc MBChB (Hons) MRCPCH PGDip (Med Ed) PGC (Healthcare Ethics and Law) Clinical Research Fellow, UCL, Great Ormond Street Institute of Child Health, UK
Anand Goomany MbChB BSc Core Surgical Trainee, Bradford Teaching Hospitals Foundation Trust, West Yorkshire, UK
May Bisharat MBBS (Hons) MSc FRCS (Paed Surg) Registrar in Paediatric Surgery, Evelina Children’s Hospital, London, UK
Amy Mitchell MBChB BSc (Hons) MSc DipClinEd MRCPCH Consultant Paediatric Oncologist, Southampton University Hospital Trust, Southampton, UK
Rachael Mitchell MRCPCH MA (Cantab) Paediatric Registrar, London Deanery, London, UK
David K K Ho MBChB DTM&H MRCPCH Clinical Research Fellow, Institute of Child Health, University College London, UK
Alexander Young MBChB MSc MRCS PGCME Trauma and Orthopaedic Surgery Registrar, Severn Deanery, UK
Christopher Harris MBChB MRCPCH Paediatric Neonatal Registrar, King’s College Hospital, London, UK
Marie Monaghan MBBS BSc (Hons) MRCPCH Paediatric Registrar, London Deanery, UK
Marylyn-Jane Emedo MBBS BSc MRCPCH Paediatric Registrar, London Deanery, London, UK
Antonia Hargadon-Lowe BMBS BMedSci MRCPCH MSc Paediatric Registrar, London Deanery, London, UK
Authors
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03 Sadhanandham Punniyakodi MBBS MRCPCH MSc Senior Training Fellow in Paediatric Endocrinology, The Great North Children’s Hospital, Royal Victoria Infirmary, Newcastle upon Tyne
Stephen D Marks MD MSc MRCP DCH FRCPCH Consultant Paediatric Nephrologist, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Hannah Linford BMBS MRCPCH Paediatric Registrar, KSS Deanery, UK
Anita Demetriou MBBS BSc MRCPCH DTMH Paediatric Registrar, London Deanery, UK
Christopher Grime MBChB MRCPCH Paediatric Registrar, London Deanery, UK.
Debasree Das MBBS BSc MRCPCH Paediatric Registrar, London Deanery, UK
Philippa King BSc MBChB MRCPCH MSc Academic Clinical Fellow, Medical Microbiology, East of England Deanery, UK
Isabel Mawson MBBS BSc MRCPCH Paediatric Registrar, London Deanery, UK
Claire Bryant BMedSc BMBS Junior Doctor, South Thames Foundation School, London, UK
Anna Chadwick MBBS BSc MRCPCH Paediatric Registrar, London Deanery, UK
Andrew Hall MBChB MRCS DOHNS ENT Specialist Registrar, Great Ormond Street Hospital, UK
Amy Moran MBBS MRCPCH Paediatric Registrar, London Deanery, UK
Michael Malley MA (Hons) Cantab MBBS MRCPCH Paediatric Registrar, London Deanery, UK
Alice Armitage MBBS BSc MRCPCH Academic Clinical Fellow, Paediatrics, London Deanery, UK
Vaitsa Tziaferi MD MRCPCH MSc Consultant in Paediatric Endocrinology & Diabetes, Leicester Royal Infirmary, UK
Kunal Babla BSc(Hons) MBBS MSc MRCPCH MAcadMEd Neonatal Registrar, London Deanery, UK
Maanasa Polubothu BSc MBChB MSc MRCPCH PGDip (Genomic Medicine) Academic Clinicial Fellow, London Deanery, UK
Sam Thenabadu MBBS MRCP DRCOG DCH MA Clin Ed FCEM MSc (Paed) FHEA Consultant Adult & Paediatric Emergency Medicine. Honorary Senior Lecturer, King’s College London
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Stephanie Connaire Final Year Medical Student, Cardiff University, Cardiff, UK
Zainab Kazmi BSc (Hons) MBChB Academic Foundation Doctor and Honorary Clinical Fellow, University of Glasgow
Pooja Parekh MBBS Paediatric Trainee, London Deanery, UK
Expert Reviewers Dr Vandy Bharadwaj Consultant Paediatric Haematologist, Southampton Children’s Hospital, Southampton, UK Prof. Robert Tulloh MA DM FRCP FRCPCH Consultant Paediatric Cardiologist, Bristol Royal Hospital for Children, UK. Honorary Professor of Congenital Cardiology and Pulmonary Hypertension, University of Bristol Prof. David Walker Professor of Paediatric Oncology, Children’s Brain Tumour Research Centre, Nottingham, UK Mr RA Wheeler MS FRCS LLB (Hons) LLM Consultant Neonatal & Paediatric Surgeon. Director, Department of Clinical Law. University Hospital of Southampton, UK. Mr Theo Joseph FRCS ENT Consultant, Royal National Throat Nose and Ear Hospital, London, UK Dr Victoria Jones MRCPCH Consultant Paediatrician, North Middlesex University Hospital, UK Dr Anne-Marie Ebdon MBBS MRCPCH FRACP Consultant Paediatrician, Queen Mary’s Hospital for Children, Epson & St.Hellier University Hospitals NHS Trust, UK Dr Sarah K Clegg MBChB BSc MRCPUK MRCPCH Consultant Paediatrician, Department of Community Child Health, Edinburgh, UK Dr Solomon Kamal-Uddin MBBS BMedSci Paediatric Registrar, London Deanery, UK Dr Delan Devakumar MBCHb MSc MRCPCH DTM&H MFPH PhD Public Health Registrar, London Deanery, UK Dr Khadija H Aljefri MBChB MRCP (UK) MRCP (Derm) Dermatology Registrar, Royal Victoria Infirmary, Newcastle upon Tyne, UK
Mr Neil Tickner MRPharmS PGDip. Clin. Pharm Lead Pharmacist Paediatrics, St Mary’s Hospital, London, UK Stephanie Connaire Final Year Medical Student, Cardiff University, Cardiff, UK Dr Pooja Parekh MBBS Paediatric Trainee, London Deanery, UK Bianca Davis School Teacher, Head of Personal Social and Health Education, London, UK Dr Sreena Das MB ChB MRCPCH Consultant Paediatrician, King’s College Hospital, London, UK Dr Simon Chapman BA BM FRCPCH Consultant Paediatrician, King’s College Hospital, London, UK Dr Isabel Farmer MBBS MRCPCH Haematology Registrar, Queen Elizabeth Hospital, London, UK Tabatha English BSc (Hons) RM Midwife, Lewisham Hospital, London, UK Kelly Frogbrook PgDip RN Paediatric Nurse, Queen Elizabeth Hospital, London, UK Dan Purnell Lead Resuscitation Officer, Lewisham and Greenwich Healthcare NHS Trust Dr Daniel Langer BSc (Hons) MRCPCH DPID Consultant in Acute & Ambulatory Paediatrics. Special interest in paediatric infectious diseases (SPIN ID) and global child health Epsom & St Helier NHS Trust, UK Lydia Shackshaft Medical Student, King’s College London, UK Sammie Mak Medical Student, Leeds University, UK Ben Evans Medical Student, University of Newcastle, UK
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03
Contents SECTION 1: CORE TOPICS 1
CHAPTER 1 ADOLESCENT MEDICINE ALICE ARMITAGE
7
HAPTER 2 C ASSESSMENT AND MANAGEMENT OF THE ACUTELY UNWELL CHILD CHRISTOPHER HARRIS
18 C HAPTER 3 CARDIOLOGY HANNAH LINFORD
35 CHAPTER 4 COMMUNITY PAEDIATRICS ANTONIA HARGADON-LOWE
50 C HAPTER 5 ENT ANDREW HALL
60 CHAPTER 6 ENDOCRINOLOGY ESHAN QURESHI, VAITSA TZIAFERI, SADHANANDHAM Z PUNNIYAKODI, MARYLYN-JANE EMEDO AND CLAIRE BRYANT
92 CHAPTER 7 GASTROENTEROLOGY MAXINE WILKIE, POOJA PAREKH, AND ZESHAN QURESHI
113 CHAPTER 8 GENETICS ISABEL MAWSON, CHRISTOPHER HARRIS, MAXINE WILKIE AND ZESHAN QURESHI
130 CHAPTER 9 HAEMATOLOGY AMY MITCHELL AND ANNA CAPSOMIDIS
141 CHAPTER 10 IMMUNOLOGY AND ALLERGY MAANASA POLUBOTHU
149 CHAPTER 11 INFECTION PHILIPPA KING, ZESHAN QURESHI AND DAVID K K HO
177 CHAPTER 14 METABOLIC MEDICINE ESHAN QURESHI, STEPHANIE CONNAIRE AND Z ZAINAB KAZMI
186 CHAPTER 15 NEONATOLOGY CHRISTOPHER HARRIS AND ZESHAN QURESHI
212 CHAPTER 16 NEUROLOGY J OHN JUNGPA PARK, ZESHAN QURESHI AND DEBASREE DAS
245 CHAPTER 17 NUTRITION CHI HAU TAN
259 CHAPTER 18 ONCOLOGY ANNA CAPSOMIDIS AND AMY MITCHELL
270 CHAPTER 19 ORTHOPAEDIC AND RHEUMATOLOGICAL DISORDERS ANAND GOOMANY AND ALEXANDER YOUNG
292 CHAPTER 20 PUBLIC HEALTH CHRISTOPHER HARRIS
297 CHAPTER 21 RENAL MEDICINE ESHAN QURESHI, RACHAEL MITCHELL Z AND STEPHEN D MARKS
313 CHAPTER 22 RESPIRATORY MEDICINE CHRISTOPHER GRIME
330 CHAPTER 23 SKIN CONDITIONS MAANASA POLUBOTHU
342 CHAPTER 24 SURGERY MAY BISHARAT
166 CHAPTER 12 INTENSIVE CARE KUNAL BABLA AND SAM THENABADU
173 CHAPTER 13 CHILDREN AND THE LAW ZESHAN QURESHI
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SECTION 2: CLINICAL CASES 372 CHAPTER 1 CLINICAL CASES: STANDARD
679 INDEX
ALL AUTHORS
438 CHAPTER 2 CLINICAL CASES: INTERMEDIATE ALL AUTHORS
487 CHAPTER 3 CLINICAL CASES: DIFFICULT ALL AUTHORS
SECTION 3: CLINICAL SKILLS 507 CHAPTER 1 HISTORY TAKING ESHAN QURESHI, CHRISTOPHER HARRIS AND Z MICHAEL MALLEY
526 CHAPTER 2 EXAMINATION AMY MORAN AND ZESHAN QURESHI
604 CHAPTER 3 COMMUNICATION ANNA CHADWICK AND MICHAEL MALLEY
613 C HAPTER 4 PRACTICAL SKILLS ICHAEL MALLEY, ZESHAN QURESHI, M ANITA DEMETRIOU AND MARIE MONAGHAN
648 CHAPTER 5 PRESCRIBING
MICHAEL MALLEY AND MARIE MONAGHAN
SECTION 4: BECOMING A PAEDIATRICIAN 658 CHAPTER 1 UNDERGRADUATE AND POSTGRADUATE ASSESSMENTS IN PAEDIATRICS MICHAEL MALLEY AND MARIE MONAGHAN
665 CHAPTER 2 A GUIDE TO BEING A JUNIOR DOCTOR IN PAEDIATRICS MARIE MONAGHAN AND MICHAEL MALLEY
674 CHAPTER 3 CAREERS IN PAEDIATRICS MARIE MONAGHAN AND MICHAEL MALLEY
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1.01
ADOLESCENT MEDICINE ALICE ARMITAGE
APPROACH TO THE ADOLESCENT CONSULTATION
CONTENTS 1 Approach to the Adolescent Consultation 1 2 2 2
Adolescent History Taking Asking Difficult Questions Puberty, Growth and Development Autonomy, Consent and Confidentiality
3 Relationships, Sexual Health and Contraception
3 Sexual Health 3 Contraception 3 Teenage Pregnancy
3 Mental Health
3 Depression 4 Self-harm 4 Suicide Risk 4 Psychosis 4 Drug and Alcohol Abuse 4 Medically Unexplained Symptoms 5 Management of Mental Health Problems in Adolescence
5 Safeguarding in Adolescence
5 Bullying 5 Sexual Exploitation and Assault
6 Transition from Paediatric to Adult Services
6 References and Further Reading
A
dolescence is the transitional phase of growth and development between childhood and adulthood. An adolescent is defined by the World Health Organisation as a person between 10 and 19 years of age. There is increasing recognition of the specific problems of this age group, including trauma, mental health issues, pregnancy and sexually transmitted diseases. The rise in healthcare usage in adolescence is multifactorial; increasing survival from chronic childhood conditions, use of drugs and alcohol, and risk-taking behaviour all play a role, and advances in perinatal care and immunisation have shifted the burden of disease away from the under-fives. Understanding the unique needs of this age group is a core skill for any physician. The law relating to children and adolescents is covered in Chapter 1.13.
Adolescent History Taking It is important to adapt the approach to the needs of the adolescent age-group. At the start of a consultation, consider the following: ӹӹ Always speak to the patient, not to their parent or carer, unless there is no alternative. ӹӹ Ask the patient if they would like to speak to alone or ask them if they would like someone to be present, such as a parent or friend. ӹӹ For any examination, offer a chaperone. ӹӹ Talk in an age-appropriate fashion. It is easy to alienate an adolescent patient by appearing patronising or by using medical jargon. ӹӹ Try to anticipate issues around consent and confidentiality. The psychosocial history is vital to the adolescent history. Many presentations will stem from an issue like drug use, a fight with a partner or worries about sexuality. A useful aide-memoire is the HEADSS tool, shown in Table 1. In general, start with more open questions and then focus questions to the information given. A common reason for missing important issues is making assumptions; for example, thinking that all young people live at home with their parents or that all young people are heterosexual.
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TABLE 1: Key aspects of adolescent history taking (HEADSS tool) Domain
Example Questions
Importance
Home and environment.
• • • • •
Young people may be living with family, in foster care or living independently. Relationships with people they live with are likely to have a major impact on their health and well-being. Someone to confide in is vital to emotional well-being.
Education and employment.
• Are you at school or college at the moment? • Do you have a job? How is that going? • What are your plans for when you finish school?
A young person may be struggling in education because of problems with their mental or physical health or problems such as bullying or domestic violence at home. Young people may need support to re-engage with education or to start applying for jobs.
Activities.
• • • •
Young people may be obese or have nutritional problems linked to their activities. There may be mental health problems, bullying or involvement in gangs.
Drugs and alcohol.
• Do you drink alcohol? How often do you drink? • Do you or any friends take drugs? What kind? • Have you ever regretted something you have done after taking drugs or alcohol?
There are links with risk-taking behaviour, isolation and damage to relationships. Addiction may indicate a need for psychological input.
Sexual health.
• Do you have a boyfriend or girlfriend? • How old were you when you first had sex? • Have you ever had or thought about having a partner of the same sex? • Have you ever been for a sexual health screen?
Giving contraceptive and sexual health screening advice is important. There may be concerns about sexual exploitation or another abusive relationship. Mental health problems can arise from worrying about gender identity or sexuality.
Suicide and depression.
• Most of the time do you feel down or depressed? • Have you ever done anything to hurt yourself like cutting your arms or taking pills? • Do you ever think about hurting yourself?
Young people may have mental health problems and need psychological interventions. It is important to assess suicide risk.
How are things at home? Has anything changed recently? Who are you living with at the moment? What is your relationship like with your foster parents? Who would you talk to if you’re worried about something?
What kind of thing do you enjoy doing? Do you play any sports or have hobbies? Are you in a group or a gang? How much time do you spend playing computer games or on social media?
Asking Difficult Questions Some of these questions can feel difficult to ask directly, and are even more difficult to answer directly. One useful tip is to use scenarios as a way into difficult questions. For example: ӹӹ “It’s quite common for young people to experiment with drugs; is that something you’ve had experience with?” ӹӹ “Some of the work I do with young people is about choosing types of contraception and talking about sexual health; has anyone ever talked to you about this?” This is also a good way to address more specific concerns that may have arisen during the consultation. For example: ӹӹ “Some young people I work with have told me that their parents fight a lot and sometimes it gets violent; is that something that happens in your home?” ӹӹ “One thing that can happen in a relationship is feeling pressured into acting or behaving in a certain way; sometimes people feel they don’t have any choice about it. Have you ever felt like that?” Questions need to ensure the young person will feel supported but not accused. For example, with the drug question, a young person may not admit to usage if they believe the police will immediately be called. The more comfortable the child, the more likely they are to give an honest response. When
questioning, the doctor must remain aware of nonverbal language, e.g. changes in body language when talking about home (breaking eye contact, fidgeting and shorter responses).
Puberty, Growth and Development Any consultation with an adolescent patient should include assessment of height, weight and pubertal status. This assessment is often forgotten, particularly in older teenagers transitioning to adult care. Young people with chronic health needs may have delayed growth and puberty compared with their peers. This is also a good opportunity to highlight problems such as obesity, eating disorders or neglect.
Autonomy, Consent and Confidentiality One of the challenges of working with adolescents is managing their emerging autonomy as they move into adulthood. The ability to develop and maintain trust and rapport with patients relies on sensitive handling of these complex issues. Remember: ӹӹ Each adolescent should be assessed on an individual basis without prejudice. ӹӹ There is no lower age limit to Gillick competence, and even younger children may be able to consent for their own medical care.
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Mental Health
RELATIONSHIPS, SEXUAL HEALTH AND CONTRACEPTION
All these forms are over 99% effective, and normal fertility returns as soon as they are removed. However, unless sex is with a regular partner and both have had recent sexual health screening, it is important to recommend additional barrier methods of contraception, like condoms. Doctors in the UK and elsewhere are legally allowed to provide contraception to females under 16 without parental consent, providing certain conditions are met (p174).
Adolescent Medicine
ӹӹ There is no lower age limit on confidentiality but there are limits to this confidentiality, such as if there is a concern that the patient is in danger. If there is a duty to break confidentiality, tell the patient beforehand, except in rare, exceptional circumstances where this may result in significantly more harm than good.
Teenage Pregnancy
Before bringing up sex and relationships, give patients an opportunity to talk alone and reassure them about confidentiality. If a young person is having sex or considering having sex, it is important to talk to them about healthy relationships, sexual health and contraception. As with drugs and alcohol, some of this may have been covered at school. Do not make assumptions based on age, culture, disability or diagnosis. If a patient is not sexually active, they may well wish to be and have questions about this. Chronic health conditions and disabilities impact on sex and relationships; therefore, doctors are potentially in a position to address these issues.
Globally, over 10% of births are to girls 15 to 19-years-old. Although the majority of these are in low and middle-income countries, both the UK and the USA continue to have high rates of teenage pregnancy. This remains a significant cause of morbidity and mortality in this age-group, particularly in younger adolescents (13 to 15-years-old) who experience higher rates of pregnancy complications and pre-term births. Risk factors for teenage pregnancy include: ӹӹ Low socioeconomic status. ӹӹ Low level of educational attainment. ӹӹ Having been a baby of a teenage parent.
Sexual Health
Any young person who becomes pregnant should be offered counselling covering abortion and adoption to allow them to make an informed choice. Remember that for some young people, particularly in certain ethnic groups, having a baby as a teenager can be a positive choice. An important part of antenatal care for younger patients is a focus on health promotion, including reducing drug and alcohol intake and optimising nutritional status. It is important to involve social care and consider the needs of both the mother and the unborn child.
Globally, young people have disproportionately high rates of sexually transmitted infections (STIs), with chlamydia, gonorrhoea, viral warts and syphilis being particularly common. The best protection is through use of condoms; these are often freely given out in health care settings and should be discussed with all young people even if on another form of contraception. Some STIs can by asymptomatic, particularly in women, so regular sexual health screening should be recommended for anyone who is sexually active. Walk-in services allow for easy, anonymous access to sexual health advice and testing. Similarly, the sending of results of STI testing by text messaging is convenient and helps preserve anonymity.
Contraception In addition to condoms, forms of long-acting reversible contraception (LARC) are a good option for adolescent patients, and providing leaflets and counselling will allow them to choose the method that they feel is most appropriate for them. Some examples of LARCs include: ӹӹ Intrauterine device (IUD). This is also called a coil; this can stay in place for five to ten years but can be removed at any time. ӹӹ Intra-uterine system (IUS). This coil releases a small amount of progestogen locally. It can stay in place for five years. ӹӹ Contraceptive injection. This lasts for eight or twelve weeks; it delivers systemic progestogen. ӹӹ Contraceptive implant. This sits under the skin and releases a small amount of systemic progestogen. It can stay in place for up to three years.
MENTAL HEALTH Half of all mental illnesses begin before 14-years-old, and 75% begin before 24-years-old. Young people suspected or known to have mental health problems should be formally assessed by a child and adolescent mental health service (CAMHS). CAMHS teams involve a range of professionals including psychiatrists, psychologists, family therapists, social workers, counsellors and nurses. Some common mental health problems are discussed below.
Depression The three core symptoms of depression are low mood, low energy levels and loss of interest in activities that were previously pleasurable (anhedonia). Other symptoms are shown in Table 2. Approximately five percent of teenagers suffer from depression at some point. Most young people will go through periods of feeling “down” or anxious. However, depression is longer lasting and interferes with the patient’s ability to function.
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TABLE 2: Symptoms of depression Core
Biological
Symptoms
Symptoms
Low mood. Low energy levels. Loss of interest.
Sleep disturbance. Reduced appetite. Weight loss or weight gain. Constipation. Loss of libido.
Cognitive Symptoms Worthlessness/low selfesteem. Poor memory. Poor concentration. Guilt. Tearfulness. Agitation.
In addition to the symptoms listed above, depression in adolescents may present with symptoms such as: ӹӹ Extreme sensitivity to criticism. ӹӹ Irritability and anger. ӹӹ Worsening performance at school. ӹӹ Unexplained aches and pains. In young people, the signs of depression may be: taking drugs, going missing or getting involved in fights. A presentation of depression can be secondary to another problem; examples in adolescence are bullying, undisclosed sexual assault or maltreatment. Management of depression in adolescent therapy involves initially identifying possible precipitants (e.g. bullying) and addressing them where possible. Cognitive behavioural psychotherapies are used more commonly than with adults. However, in moderate to severe depression, doctors may choose to prescribe medication, e.g. a selective serotonin reuptake inhibitor (SSRI). Fluoxetine is the preferred choice in adolescents, although it may be associated with an increased risk of suicide. Both approaches can be used simultaneously.
ӹӹ ӹӹ ӹӹ ӹӹ
Have you ever tried to hurt yourself? Have you had thoughts about wanting to kill yourself? Have you ever tried to kill yourself? Have you ever made plans to kill yourself (for example, collecting tablets or writing a note)?
Any concerns about patient safety need to be escalated following child protection procedures, ideally with an assessment by someone trained in child and adolescent mental health.
Psychosis The definition of psychosis is when a person “loses touch with reality” and may be characterised by: ӹӹ Hallucinations. When a person sees, hears or otherwise perceives things that are not present; for example, hearing voices. ӹӹ Delusions. When a person holds a belief that is untrue despite logical evidence to the contrary; for example, believing that their parent is trying to kill them. Psychosis is a symptom of several conditions, including schizophrenia, bipolar disorder, autoimmune disease and meningoencephalitis. Schizophrenia has a prevalence of 1% in middle to late adolescence. Many more people will have at least one psychotic episode in their lives and the first episode of psychosis commonly occurs in adolescence or in the early 20s. Psychosis is an extremely distressing experience for patients and their families. Despite antipsychotic medications, psychotic illness such as schizophrenia continues to have a poor prognosis, with multiple relapses and high rates of suicide.
Self-harm
Drug and Alcohol Abuse
Common forms of self-harm in adolescence include: ӹӹ Cutting the arms or legs with a sharp object. ӹӹ Taking an overdose of medications (commonly paracetamol). ӹӹ Alcohol or illicit drug intoxication.
Many young people experiment with drugs and alcohol and, for some, this can become an addiction. Warning signs include: ӹӹ Change in behaviour. ӹӹ Hanging out with a new group of friends. ӹӹ Deterioration in academic performance. ӹӹ Getting involved in fights or shoplifting.
Self-harm is linked with attempted suicide, but this is not always the case. Depression is a common comorbidity. In assessing and managing these children, perform a risk assessment and explore the intent of suicide. Then explore the circumstances leading to the self-harm episode. Bear in mind that even those who self-harm frequently may have different reasons for each episode. Young people who present to hospital with acute self-harm will need to be formally assessed by the mental health team and will have a clear follow-up plan in place on discharge.
Suicide Risk Any patient with depression or presenting following self-harm should have their suicide risk assessed. Never be afraid to ask directly about suicide. Questions to ask include:
Beyond the direct health effects of drugs and alcohol, these substances can isolate young people from their friends and family and increase risk-taking behaviour. These problems can be easily missed, as young people will often try to hide drug and alcohol use.
Medically Unexplained Symptoms Adolescent patients may present with symptoms such as pain, tiredness or dizziness, for which, despite investigations, no medical cause is evident. This is also called “somatisation disorder”. These symptoms are more common in women, patients with depression, those who have recently had a
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Safeguarding in Adolescence
Management of Mental Health Problems in Adolescence As with adults with mental health problems, adolescent patients can be managed in an inpatient or outpatient setting depending on their diagnosis and needs. In younger patients, the focus is more on treating the family unit (e.g. through family therapy), and medication is used less frequently. The two broad categories are talking therapies and medication.
Talking Therapies ӹӹ Counselling. This gives young people with mental health problems an opportunity to talk about their problems oneto-one with an empathetic listener. This is useful for young people with most types of mental health or behavioural problems. ӹӹ Family therapy. This may be helpful for behavioural problems or addiction, where difficulties or conflicts may exist within the family as a whole. It may help family members to see each other’s perspectives and be honest with each other. ӹӹ Cognitive behavioural therapy (CBT). This may be helpful for many mental health problems including depression, anxiety and psychosis. CBT teaches techniques for overcoming or controlling thoughts or behaviours. ӹӹ Psychotherapy. This seeks to address the root causes of thoughts and behaviours by talking about the past; for example, childhood experiences.
Medication ӹӹ Antidepressants. These can be used with other therapies to treat depression and anxiety symptoms. Various types are available but commonly used drugs include selective serotonin reuptake inhibitors (SSRIs) such as fluoxetine and sertraline. Side effects are common and include dry mouth, tiredness and headache. ӹӹ Antipsychotics. These are used to treat psychosis and can have benefit as mood stabilisers as well. Examples include haloperidol, risperidone and clozapine. Side effects include autonomic effects, movement disorders and weight gain.
ӹӹ Stimulants. These are helpful for controlling symptoms of Attention Deficit Hyperactivity Disorder (ADHD) and can reduce inattention and impulsiveness. The most common example is methylphenidate (Ritalin).
Adolescent Medicine
significant medical problem or those who have experienced a bereavement. This is a difficult diagnosis to make as it is a diagnosis of exclusion, making these patients very challenging to manage. Rarely, the patient or their carer may be knowingly fabricating symptoms (this is known as factitious or fabricated illness). If the motivation is a reward for feigning or exaggerating illness, such as financial benefit or attention, this is specifically known as malingering. However, the majority are not “faking it”, and if they feel judged or disbelieved, their condition is likely to worsen rather than improve. The best approach is for a single consultant to coordinate the patient’s care, working closely with the primary care physician and offering psychological support.
SAFEGUARDING IN ADOLESCENCE The adolescent age group presents its own safeguarding challenges. Overlap may exist between victims and perpetrators; for example, a teenage parent who is on a child protection plan and is neglecting her own child.
Bullying A National Society for the Prevention of Cruelty to Children (NSPCC) study found that almost half of all children have been bullied at some point in their lives. As such, bullying represents a major cause of maltreatment in the adolescent group. It may involve physical assault but can also include verbal, non-verbal, exclusion, racial and sexual bullying. It may also take place online or via text messaging. All adolescent patients should be asked about cyberbullying, social networking sites and sexual bullying, which is an increasing problem. Children who are bullied may show a range of symptoms and, at worst, may present to health services following physical assault or self-harm. It should be part of the differential diagnosis in any adolescent presentation, particularly with mental health problems and self-harm.
Sexual Exploitation and Assault Sexual exploitation remains a largely hidden problem worldwide. It can manifest as physical, sexual, emotional and financial abuse or as coercive control. It may occur within young people’s relationships and may be influenced by hierarchies within and external to any relationships. Typically, those in a position of power coerce a young person into sexual activities. Both boys and girls are at risk and many young people are unaware that abuse is taking place. One in four girls and one in ten boys are likely to experience sexual abuse in the UK. Notably, the figures for boys may be underestimated as some evidence indicates that many do not seek help, feeling too ashamed to admit victimhood. Signs that a young person may be being exploited include: ӹӹ Relationships with older men. ӹӹ Behavioural changes (including sexual promiscuity). ӹӹ Going missing. ӹӹ Self-harm. In the UK, over one-third of all presentations of rape and sexual assault are in the adolescent age bracket. Globally, up to one-third of girls report their first sexual experience as being forced. Fear of being judged or not believed may prevent young people from seeking help. Sexual assault by partners or relatives is significantly less likely to be reported.
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Any young person who presents after sexual assault should be referred to a sexual assault referral centre. These are one-stop specialist medical and forensic services where a patient can receive acute medical and emotional support, have forensic samples taken if necessary, and be referred on for counselling or tests. After assault, patients have high rates of psychological morbidity, particularly post-traumatic stress disorder (PTSD).
TRANSITION FROM PAEDIATRIC TO ADULT SERVICES Transitional care relates to children and young people with or without chronic disorders being transferred to adult services at a certain age. This process is a challenge in the healthcare of young people. Variable policies and health systems structures often leave patients “in limbo” between services and unsure of where to turn for help. Further challenges are presented by the increasing numbers of patients surviving into adulthood with what were previously considered paediatric conditions, such as congenital heart disease and metabolic conditions. Good practice in transitional care includes: ӹӹ Introducing the concept of transition early (for example, at 13 or 14-years-old). ӹӹ An individualised age of transition (depending on the needs of the patient, their disease control and level of maturity). ӹӹ A transitions coordinator or keyworker (who remains in contact with the patient throughout transition). ӹӹ A written transition plan individualised to each patient. ӹӹ “Transition clinics” with the paediatric and adult team together, so that a more formal handover of the patient occurs. ӹӹ Access to staff with training in the needs of adolescents and young adults.
REFERENCES AND FURTHER READING 1 Cohen E et al. HEADSS, a psychosocial risk assessment instrument: Implications for designing effective intervention programs for runaway youth. J Adolesc Health, 1999; 12: 539-44. 2 Dick B, Ferguson JB. Health for the world’s adolescents: A second chance in the second decade. J Adolesc Health. 2015; 56:3-6. 3 Ford T, Goodman R, Meltzer H. The British child and adolescent mental health survey 1999: the prevalence of DSM-IV disorders. J Amer Acad Child Adolesc Psychiatry. 2003; 42:1203-11. 4 Cawson P et al. Child maltreatment in the United Kingdom: a study of the prevalence of abuse and neglect. London: NSPCC. 2000. 5 Berelowitz S et al. I thought I was the only one. The only one in the world. The Office of the Children’s Commissioner’s Inquiry into Child Sexual Exploitation in Gangs and Groups: Interim report. 2012. 6 Viner R. Transition from paediatric to adult care. Bridging the gaps or passing the buck? Arch Dis Child. 1999; 81:271-5. 7 Crowley R et al. Improving the transition between paediatric and adult healthcare: a systematic review. Arch Dis Child 2011: archdischild202473. 8 NICE CG16. Self-harm: The short-term physical and psychological management and secondary prevention of self-harm in primary and secondary care. 1994. https:// www.nice.org.uk/guidance/cg16. 9 Courvoisie H, Labellarte MJ, Riddle MA. Psychosis in children: diagnosis and treatment. Dialogues Clin Neurosci. 2001; 3:79-92.
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1.02
ASSESSMENT AND MANAGEMENT OF THE ACUTELY UNWELL CHILD CHRISTOPHER HARRIS
INTRODUCTION
CONTENTS 7 Introduction
8 Rapid Assessment 8 History 8 Examination
10 Initial Management
10 Resuscitating a Patient Using the DR ABCDE Approach 10 Advanced Paediatric Life Support 13 Other Important Considerations during Resuscitation
13 Key Investigations
13 Blood Glucose 13 Blood Gas 14 Venous Blood Samples 14 Radiological Imaging
14 Trauma 15 Ongoing Care
15 The Child Requiring Admission 15 Paediatric Intensive Care 15 Sending a Patient Home 15 Care in the Community 15 Specialist Follow Up
15 The Death of an Infant or Child
16 Sudden Unexpected Death in Infancy 16 Debriefing
17 References and Further Reading
C
linicians are fearful of treating children and infants in the Emergency Department (ED), and, more generally, of making medical decisions concerning children. Junior doctors manage children in many settings (Box 1).
Box 1: Settings in which unwell children may be encountered • Primary Care. • Emergency Department. • Paediatric Emergency Department. • Paediatric wards. • Adult specialties with paediatric cover (surgery, orthopaedics, ENT and dermatology).
This chapter provides a framework for assessing any child that presents for medical attention to ensure they receive timely and effective care, with a particular focus on dealing with the very sick child. When approaching a potentially sick child, bear in mind that children are not just small adults. Different challenges and techniques must be addressed to get to the bottom of the problem quickly. Consider the following: ӹӹ The parents or carer may not have the whole story if the child has been in the care of someone else, and so a collateral history may be necessary. ӹӹ The parents’ “sixth sense” regarding their child should always be taken seriously, but equally, parents may underestimate the seriousness of their child’s condition. ӹӹ Younger children may not be able to give any history, or the history given may be misleading. ӹӹ All children, but particularly younger infants, may have underlying congenital abnormalities that have remained undetected until presentation in extremis.
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Assessment and Management of the Acutely Unwell Child
ӹӹ Examination interpretation depends on the cooperation of the child, and this can lead to signs being missed or misinterpreted. ӹӹ Children can compensate physiologically for severe illness, meaning that signs of deterioration may not be evident until late in the illness. ӹӹ Children are dependent on adult carers for many things, particularly in early life. This can lead to opportunities for abuse, which can be easily missed if not assessed for specifically. ӹӹ Decisions made during childhood with regards to health can impact all areas of development.
RAPID ASSESSMENT The initial approach should begin with a comprehensive review of the child’s ABC: Airway, Breathing and Circulation. This should be done on all children and can be done during the introduction to the patient and their family. If a baby is crying with no stridor or a child is able to say their name, it can be assumed that their airway is patent. Clues on respiratory status are evident from an assessment of how comfortable the child is, whether they can speak in sentences, and their colour. A child who is running around the waiting room and interested in playing with all the toys and equipment will probably have little in the way of circulatory issues. This initial screen can be useful in deciding whether the child needs immediate resuscitation or whether there is time to get a more detailed history before deciding on a management plan. If there are any signs of compromise to A, B or C, these should be addressed immediately, and help should be sought. “Red flag” signs that might be identified are summarised in Table 1.
Parents or a chaperone should be present during examinations. It is common for physical signs, such as non-blanching rashes, to appear on the background of a fairly benign history, and these signs may develop quickly; for example, a non-blanching rash appearing in the groin since the parents last changed the baby.
General Observing the general condition of a child is vital. A child running around in the waiting area is unlikely to require emergency intervention, but a child who is lethargic and not interested in their surroundings may be seriously unwell. The interaction of the child, the hygiene status and the appropriateness of the clothing can yield important information. Any medical devices or equipment may give clues to underlying conditions and therapy compliance. For example, an inhaler may suggest asthma, but if it is being carried without a spacer device, it may highlight concerns regarding inhaler technique.
Respiratory Inspect the chest wall and nares for signs of respiratory distress and cyanosis. Listen for stridor or a barking cough. Palpate the trachea to ensure it is central and not displaced like in pneumothorax. Percuss the chest wall, particularly at the lung bases, to identify consolidation or effusion in the older child (>5-years-old). Auscultate for wheeze or added sounds, indicating obstruction or consolidation.
Cardiovascular
A focused history is an important part of the assessment of a sick child. However, when a child is very sick, the emotional distress of the parents can make this difficult. If a child requires immediate medical intervention, this should be addressed while another member of the medical team establishes the history from the family member. Key questions are summarised in Table 2.
Inspect for signs of congenital cardiac disease, such as clubbing of the fingers, shortness of breath or cyanosis. In the older child, it may be possible to see a raised jugular venous pressure (JVP). Palpate the apex beat and the femoral pulses. Auscultate the heart: some heart murmurs may be undetectable at birth and may only be heard at 2 to 4-weeks-old when the left ventricular pressure becomes greater, allowing flow across some heart lesions. Palpate the abdomen for an enlarged liver and auscultate the lung fields. Measure the blood pressure and compare it to age-related normal values.
Examination
Abdominal
An assessment of airway, breathing and circulation should be performed in any encounter. Following this assessment, if the child is stable, a full examination of the child should be done. Often, the system responsible for illness is not clear because of an uncertain history or the global effect of serious illness. Attention to red flag signs is important, as well as a screening of all systems. Do not cut short an examination due to fears of making a child cry. This is particularly true of babies and young children, who must be examined fully, looking at the entire body, whilst still making efforts to protect the child’s modesty.
Inspect the size of the abdomen and any visible peristalsis. Palpate the whole abdomen for tenderness and masses. Palpate and percuss for abdominal organomegaly and ascites. Listen for bowel sounds. Also note “tinkling” sounds in obstruction or absent sounds in perforation. The acute abdomen presents with a child reluctant to move or with a distended abdomen tender to palpation, with possible rebound tenderness. Remember to look at the face of the child when palpating the abdomen to see if it is tender. Distracting questions may help to discern between voluntary and involuntary guarding.
History
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RAPID ASSESSMENT
ASSESSMENT AND MANAGEMENT OF THE ACUTELY UNWELL CHILD
TABLE 1: Red flag signs needing urgent intervention Red Flag
Possible Cause
Assessment
Management
Non-blanching rash.
• • • •
• Assess the rash, and areas affected. • Full blood count (FBC), blood film, coagulation, blood cultures, CRP, meningococcal PCR.
• Urgent antibiotics if possible meningococcal sepsis. • Fluid resuscitation.
Traumatic injury.
• Internal bleeding. • Head injury.
• Full trauma assessment regardless of how well the child looks.
• Contact trauma team and immobilise the cervical spine.
Soot around the mouth or nose.
• Smoke inhalation.
• Chest X-ray (CXR). • Oxygen saturations. • Carbon monoxide levels.
• Consider securing the airway early.
Major burns.
• House fire. • Accident. • Chemical exposure.
• Airway assessment. • Calculate percentage of surface area affected. • Intravascular fluid volume. • Circumferential burns.
• Consider securing the airway. • Release circumferential burns (to remove constriction effect) if respiration compromised. • Fluid resuscitation. • IV antibiotics. • Discussion with local burns centre and the paediatric intensive care unit (PICU).
Faltering growth.
• Underlying medical abnormality. • Child protection issues.
• Careful examination and social history.
• Close observation due to risk of deterioration.
Unexplained bruising or bleeding.
• Leukaemia. • Child protection issues. • Thrombocytopaenia.
• Careful examination and social history. • FBC, coagulation and blood film.
• Stop massive bleeding. • Ideally take blood samples pre transfusion of products.
Sudden onset coughing, wheezing or stridor.
• Foreign body inhalation (even if not directly observed).
• Unilateral polyphonic wheeze. • CXR may be normal, consider inspiratory/expiratory films.
• • • •
Delayed central capillary refill time (>2secs).
• • • •
• Identify underlying cause, e.g. swollen lips/urticaria. • Temperature. • Inflammatory markers.
• 20ml/kg 0.9% saline bolus (10ml/kg boluses in trauma, diabetic ketoacidosis (DKA), neonates, raised intracranial pressure, cardiac conditions).
Tachycardia.
• Sepsis. • Shock. • Supraventricular tachycardia (SVT).
• Identify underlying cause. • ECG.
• Vagal manoeuvres. • Adenosine. • Electrocardioversion or chemical cardioversion may be required if SVT causing deterioration.
Bradycardia.
• Pre-terminal event. • Heart block.
• Ensure pulse present. • Identify underlying cause. • ECG.
• CPR if no pulse. • Discuss with cardiologist urgently if heart block.
Meningococcal sepsis. Leukaemia. Henoch-Schönlein purpura. Thrombocytopaenia.
Septic shock. Hypovolaemic shock. Cardiogenic shock. Anaphylactic shock.
Keep child calm. Oxygen as required. Examination by ENT specialist. Consider PICU transfer if needed.
TABLE 2: Key questions in a focused history Question
Explanation
What happened?
Mechanism of injury. Cause of deterioration.
How long has your child been unwell?
Acute or rapid worsening versus more gradual decline.
Any illnesses in the past or PICU/NICU admissions?
Underlying conditions, such as asthma, diabetes, allergy, cardiac conditions or metabolic conditions.
Any medications?
Potential overdose, missed medication (e.g. diabetes and insulin), allergies (e.g. new antibiotics).
Anyone unwell in the family?
Potential infectious cause of illness, potential medication available for accidental ingestion. (Continued)
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Assessment and Management of the Acutely Unwell Child
TABLE 2: (Continued) Question
Explanation
Any family history of illness?
Sudden deaths in the family (long QT syndrome), atopy (asthma), systemic lupus erythematosus (SLE) (heart block in infants born of mothers with anti-Rho antibodies).
Any foreign travel in the last year?
Helps to widen or narrow the differential diagnosis and subsequent investigations, e.g. malaria, Ebola.
Any discussions regarding resuscitation or end of life care?
It is common for children with life-limiting conditions to be rushed into hospital in the final stages as parents get worried or find they are unable to cope with events unfolding. They may want support at end-of-life rather than immediate life-prolonging measures. Examples include terminal cancer, severe genetic conditions such as spinomuscular atrophy, and heart disease not amenable to surgical correction.
If time permits: • Immunisations up to date? • Who was caring for the child when they became unwell? • Any other witnesses to the event? • Other services involved, e.g. police, social services? • Any other casualties and are they being cared for? • Are there any other children in the house and are they being cared for? • Can I call someone to support you?
These questions build a picture of the overall health of the family and provide more information to piece together what has happened. It is important to recognise how difficult it can be for the parents or other members of the public to see and manage an unwell child until help arrives. This is an opportunity to safeguard others involved in an accident or those forgotten at home in the rush to get to hospital.
Neurological and Developmental In an emergency, the AVPU scale can be helpful in quickly assessing neurological status. A paediatric GCS will give more information if time allows. Examine the power, tone and reflexes. Cranial nerves should also be examined. New focal neurological signs are an important indicator that further imaging is warranted. Assess if the child responds appropriately and has reached their gross motor, fine motor, social and language developmental milestones.
INITIAL MANAGEMENT The initial management of a child identified as peri-arrest or very unwell shares a common pathway, regardless of aetiology. Perform life-saving management prior to initiating treatment of the underlying condition. Although meningococcal sepsis requires antibiotic therapy, if the child is not breathing and has no circulation, antibiotics will have little effect.
Resuscitating a Patient Using the DR ABCDE Approach Once you have identified that a child might be seriously unwell, ensure it is safe to approach them for assessment. In other words, assess for danger (D). The next thing to do is to assess if they are responsive (R). This can be done by asking them a question, followed by gentle shaking if there is no response. A sternal rub can be used if there is still a doubt. The next step is to assess the airway (A), breathing (B), circulation (C), disability (D) and then exposure (E). The assessment and management at each stage is summarised in Tables 3-7. Throughout, the cervical spine should be immobilised if there is a concern about potential injury due
to trauma. Address any abnormalities in airway, breathing or circulation, in this order. Do not be distracted by other injuries until ‘A, B and C’ are stable. Don’t ever forget to measure glucose, as hypoglycaemia is an important cause of reversible decline.
Advanced Paediatric Life Support The Advanced Paediatric Life Support (APLS) algorithm is a useful tool for managing the unresponsive patient, and is summarised in Figure 1. Important differences, when compared to the advanced life support algorithm for adults, are: ӹӹ In a child under 2-years-old, the airway is opened by putting the child’s head in a neutral position and a chin lift is used. For older children, head tilt and chin lift is used, also known as the “sniffing the morning air position”. ӹӹ In neonates/smaller children, oropharyngeal airways (if used) are directly placed in the mouth in the position they are intended to sit. This contrasts to older children/adults, where oropharyngeal airways are put in upside down, and then twisted round to the correct position after being advanced. They are sized identically, i.e. from the angle of the mandible to the incisors. Nasopharyngeal airways are used identically as in adults. ӹӹ Cardiopulmonary resuscitation (CPR) begins with five ventilation breaths, then compressions to ventilation breaths at a rate of 15:2. This is because children are more likely to have a respiratory rather than cardiac problem. ӹӹ Technique for chest compression is different to adults (Figure 2 and 3). Compressions are also given at a faster rate, of 100-120 per minute. ӹӹ Ventilation breaths are given at a rate of 10-12 per minute, once the airway is secured.
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INITIAL MANAGEMENT
ASSESSMENT AND MANAGEMENT OF THE ACUTELY UNWELL CHILD
TABLE 3: Airway Assessment
Cause of compromise
Management
• Talk to the child. • Listen for stridor. • Consider obstruction in the airway, e.g. vomit, foreign body, swelling. • Check air entry in the chest.
• • • • •
• Keep the child calm, if the child is conscious and maintaining own airway – do not lay the child down if conscious. • If unconscious, place those 2-years-old in the “sniffing the morning air” position (head tilt and chin lift). • Airway adjuncts such as oropharyngeal or nasopharyngeal airways. • Intubation by skilled person only.
Croup. Epiglottitis. Foreign body. Tumour. Reduced consciousness.
TABLE 4: Breathing Assessment
Cause of compromise
Management
• Respiratory rate, oxygen saturation. • Look at chest movement, and work of breathing, e.g. accessory muscles, intercostal recession, grunting. • Palpate the trachea. • Percuss the chest wall. • Auscultate the chest.
• • • • •
• • • • •
Pneumothorax. Haemothorax. Consolidation. Flail chest. Respiratory splinting due to pain. • Anaphylaxis.
Oxygen. Relieve any pneumothorax or haemothorax. Pain relief, if appropriate. Ventilation. Treat anaphylaxis/sepsis.
TABLE 5: Circulation Assessment
Cause of compromise
Management
• Pulse (rate, rhythm, and volume). • Central capillary refill time. • Blood pressure. • Heart sounds. • Cardiac monitor.
• • • • • • • •
• Chest compressions, if no pulse. • Fluid bolus 20mL/kg (10 mL/kg boluses in trauma, diabetic ketoacidosis (DKA), neonates, raised intracranial pressure, cardiac conditions). • Inotropes. • Treat sepsis. • Electrocardioversion or chemical cardioversion. • Correct electrolytes. • Drain pericardial effusion.
Sepsis. Arrhythmia. Shock. Congenital cardiac disease. Hypoxia. Electrolyte abnormality. Cardiac tamponade. Anaphylaxis.
TABLE 6: Disability Assessment
Cause of compromise
Management
• • • •
• • • • • •
• • • • •
AVPU score/GCS. Pupillary responses. Focal neurological signs. Blood glucose.
Seizure. Space occupying lesion. Intracranial bleed. Raised intracranial pressure. Drug overdose. Sepsis.
Intubate if AVPU score is P (GCS 220 bpm in an infant and >180 bpm in a child). ӹӹ Not responsive to a fluid bolus. ӹӹ No P waves visualised on the ECG reading.
Management Stable Child If the patient is relatively well, vagal manoeuvres can initially be attempted. These vary depending on the age of the patient. The child must be attached to a heart monitor/ECG so that cardioversion can be captured and sent to the local tertiary unit. In infants, ice immersion can be attempted. This involves wrapping the infant and immersing its face into a basin of ice for approximately 10 seconds. This stimulates the diving reflex and can trigger the heart rate to return to normal. A variation of this is placing a bag containing crushed ice onto an infant’s face for 15–30 seconds. In children, a Valsalva manoeuvre can be attempted. One way of getting children to successfully comply with this is by asking them to blow into an empty 10 mL syringe.
Unstable Child In patients that are more acutely compromised or in those in whom initial vagal manoeuvres don’t work, adenosine may be given or DC cardioversion may be needed.
CARDIOLOGY
ӹӹ Haematology and biochemistry tests. These are useful in determining the underlying health of the patient and the effect their condition is having on the rest of their body. Anaemia, infection or dehydration may exacerbate a tachycardia. Imbalances of electrolytes (e.g. potassium, magnesium, calcium) or thyroid dysfunction may also trigger an arrhythmia. Magnesium levels have to be specifically requested as they are not part of a normal electrolyte work up. ӹӹ Echocardiogram. This is used to identify any underlying structural cardiac abnormalities.
Complications In the acute setting, SVT can lead to cardiovascular compromise, insufficient cardiac output, shock and ultimately death. Treatment also has complications. Vagal manoeuvres and adenosine can lead to asystole and should be done under careful monitoring, with full resuscitation facilities available. Following resolution of SVT, further ECG abnormalities may be detected. An example of this would be WolffParkinson-White syndrome (Figure 4). This is characterised by a slurred R wave, known as a delta wave, across all leads. The delta wave is caused by an accessory conduction system which bypasses the AV node, thereby shortening the PR interval and making the patient prone to episodes of re-entry tachycardia.
Prognosis In general, patients that have a single episode of SVT have a good prognosis, particularly with an uncomplicated presentation. SVT episodes may be recurrent. In the long-term, particularly if a patient has frequent episodes, prophylactic measures should be considered under the advice of a paediatric cardiologist. This would initially involve advice with regard to vagal manoeuvres and when to summon help. The patient may also be commenced on anti-arrhythmic medication. Those on longer term medications may suffer side effects, although SVTs can generally be well controlled, and medications are stopped after having an ablation. Patients may undergo radiofrequency ablation of their accessory pathway when they are teenagers, which renders that pathway nonfunctional.
FIGURE 4
Wolff-Parkinson White Syndrome. Short PR interval and slurred R wave (delta wave).
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CARDIOLOGY
Ventricular Tachycardia Aetiology Ventricular tachycardia (VT) is a broad complex tachycardia. The complexes are wide because the electrical activity originates within the ventricles themselves, resulting in a slower conduction than via the specialist conducting fibres within the heart (bundle of His and Purkinje fibres).
ӹӹ For patients not in shock, treatment with amiodarone is recommended. ӹӹ For those in shock, synchronized DC cardioversion is preferred. The senior clinician must always make the treatment decisions and, if possible, following discussion with a tertiary cardiac specialist.
Clinical Features
Complications
Although it may be possible to have short runs of VT and be asymptomatic, VT usually presents in a very compromised, tachycardic, hypotensive patient who may go on to have a cardiac arrest.
VT is associated with sudden death, and any suggestion of VT on ECG needs prompt evaluation by a senior clinician as the risks of cardiac arrest and further deterioration to ventricular fibrillation are high.
Investigations
Prognosis
It is important to review these patients frequently and to look for reversible causes of arrhythmia in their histories, examinations and biochemical evaluations. Investigations are similar to that for SVT. Figure 5 shows the ECG appearance of ventricular tachycardia.
This is highly dependent on the aetiology and clinical situation. Short runs of asymptomatic VT could go unnoticed over an entire lifetime, whereas VT could also be first noted at a cardiac arrest.
Management Any patient without a pulse requires cardiopulmonary resuscitation (CPR), cardiac defibrillation and possibly adrenaline/amiodarone administration. Patients with a pulse are divided into those in shock and those who are not.
Heart Block As with adults, children can get first degree (slowed conduction of atrial impulses to ventricles, causing prolonged PR interval), second degree (some atrial impulses are conducted to the ventricles, causing “dropped beats”) or third degree heart block (complete dissociation between atrial and ventricular activity (Figure 6)).
FIGURE 5
Ventricular tachycardia. Broad complexes, with no p waves visible.
FIGURE 6
P waves
QRS complex Complete heart block. No correlation between atrial complexes (p waves) and ventricular complexes (QRS complexes)
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CONGENITAL HEART DISEASE
Congenital Heart Block Congenital heart block is most often encountered in a baby whose mother suffers from connective tissue disorders with anti-Ro or anti-La antibodies (e.g. systemic lupus erythematosus; SLE). These antibodies are thought to attack the conduction system within the developing heart and cause atrophy of the AV node. This faulty node causes heart block, or sometimes intrauterine death or a hydropic foetus. Whenever a maternal history of connective tissue disorder is encountered or when anti-Ro or anti-La antibodies are found, an ECG must be performed on the baby after birth.
Long QT Syndrome Long QT syndrome is a syndrome in which the QT interval measured on ECG is prolonged. The corrected QT interval or QTc (from the beginning of the Q wave to the end of the T wave) should last less than 440 ms (or 490 ms if 38°C.
3
Vascular phenomena. • Septic pulmonary infarcts. • Intracranial haemorrhage. • Conjunctival haemorrhages. • Major arterial emboli. • Janeway lesions (small non-tender erythematous/macular lesions found on the soles of the feet or palms of the hands only).
4
Immunologic phenomena. • Osler nodes (small, painful and tender swellings found on the palms of the hands and soles of the feet only). • Roth Spots (retinal haemorrhage). • Glomerular nephritis.
5
Microbiological evidence. • Positive blood culture not typical of endocarditis.
6
Echocardiographic findings. • Consistent with diagnosis but do not meet major criteria.
Management At least two sets of blood cultures are taken, ideally before commencing antibiotics. If diagnosed, patients require a four to eight week course of antibiotics.
Prognosis Prognosis is good if appropriate antibiotics are started early. However, many infections are diagnosed late and are caused by resistant organisms: the mortality rate is extremely high in these cases.
CARDIOLOGY
Infective Endocarditis
TABLE 15: Modified Duke criteria for diagnosis of infective endocarditis
Kawasaki Disease Aetiology This is the most common systemic vasculitis of childhood. It is an acute febrile vasculitis, involving small and medium sized arteries. Kawasaki disease tends to affect children between six-months-old and six-years-old and is more prevalent in people of Japanese and Afro-Caribbean origin.
Clinical Features Children will present with a high fever, which is difficult to control, and irritability. Biochemically, they will have elevated inflammatory markers but no clear focus of infection. Children may also report abdominal pain, diarrhoea and vomiting and may have a characteristic thrombocytosis on full blood count, although this does not usually occur until at least a week later. Younger infants are more likely to be affected by significant complications and can present with “incomplete” or atypical symptomology.
Diagnostic Criteria Kawasaki disease is defined by a fever lasting more than five days without any clear focus of infection, plus any four of the following: ӹӹ Bilateral non-purulent conjunctivitis. ӹӹ Mucositis. Dry red lips / strawberry tongue. ӹӹ Cervical lymphadenopathy. ӹӹ Extremity changes. Red or indurated hands or feet, eventually leading to desquamation (peeling). ӹӹ Polymorphous rash.
Coronary Artery Disease The vasculitis can affect the coronary arteries, leading to coronary artery aneurysms. These may rupture; however, they more often resolve but leave behind scar tissue. This scar tissue causes narrowing of the arteries and can lead to ischaemic damage to the myocardium.
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CARDIOLOGY
Management Treatment consists of high dose aspirin (acting as an anti-platelet and anti-inflammatory) and intravenous immunoglobulin (IVIG). Aspirin therapy is continued for several weeks—and longer if any abnormal findings are observed on echocardiograms. Children must be followed up with regular ECGs and an echocardiogram to look for the presence of coronary artery aneurysms.
Prognosis With prompt treatment, prognosis is good, especially in older children. 20% of cases are IVIG resistant and higher risk patients may need IV high dose steroids or infliximab (anti TNF).
Rheumatic Fever Aetiology This is an autoimmune inflammatory process occurring approximately 20 days following acute infection with a group A beta haemolytic streptococcal organism (most commonly, group A strep pharyngitis). The prevalence of rheumatic fever in Europe has vastly decreased since the widespread use of effective antibiotics during streptococcal infections.
Clinical Features The Jones diagnostic criteria for acute rheumatic fever require recent infection with a group A streptococcus, plus two major or one major and two minor criteria (Table 16).
Management Acute rheumatic fever can be treated with anti-inflammatory medications and antibiotics if a group A streptococcus growth has been identified.
Prognosis Following prompt and adequate treatment, patients will have no long-term ill effects. If, however, recurrent or prolonged inflammation occurs, this can cause fibrotic changes and longterm complications, especially within the heart.
Myocarditis Aetiology This is an inflammation of the heart muscle itself, which may be caused by an autoimmune process, a toxin or an infective process. The most common aetiology is viral
TABLE 16: Modified Jones criteria Major Criteria
Minor criteria
• Carditis. • Arthritis (generally involving the large joints). • Erythema marginatum. • Sydenham chorea. • Subcutaneous nodules.
• • • •
Arthralgia. Increased PR interval. Fever. Increased ESR/CRP.
infection (often adenovirus or enterovirus, commonly Coxsackie virus).
Clinical Features Children with myocarditis can present with a wide spectrum of symptoms, and these are all largely non-specific. Signs and symptoms can include: ӹӹ Gallop rhythm. ӹӹ Abnormal ECG (non-specific ST changes and decreased QRS voltage). ӹӹ New cardiomegaly. ӹӹ Difficulty in breathing. ӹӹ Arrhythmias. Young children can present with cardiovascular collapse, arrhythmia and sudden death. In a shocked child, be wary if they worsen following a fluid bolus, as they may have been pushed into heart failure.
Management Diagnosis is largely clinical, although some centres use cardiac MRI for detailed imaging. Simple measures, such as diuretic therapy and ACE inhibitors to reduce afterload, can be instigated initially. These patients will often require management in an intensive care setting. Treatments have included intravenous immunoglobulin therapy, as well as corticosteroid therapy, to alter inflammatory processes and damage occurring within the myocardium.
Prognosis With the correct treatment, the majority will have complete recovery. Neonates have the poorest prognosis.
Pericarditis Aetiology This is an inflammation of the fibroelastic tissue covering of the heart, often with accompanying pericardial effusion. It is usually secondary to a viral infection. However, recurrent pericarditis or prolonged inflammation can lead to more significant issues, such as constrictive pericarditis.
Clinical Features It presents with pleuritic central chest pain that improves on leaning forward. Examination may reveal a pericardial rub, which is the squeaky sound made when the two layers of inflamed pericardium move against each other. This will also be louder as the patient sits forward.
Management In general, anti-inflammatory medicines and supportive care are all that are needed. An echocardiogram should be performed to look for possible effusions. ECG may show
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REFERENCES AND FURTHER READING
saddle-shaped ST elevation, although the most specific finding for pericarditis is PR depression.
Viral pericarditis is usually self-limiting, with no longterm sequelae. Bacterial pericarditis can be life threatening, particularly if diagnosis is delayed.
CARDIOMYOPATHIES A cardiomyopathy is a disease of the heart muscle itself. This differs from myocarditis in that the muscle is altered by the condition and it is not necessarily an acute inflammation.
Aetiology The cause is often genetic, but cardiomyopathy can also be associated with certain drugs and toxins, connective tissue disorders, post viral complications, sarcoidosis and amyloidosis. There are three main categories of cardiomyopathy: ӹӹ Dilated cardiomyopathy. The ventricles become dilated and less able to pump blood around the body. ӹӹ Hypertrophic cardiomyopathy. The ventricles become thickened and lack the capacity to pump blood adequately around the body. ӹӹ Restrictive cardiomyopathy. The muscle itself does not change in size or shape, but its compliance decreases as the ventricles become stiff.
Clinical Features Each pathology can initially present with non-specific symptoms of shortness of breath and increased fatigability. They can then progress on to heart failure.
Management Symptoms can largely be treated with medical therapies, like diuretics and inotropes. Sometimes pacemakers are needed to help control arrhythmias caused by the faulty cardiac muscle. Heart transplant is reserved for cases in which symptoms remain resistant to medical management.
Prognosis Predicting the long-term outlook is difficult, but hypertrophic cardiomyopathy tends to have better outcomes than dilated or restrictive cardiomyopathies.
REFERENCES AND FURTHER READING Cardiac Rhythms 1 Advanced Life Support Group. Advanced Paediatric Life Support. Fifth edition. Hoboken NJ: Wiley-Blackwell Publishing; 2005. 2 Tasker R, McClure R, Acerini C. Oxford Handbook of Paediatrics. Second edition. Oxford UK: Oxford Medical Publications; 2013.
1 Chan T et al. ECG in Emergency Medicine and Acute Care. St. Louis MO: Elsevier Mosby; 2005. 2 Hampton J. The ECG Made Easy. Seventh edition. London UK: Churchill Livingstone; 2008. 3 Park M. Pediatric Cardiology for Practitioners. Fifth edition. St Louis MO: Mosby 2007. 4 Surawicz B, Knilans T. Chou’s Electrocardiography in Clinical Practice. Sixth edition. Philadelphia PA: Saunders Elsevier; 2008.
CARDIOLOGY
Prognosis
The Paediatric ECG
Arrhythmias 1 Doniger S, Sharieff G. Pediatric dysrhythmias. Pediatr Clin North Am. 2006; 53:85. 2 Josephson M, Wellens H. Differential diagnosis of supraventricular tachycardia. Cardiol Clin. 1990; 8:411. 3 Fleming S, Thompson M, Stevens R, et al. Normal ranges of heart rate and respiratory rate in children from birth to 18 years of age: a systematic review of observational studies. Lancet. 2011; 377:1011. 4 Samuels M, Wieteska S. Advanced Paediatric Life Support the practical approach. Fifth edition. Hoboken NJ:WileyBlackwell Publishing; 2005. 5 Wren C. Catheter ablation in paediatric arrhythmias. Arch Dis Child. 1999;81:102-4
Congenital Heart Disease 1 Marino B, Bird G, Wernovsky G. Diagnosis and management of the newborn with suspected congenital heart disease. Clin Perinatol. 2001; 28:91. 2 Sasidharan P. An approach to diagnosis and management of cyanosis and tachypnea in term infants. Pediatr Clin North Am. 2004; 51:999. 3 Blaz W et al. Estimation of usefulness of non-invasive cardiovascular diagnostic screening methods in early detection of critical congenital heart defects in newborns. Arch Dis Child. 2014; 99:a120. 4 Perloff J. Ventricular septal defect. In: The Clinical Recognition of Congenital Heart Disease, Fifth edition. Philadelphia PA:W.B. Saunders Company; 2003. 311. 5 Van Der Linde D et al. Birth Prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol. 2011; 58:2241.
Heart Murmurs 1 Frank J, Jacobe K. Evaluation and management of heart murmurs in children. Am Fam Physician. 2011;84:7.
Heart Failure 1 Rosenthal D et al. International Society for Heart and Lung Transplantation: Practice guidelines for management of heart failure in children. J Heart Lung Transplant. 2004; 23:1313. 2 Madriago E, Silberbach M. Heart failure in infants and children—Pediatrics in review. Am Acad Pediatr. 2010; 31:1.
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3 Kantor P, Mertent L. Heart failure in children. Part I: Clinical evaluation, diagnostic testing, and initial medical management. Clinical practice. Eur J Pediatr. 2010; 169:269-79.
Infection and Inflammation of the Heart 1 Eleftheriou D, Levin M, Shingadia D. Management of Kawasaki disease. Arch Dis Child. 2013; 0:1-10. 2 NICE. Prophylaxis against infective endocarditis: Antimicrobial prophylaxis against infective endocarditis in adults and children undergoing interventional procedures. 2008. https://www.nice.org.uk/guidance/cg64
3 Hoyer A, Silberbach M. Infective endocarditis—Pediatrics in review. Am Acad Pediatr. 2005; 26:11.
Cardiomyopathies 1 Steven D, Colan M. Hypertrophic cardiomyopathy in childhood. National Institutes of Health. Heart Fail Clin. 2010; 6: 433-44. 2 Burch M et al. Dilated cardiomyopathy in children: determinants of outcome. Br Heart J. 1994; 72:246-50.
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1.04
COMMUNITY PAEDIATRICS ANTONIA HARGADON-LOWE
DEVELOPMENT Normal Development and the Developmental Assessment Developmental history is often missed when seeing children in acute settings or in primary care. This may be due to time constraints in a busy Emergency Department (ED) or outpatient clinic. However, some information can be gathered quickly with adequate knowledge and practice. Picking up developmental problems early can have a significant impact on that child’s outcome. The time taken and depth of questioning on development depends on the clinical scenario (Table 1). TABLE 1: Developmental assessment in different settings
CONTENTS 35 Development
35 Normal Development and the Developmental Assessment 37 Routine Childhood Surveillance 39 Developmental Delay
41 Important Conditions
41 Autistic Spectrum Disorder 43 Attention Deficit Hyperactivity Disorder 43 School Refusal 44 Toddler Tantrums 44 Sleep Disorders 44 Learning Difficulties
45 Safeguarding
45 Categories of Abuse 46 Presentation of Child Abuse 47 Management of Abuse
48 References and
Further Reading
Scenario
Suggested Approach
3-year-old child presenting to ED with a fever and ear pain, and no known developmental concern.
Brief questioning about milestones and parental concerns, e.g. • Do you feel your child is able to do the same things other 3-year-olds can? • At what age did she smile, say her first word and walk?
Outpatient clinic review of 2-year-old boy who has had three seizures in the last few months.
More in-depth questioning is required as relevant to the presenting complaint. The number of questions will depend on the answers and whether concerns emerge. Ascertain whether he is similar to other children of his age. Developmental regression should also be enquired about, i.e. has he lost any skills previously developed. To start, ask a couple of questions in each developmental domain to assess whether he is meeting expected targets for his age: • Gross motor. Is he able to jump? Is he able to climb stairs, and if so, how does he do it? • Fine motor. Can he draw a straight line? How big a tower block can he build with building blocks? • Language/hearing. Can he speak in short sentences? Can he obey simple commands? • Social/Self-Care. Can he eat with a fork/spoon? Does he socially interact (good eye contact and smiles) with adults and other children? • General. Has he lost any skills that he could previously do?
Community paediatric In this situation, the reason for the referral is “developmental outpatient appointment concerns”, so a full assessment taking approximately one hour is following a referral from necessary using one of the available tools. nursery school with concerns of development.
Developmental Milestones Development is usually categorised into distinct areas; commonly the following domains are used: “Gross Motor”, “Fine Motor/Vision”, “Language” and “Social/SelfCare” (Table 2). This is done because developmental milestones can often be grouped together, and if one sector is affected but not the others, it may indicate a particular group of pathologies. For example, language delay may be secondary to hearing loss, whereas hearing loss is unlikely to cause isolated gross motor delay.
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TABLE 2: Average developmental milestones Gross Motor
Fine Motor / Vision
Language/Hearing
Social / Self-Care
6 weeks.
• Holds head briefly in ventral suspension. • Some head lag. • Primitive reflexes. • Symmetrical limb movement.
• Holds fists closed. • Turns to light. • Fixes and follows through 90 degrees – especially faces.
• Cries. • Responds to mother’s voice. • Startles to noise.
• Smiles.
3-4 months.
• No head lag. • Raises chest (resting on elbows) when prone. • More vigorous limb movements.
• Holds object placed in hand. • Reaches for objects. • Fixes and follows through 180 degrees.
• Cooing. • Quietens to mother’s voice.
• Laughs.
5-6 months.
• Sits supported / unsupported with curved back. • Lifts chest on extended arms when prone. • Rolls front to back and later back to front.
• Brings objects to midline. • Transfers between hands. • Consciously releases objects. • Starts mouthing objects.
• Early babbling (with consonants).
• Screams (happy). • No stranger anxiety.
8-9 months.
• Sits unsupported with a straight back. • May crawl / bum shuffle.
• Early pincer grip (uses thumb with all fingers). • Looks for fallen objects.
• Developed babbling – repetitive consonants. • Says “Mama” and “Dada” non-specifically.
• Stranger anxiety develops. • Plays peekaboo.
10 months.
• Pulls to stand. • Cruising.
• Developed pincer grip – picks ‘grains of rice’ off the floor.
• Understands “No”.
• Understands concept of “bye-bye”.
12 months.
• Early walking – unsteady / broad based gait.
• Bangs objects together. • Casts objects. • Object permanence – looks for hidden toys. • Points at things.
• Few individual words. • Imitates sounds and speaks jargon with conversational intonation. • Understands names and simple objects.
• Finger feeds. • Can hold spoon and attempt use. • Waves bye-bye / claps. • Points to convey desire, shortly followed by pointing for excitement.
15 months.
• More confident walking.
• Builds tower of two or three bricks. • Turns thick cardboard pages.
• Obeys simple commands. • Can identify common objects.
• Uses cup and spoon.
18 months.
• Squats to pick up object. • Running.
• • • •
• 10-20 words including common objects. • Common two word phrases e.g. “all-gone”. • Points to body parts.
• Removes socks / shoes. • Imitative play / domestic mimicry (copies parents’ actions at home).
2 years.
• Jumping. • Kicks ball. • Climbs stairs two feet per step / holding on.
• Circular scribble. • Draws straight line (at 2.5 years). • Builds tower of six blocks.
• Has 50+ words. • Links words together to make two or three word sentences. • Understands functions of objects (which one do you eat with? [said whilst pointing to a fork and a pencil]), and verbs (who is running?). • Obeys two-part commands.
• • • •
3 years.
• Rides a tricycle. • Copies a circle. • Goes upstairs with one foot • Builds a tower of nine per step, and downstairs bricks. with two feet per step. • Copies a bridge and stairs with six bricks.
• Knows several nursery rhymes. • Complex four to five word sentences. • States first and last name. • “What” and “Who” questions. • Understands prepositions e.g. under / behind. • Understands sizes e.g. big/ little, tall/small.
• Washes hands. • Plays with other children. • Understands concept of sharing. • Pretend play.
Builds tower of four blocks. To and fro scribble. Points to pictures in books. Turns pages in book a few at a time.
Feeds with fork + spoon. Starts toilet training. Temper tantrums. Plays alongside other children (parallel play). • Symbolic play.
(Continued)
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COMMUNITY PAEDIATRICS
TABLE 2: Continued Gross Motor
Fine Motor / Vision
Language/Hearing
Social / Self-Care
4 years.
• Hops. • Walks up and down steps like adult.
• Copies a cross. • Copies stairs with 10 bricks.
• Can tell descriptive account of events. • Uses “why”, “when” and “how”. • Understands negatives (e.g. which one is not a boy?) / three-part commands.
• Undresses but cannot dress independently. • Understands turn-taking.
5 years.
• Skips. • Catches ball.
• Copies a triangle / square.
• Tells a complex story using all tenses – future and past.
• Dresses, including buttons and zips. • Can eat with knife and fork. • Chooses and names best friends. • Imaginative play including role playing and made up stories.
Figure 1 shows the progression of grip and Figure 2 shows the progression of gross motor control. Milestones are important but sometimes difficult to learn, mostly because the “normal” ages vary. It is also important to distinguish between “average” and “when to be worried”. As a child gets older, the “normal” range for a milestone spans a greater period. Note also that corrected gestational age needs to be calculated in premature babies less than 2-years-old. Corrected gestational age is calculated as:
FIGURE 2 A
B
C
E D
Corrected gestational age = Chronological age – (number of weeks premature) Therefore, a 10-month-old child born two months early has a corrected gestational age of 8-months-old. Developmentally, the child should only be expected to perform in line with an 8-month-old child, not a 10-month-old child. Certain thresholds for referral are important to recognise, as summarised in Table 3.
Routine Childhood Surveillance Children and their families are assessed at various key points during their early years. Table 4 summarises the current assessments carried out in the UK based on the 2009 Department of Health “Healthy Child Programme”. The aim is to pick up
Gross motor progression in first year. (A) Raises chest when prone (three to four months). (B) Sits unsupported with curved back (five to six months). (C) Sits unsupported with straight back (eight to nine months). (D) Cruising (10 months). (E) Early walking (12 months).
any developmental concerns early and implement a package of support tailored to that child to address the underlying issues.
Growth and Development
FIGURE 1
A
B
Development of grip in first year. (A) Palmar grip (three to four months). (B) Early pincer grip (eight to nine months). (C) Fine pincer grip (10 months).
C
Until 5-years-old, the child’s feeding, growth and development should be monitored. The “Red Book” or parent held health record is given in the first few weeks of life in the UK. This contains a record of vaccinations, early midwife and health visitor assessments, growth charts and general advice on the care of a young child. At each of the screening points, the weight can be plotted on their growth chart. Through the first year, this may be done frequently to ensure they are growing adequately, and then less frequently thereafter. All practitioners
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should ensure vaccinations are up-to-date at any encounter with a child from birth to 5-years-old.
Vision Routine visual screening is done initially at the newborn and six-to-eight week check with a red reflex. It is then performed at pre-school or during school entry at 4 to 5-years-old. Concerns outside of these checks will lead to a referral to either an optometrist, ophthalmologist (medically qualified) or an orthoptist. TABLE 3: Some important situations where referral for further specialist paediatric assessment is indicated Age
Feature
6-8 weeks.
• • • • •
12 weeks.
• Persistent squint.
8 months.
• Persistent primitive reflexes. • Not vocalising.
1 year.
• Hand preference. • Not responding to their own name.
18 months.
• Not walking.
2 years.
• No or few words spoken by child.
3 years.
• • • • •
Asymmetrical Moro reflex. Unable to fix and follow. Not smiling. Excessive head lag. No startle to sound.
Squints “Neonatal misalignments” are common in the first two months (where the eyes do not focus on the same spot) but this usually reduces in the second month. If it is persistent at 10-12 weeks, specialist referral is warranted, as it may represent a squint. The earlier the detection and referral, the more likely specialists are able to correct the squint, allow binocular vision and depth perception to develop as normal, and prevent amblyopia (a lazy eye where the brain chooses to ignore its image), thereby minimising visual dysfunction.
Red Reflex The “red reflex” can be assessed at any age but is routinely done at the newborn and six-to-eight week check. This term refers to the red reflection of light from the retina when looking through an ophthalmoscope. This test is routinely done at birth to screen for problems such as cataracts and retinoblastomas (where the red reflex is absent and instead there is a “white reflex” or leukocoria). The absence of the red reflex should prompt urgent referral to an ophthalmologist or paediatrician.
Fixing and Following
Not speaking in sentences. Not interacting with other children. Not following simple commands. Unable to use the toilet. Unable to use a spoon.
Fixing and following across the midline up to 180 degrees occurs by three-months-old. The visual pathway then continues to develop during infancy, with rapidly improving acuity in the first six months, reaching near adult levels at approximately 12 months. Colour vision is well developed by six months. Visual acuity tests, summarised in Table 5, are age dependent. The “Ishihara tests” for colour vision are usually used for those more than 10-years-old but have been adapted for use on children from 3-years-old using shapes instead of numbers.
TABLE 4: Routine surveillance of children Time Frame
Assessor
Monitoring
Newborn examination.
Paediatric doctor or midwife.
The baby is examined from head to toe, looking for any obvious abnormalities. The eyes are assessed for a red reflex. Feeding is checked, as is whether the baby is passing urine and has opened their bowels. Weight and head circumference are checked. Hips are assessed and the heart is auscultated for the presence of a heart murmur. A newborn hearing screen is usually carried out and it is ensured that the parents have a basic understanding of how to look after a baby, and when to seek help.
Early newborn baby review 7–14 days.
Health visitor or midwife.
The baby is reassessed to ensure adequate feeding and to identify any parental anxieties or mental health concerns (e.g. postnatal depression) that may have emerged. Such reviews are usually frequent, and in the UK, this is also when the Guthrie test is performed (p210).
6–8 week review.
Primary care doctor.
An assessment similar to the newborn assessment is repeated, particularly focusing on identifying problems with the eyes, hips, heart and testicles. General matters of concern are also addressed.
8–12 months.
Primary care doctor.
Full developmental check is carried out. Growth and feeding are looked at. Any problems identified are addressed as they arise.
2–2.5 years.
Health visitors.
Full developmental check is carried out. Social developmental concerns that may affect a child’s ability to access the educational curriculum are usually more obvious at this age. Any children with concerns will be referred on for a specialist assessment.
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DEVELOPMENT
Hearing The critical age for intervention in sensorineural hearing loss (SNHL) is before six-months-old; therefore, it is important to assess hearing early. In the UK, the Newborn Hearing Screen has been in place since 2006, replacing the distraction test at 8-months-old. If the child fails the test, or has certain risk factors, they should be referred to an audiologist. Table 6 summarises hearing testing.
Developmental Delay Developmental delay is a broad term and covers delay in any of the four main areas, whilst the term global developmental delay indicates that two or more areas are delayed.
Aetiology Causes include: ӹӹ Neurological ›› Congenital. e.g. embryological disorders of brain development, antenatal vascular event. ›› Acquired (static). e.g. hypoxic ischaemic encephalopathy (from birth asphyxia), intraventricular bleeds, prolonged hypoxia (arrest, seizure) or hypoglycaemia, traumatic brain injury, ischaemic stroke. ›› Acquired (progressive). e.g. brain tumour, subsclerosing panencephalitis. ӹӹ Infection ›› Congenital. e.g. TORCH (Toxoplasmosis, Other, Rubella, CMV, Herpes) infections, HIV. ›› Acquired. e.g. meningitis, herpes encephalitis.
COMMUNITY PAEDIATRICS
The shapes are made up of multiple dots of one colour on a background of dots of various other colours.
TABLE 5: Summary of visual acuity tests in children Test
Age Range
Description
Cardiff Acuity Test.
1 to 3-years-old/Those with developmental delay.
Works on the principle of preferential looking; i.e. a child will prefer looking at an image of an object, when compared to a plain image.
Stycar Letter Matching Test.
3 to 5-years-old.
There are versions with shapes and balls for younger children. The child is given a key card of letters and is presented with single letters of reducing size at three metres and asked to point to the matching letter on their key card. It uses only five letters at first (VTOHX) and then increases to seven then nine letters. The “Sheridan Gardiner test” is very similar.
Snellen Chart.
5-years-old and older.
Many letters on one chart (of varying font size) are presented to the child at a distance of 6 metres. The child is asked to either point to a matching letter or say the letter.
TABLE 6: Summary of hearing tests in children Test
Age Range
Description
Oto-acoustic emissions (OAE).
From birth.
Used for neonatal screening (first line) and work on the basis that the inner ear produces a sound in response to noise, which is recorded. It will therefore miss sensorineural hearing loss (SNHL) if the cochlear function is normal.
Auditory brainstem responses.
From birth.
Used if OAE fails. For this, clicks are presented to each ear and electrodes on the scalp pick up responses. Auditory brainstem responses do not distinguish between conductive hearing loss (CHL) and SNHL, and is not possible to carry out if the infant is moving.
Distraction testing.
6 to 9-months-old (although can be used up to 24-months-old).
Involves a parent and two examiners – one gains the child’s attention whist the other makes a noise behind the child. In a positive test, the child should move their head towards the noise.
Performance testing.
2-years-old.
Requires the child to respond to different instructions/tasks at various frequencies and volumes. It does rely on appropriate receptive language development so needs careful interpretation.
Discrimination testing, e.g. “McCormack Toy Discrimination test”.
2-years-old.
Asks the child to identify objects or pictures using words that sound similar.
Pure tone audiometry.
4-years-old.
Headphones are placed on the child and sounds of different volumes and frequencies are created. The child must indicate when they hear a noise and on which side. It can differentiate between CHL and SNHL.
Tympanometry.
All ages.
Tests for middle ear pathology – a sound is passed into the ear, the amount reflected back is measured and then the compliance is determined by repeating this procedure at varying pressures. The results are then graphed.
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ӹӹ Neuromuscular disorder. e.g. Duchenne Muscular Dystrophy. ӹӹ Endocrine abnormalities. e.g. hypothyroidism. ӹӹ Genetic. e.g. Down syndrome, phenylketonuria. ӹӹ Pervasive developmental disorders. e.g. autism. ӹӹ Nutritional. e.g. vitamin deficiencies and malnutrition. ӹӹ Others. e.g. isolated speech and language delay, prematurity, metabolic disorders, idiopathic.
Investigations Tables 7 and 8 summarise investigations that might be considered in global developmental delay.
Management If concerns are raised about possible developmental delay, the child should be referred to a community paediatrician. The majority of referrals come from health visitors, nurseries, schools, or speech and language therapists, with some coming from primary care physicians or hospital doctors – either with concerns picked up by them or from the parents. The community paediatrician will see the patient for a much longer period (usually at least an hour) and will perform a detailed developmental assessment and wider assessment of the well-being of the child and the family.
TABLE 7: Routine investigations in Global Developmental Delay Investigations
Justification
Full blood count (FBC) + Haematinics.
Iron deficiency, folate / B12 deficiency — can cause developmental delay and learning difficulties.
Urea and electrolytes (U&Es).
Renal failure and hyponatraemia both may cause poor growth. Renal osteodystrophy can lead to gross motor delay. A baseline is also useful for any future tests.
Creatinine kinase.
Early detection of Duchenne Muscular Dystrophy.
Thyroid function tests.
Hypothyroidism is a well-documented cause of developmental and intellectual delay. If treatment is delayed, its effects are irreversible.
Liver function tests (LFTs).
Abnormalities may suggest further investigations such as those to look for an underlying metabolic disorder.
Bone profile (+ Vitamin D).
Vitamin D deficiency can cause motor delay. Hypocalcaemia can represent this or another underlying disorder with delayed development (e.g. 22q11 deletion).
Hearing test.
Speech and language delay may be as a result of a hearing defect, but genetic disorders with global delay may also involve conductive or sensorineural hearing loss.
TABLE 8: Second-line investigations in Global Developmental Delay Investigations
Justification
Karyotype.
Karyotyping may identify trisomy disorders or Turner syndrome.
DNA testing.
The FMR1 DNA test can determine if Fragile X Syndrome is present. Fragile X can cause developmental delay, without necessarily showing obvious dysmorphism early on.
Array comparative genomic hybridization (CGH).
Array CGH can identify chromosomal microdeletions or microduplications and may explain previously unknown causes of developmental delay. If a defect already recognised is detected, it may help with prognosis, but if not, then it may contribute to research and help future children. Testing of other family members is offered to clarify if the genetic abnormality is a new finding in the child or inherited from a parent.
Metabolic screen.
Inborn errors of metabolism, e.g. mucopolysaccharide disorders, peroxisomal disorders (both usually dysmorphic), organic and aminoacidaemias, and mitochondrial disorders. These are found in a very small proportion of developmental delay (approximately 1%).
Lead.
Lead toxicity could be found in children with developmental delay and no other symptoms, and is treatable.
Biotinidase.
Biotinidase deficiency can present as developmental delay as its only sign and is treatable.
Brain magnetic resonance imaging (MRI).
This may identify a structural abnormality or damage resulting from a static event (e.g. infection, trauma, etc.) or a progressive neurological disorder. This may require a general anaesthetic in younger children.
Electroencephalogram (EEG).
For children with developmental regression, or associated seizures, an EEG may show typical features of an epilepsy syndrome, e.g. West syndrome, Landau-Kleffner syndrome.
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IMPORTANT CONDITIONS
There are only a few causes of reversible developmental delay. This means most children with developmental delay suffer chronic morbidity. In all cases, the underlying cause needs to be treated, if possible. However, many cases are of unknown
aetiology, with management centred on supportive therapy and the aim of maximising the underlying potential in that individual child. Table 9 summarises specialists that may be involved.
IMPORTANT CONDITIONS Autistic Spectrum Disorder Autistic Spectrum Disorder is a developmental disorder characterised by symptoms in three domains: ӹӹ Social interaction. Lack of ability to have normal social interactions. ӹӹ Social communication. Significant speech delay, with some never acquiring speech, and lack of non-verbal communication (e.g. eye contact, social smiling, facial expressions, gestures).
COMMUNITY PAEDIATRICS
Various assessment tools are available for measuring the developmental level: ӹӹ The Griffiths assessment is one of the most comprehensive developmental assessment tools and is often used in the UK, though it requires additional training so is not expected knowledge of a non-specialist. ӹӹ Specialised testing kits are also available to facilitate developmental testing of children with a sensory impairment, e.g. the Reynell Zinkin assessment kit for children with a visual impairment.
TABLE 9: Specialists that may be involved in the care of a child with developmental delay Specialist
Role
SALT (speech and language therapist).
Provide assessment and intervention for children with swallowing and communication disorders.
Occupational therapist.
Identify and manage fine motor problems, coordination, sensory processing difficulties and executive functioning. They can advise on and coordinate adaptation of the house and equipment to the child’s needs.
Physiotherapist.
Identify and manage gross motor disorders. They can advise on and coordinate provision of any necessary equipment to the child to aid these skills.
Health visitor.
General health surveillance, screening and advice to parents and their children under 5-years-old.
Geneticist.
Provide advice on possible genetic disorders, testing and implications for future family planning.
Neurologist / Neurodisability specialist.
Provide a specialist neurological opinion on possible aetiology, arranges and interprets specialist investigations, and provides specific therapies (for example, in cerebral palsy, prescribe baclofen injections for reducing spasticity).
Audiologist.
Through a variety of tests, they can provide information on the child’s hearing and any associated defect. They coordinate treatment, if indicated.
Social worker.
Disability social workers specialize in working with children with disabling conditions. Social workers are also involved with a family if there are child protection concerns.
Educational psychologist.
Carry out specific assessments on a child’s ability to learn and perform in various learning functions. They will provide a summary of the child’s relative IQ in all these areas.
Hospice / Respite care.
This may be necessary if the child’s condition is terminal.
Ophthalmologist.
Assess and offer treatment for visual difficulties.
CAMHS (Child and Adolescent Mental Health Services).
CAMHS offer psychiatric support and therapy to children with possible mental health disorders including severe behavioural difficulties.
Hospital paediatrician.
May be required for basic management of things such as enuresis, constipation, reflux, pain or specific organ involvement.
Community paediatrician.
Assess the development of a child in detail, including emotional and behavioural aspects. Generally, community paediatricians co-ordinate all the care of the child and ensure no issue is left unaddressed. Advocacy for vulnerable families is a significant part of the role of the community paediatrician.
School teachers/staff.
There may be a Special Educational Needs Coordinator (SENCO) for the school, who ensures every child’s learning needs are met including those with developmental delay and/or learning difficulties. This is often provided by the school with extra support. However, if additional support and funding are required, a “Statement of Special Educational Needs” should be applied for. This is granted by the local council and states that a full assessment has been carried out and the child is entitled to extra monetary support to enable them to access the curriculum. The school also may host Team around the Child (TAC) meetings: this is where all personnel involved in a child’s care meet and discuss best courses of action.
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ӹӹ Rigidity of thought and behaviour (social imagination). Dislike of routine change, need for stereotyped and repetitive activities, and lack of ability to imagine.
Aetiology Autism is apparently increasing in the UK population. This could reflect increased awareness, earlier recognition and improved diagnosis. Autism is currently estimated to affect approximately 30–60 per 10,000 people. The exact aetiology is unknown but genetic associations are being increasingly recognised. There are theories regarding environmental factors but no strong evidence. Extreme emotional deprivation can produce autistic features in children and safeguarding concerns should be carefully looked for in the history. Attachment Disorder and Autism Spectrum Disorder can present in a very similar fashion, but “sensory sensitivities” are more common in autism. This refers to difficulty in processing information from sensory channels, e.g. smell, taste and touch, and is a feature of autism. In 1998, a link was suggested between autism and the measlesmumps-rubella (MMR) vaccine, but this has subsequently been discredited. Nevertheless, the initial publication led to a reduction in MMR vaccinations and a subsequent increase in cases of measles. Parents should be opportunistically advised that no link exists between the MMR vaccine and autism and they should be encouraged to still vaccinate. Ten to fifteen percent of cases of autism have a diagnosable underlying cause, including: ӹӹ Tuberous sclerosis. ӹӹ Fragile X syndrome. ӹӹ Phenylketonuria. ӹӹ Associations with chromosomal microdeletion syndromes. Primary autism can also be associated with depression, ADHD and other behavioural disorders, as well as Tourette syndrome. People with autism frequently
have lower IQs than average, but this is not always the case.
Clinical Features Children may present: ӹӹ At birth, with minimal social skills (limited social smiling, limited eye contact and minimal enjoyment in games). ӹӹ Following regression or arrest of social skills, having initially been a sociable baby with normal development. ӹӹ Much later, following a social challenge such as progression to senior school (more common in girls). The spectrum of severity ranges from those who are able to function with just a little extra support in mainstream schools and will go on to have normal relationships and jobs, to those who never develop any speech, never become toilet trained and always have severe learning difficulties. Common features include: ӹӹ Delayed or lack of pointing. Children with autism may use the carer’s hand to point to things instead. ӹӹ Delayed speech. Children may have limited language development to varying degrees. ӹӹ Lack of response to name. This is noticeable from an early age. ӹӹ Poor eye contact. Often children with autism are described as being “in their own world”. ӹӹ Delayed and disordered play development. Children may be observed not playing with or interacting with other children (in a conventional way). They may have rituals or certain play that is repetitive in nature, including lining things up or being obsessed with a certain toy or topic. These children lack imagination and often do not demonstrate makebelieve play or role-playing games. ӹӹ Strong dislike for routine change. This may include things like the route to school or an order in which things happen, and change will lead to a tantrum.
ӹӹ Motor mannerisms. Examples include hand flapping or jumping. ӹӹ Sensory symptoms. There may be a dislike of loud noises and certain textures – most commonly of food and clothing. Children may display sensory seeking behaviour such as excessive play with water/sand or touching/stroking people’s skin or hair. ӹӹ Regression. Some parents will report apparently normal language progression until around 15-monthsold, followed by loss or regression of language skills. It is very important to ask about possible regression in the history, as this can be a feature; i.e. normal initial development does not rule out autism. However, developmental regression needs further investigation to rule out other causes, e.g. Rett Syndrome. Children with higher functioning autism may have an exceedingly high level of knowledge about a peculiar topic, or an aptitude to remember details or numbers above that of children their age or even adults.
Investigations Investigations vary depending on the clinical findings, but are broadly similar to that for developmental delay. There are no known biomarkers for autism, but screening tools include: ӹӹ Gilliam Autism Rating Scale (GARS2) for pre-school children. ӹӹ Social Responsiveness Scale (SRS) for older children. ӹӹ Modified Checklist for Autism in Toddlers. Clinical assessment and diagnosis is based on an in-depth history and prolonged clinical observation through a variety of tasks and assessments. Multi-disciplinary assessment by both a community paediatrician and a speech and language therapist are recommended for autism assessment and diagnosis. The “Autism Diagnostic Interview” and “Autism Diagnostic Observation Schedule” are considered gold standards for this.
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IMPORTANT CONDITIONS
The management is multi-agency and complex. With certain strategies, children with autism can potentially achieve a higher functional level and quality of life. Since many professionals are involved, a “keyworker” usually coordinates and plans individually tailored care for each child (including outreach, one-on-one and group interventions). Some possible therapies for the child include: ӹӹ Speech and Language Therapy (SALT). These professionals form the mainstay of management and provide ongoing support and therapy, often teaching the child and family Makaton (a language programme using signs and symbols) in order to communicate. ӹӹ Psychosocial intervention. Techniques can be employed to try and increase the child’s attention, interaction, engagement and reciprocal communication. This may include play-based therapies. Cognitive behavioural therapy may be appropriate in older children if they have the verbal and cognitive ability to engage with it. ӹӹ Behavioural intervention. This includes assessing factors which might contribute to challenging behaviour, e.g. the physical environment, and making adaptive changes (for example making the environment less noisy). Co-existing medical disorders may also contribute to challenging behaviour and therefore need to be addressed. Psychological and psychiatric illness, e.g. depression or anxiety, can co-exist and need to be identified and therapy instituted where required. ӹӹ Sleep problems. This is common in autism, and triggers should be identified, e.g. background noise, irregular bedtimes or potentially co-morbidities such as hyperactivity. Medication, such as melatonin, can be considered. ӹӹ Adaptation skills. It is helpful also to think about general support
for development, and access to community services; for example, public transport and leisure facilities. Education needs to be supported, and it may be the case that such children require a statement of special educational needs. ӹӹ Pharmacological intervention. If psychosocial interventions are unhelpful, and if behaviour remains very challenging, then antipsychotic medication can be considered and started by a specialist. This is very rarely done. The needs of carers and families should also be identified. They may require personal, social and emotional support, as well as practical support in providing care.
Attention Deficit Hyperactivity Disorder Attention deficit hyperactivity disorder (ADHD) is a condition characterised by three themes: 1 Hyperactivity. Fidgety, constantly on the move, reduced sleep requirement. 2 Inattention. Easily distracted, poor concentration. 3 Impulsiveness. Acts without reflection, impatient.
Aetiology Genetic and environmental factors have been implicated in the aetiology. There are common co-existing and not mutually exclusive diagnoses including: ӹӹ Mood disorders. ӹӹ Conduct disorders. ӹӹ Learning difficulties. ӹӹ Communication disorders (including autism). ӹӹ Anxiety disorders. ӹӹ Movement disorders (including Tourette syndrome and tics). As a result, care must be made in diagnosis. There is also an increased frequency in underlying conditions including: ӹӹ Neurofibromatosis. ӹӹ Angelman syndrome.
ӹӹ Fragile X syndrome. ӹӹ Foetal alcohol or drug exposure. ӹӹ Very low birth weight/extremely premature infants.
Investigations ADHD is diagnosed clinically, by a child psychiatrist or community paediatrician, without any biochemical tests unless alternate diagnoses are being considered. Children suffering from ADHD tend to be either the predominantly hyperactive and inattentive type or the impulsive type. For diagnosis, the condition must: ӹӹ Have an early onset. ӹӹ Have moderate impairment to emotional, educational and social functioning. ӹӹ Demonstrate chronicity.
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Management
Features should occur in two or more settings (e.g. at home and at school) to demonstrate that the behaviour is not specific to one environment. Diagnosis is supported with scoring charts, assessing for traits in the three themes described above. The most common method uses a Conner’s questionnaire. From this, a score will be generated which strongly supports but does not make the diagnosis.
Management Management involves an MDT approach, with education, behavioural intervention, medication and ensuring a healthy, nutritionally balanced diet. If a link is found with specific food groups, including artificial colourings/ additives, then exclusions/adaptations to the diet may be indicated; however, this should be done in conjunction with dietetic advice. The first line medication is usually methylphenidate (a dopamine/ noradrenaline reuptake inhibitor).
School Refusal Stressful life events can often trigger school refusal. This may manifest in a number of chronic symptoms with no organic basis, such as headaches, stomach aches, nausea or diarrhoea, but can also present with tantrums,
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difficult behaviour or separation anxiety. This may represent a normal part of development and may be short lived, or it may represent more underlying problems, including an emerging underlying anxiety disorder or safeguarding concerns. Each case should be individually assessed, with consideration given to referral to child and adolescent mental health services if needed.
Toddler Tantrums
Learning Difficulties Learning difficulty describes the restriction that a child has within an area of their learning development. Learning difficulties can be general or be specific to certain areas, including literacy, numerical reasoning, non-verbal and verbal reasoning, and memory. They can be subcategorised as mild, moderate or severe. The terms “learning disability” and “learning difficulty” are used interchangeably.
Temper tantrums can start at around 18-months-old and are very common in toddlers. One reason they might occur is that children experience frustration because they have desires they want to express but do not have the language to express them. Management involves trying to understand the reason for the tantrum (for example, being hungry or feeling frustrated at something) and finding distractions for the child. The temptation to shout back at the child should generally be resisted, as this may worsen the situation. Temper tantrums are self-limiting and become less frequent as the child grows older.
Aetiology
Sleep Disorders
Learning difficulties are usually identified by school teachers due to failure to meet academic standards. It is most accurately assessed by educational psychologists but the child often also sees a community paediatrician to rule out an undiagnosed developmental disorder. They may also exist in those with pre-existing conditions where learning difficulties were predicted. Investigations may be helpful in identifying an organic cause for the learning difficulty, which may be reversible. They are broadly similar to that for developmental delay.
Nightmares Nightmares occur during Rapid Eye Movement (REM) sleep and so usually occur in the second half of the night. They are common at 5 to 10-years-old, and children often remember the content of the nightmare. If they occur less than once a week, parents should be reassured, but if they are high frequency, contain repetitive themes, or disrupt sleep, this would warrant further attention, especially to look for psychosocial triggers.
Night Terrors Night terrors involve partial arousal from non-REM sleep, usually early in the night, and peak at 4 to 7-years-old. The child will wake abruptly, screaming and appearing terrified. Then they appear to go back to sleep. Their eyes are usually open but they are not fully awake. Children can usually remember the content of the dreams, but there is no recollection of episodes of night terrors. Night terrors are usually benign and self-limiting, so no specific management is generally required.
Sleepwalking Sleepwalking occurs in older children, usually at 8 to 12-years-old, and, like night terrors, is not remembered. At the time, they will look wide awake and can often travel significant distances. Safety is an issue and stair gates or locks may be needed. It can also be associated with micturition. No specific management is usually required, although ensuring adequate sleep, reducing stress and avoiding excess stimulation before sleep may be helpful.
Learning difficulties may be idiopathic, particularly if mild, and may also be a result of neglect or lack of mental stimulation. Other potential causes are broadly similar to those which cause developmental delay, and include: ӹӹ Genetic. e.g. Down syndrome, Fragile X syndrome. ӹӹ Neonatal complications. e.g. congenital infection, hypoxic ischaemic encephalopathy, prematurity. ӹӹ Acquired illness/injury. e.g. neurovascular insult, road traffic accident, child abuse.
Investigations
Management No medical treatments are available for learning difficulties, unless they are the result of an underlying medical disorder. The emphasis is on providing greater support. The child may have an “Individual Educational Plan” formulated, where specific targets are identified realistic to their capabilities. Certain measures are then put in place to help achieve this, such as small group work, extra support in certain areas, or some one-to-one work. The child may also benefit from going to a school that specialises in providing support for those with additional learning needs. At a young age, children are often mislabelled as having behavioural problems despite their symptoms being related to the discrepancy between their functioning age and their chronological age. Without extra support for the child, consequences include poorer academic performance, anxiety, depression, behavioural problems, and unemployment and financial instability when older. This may also trigger truancy and bullying.
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SAFEGUARDING
Categories of Abuse Child protection is relevant in every aspect of paediatrics, and more generally for anyone dealing with children. According to the National Society for the Prevention of Cruelty to Children (NSPCC), in the UK approximately two children die per week from homicide, neglect or “undetermined intent”. Abuse can be divided into four categories: ӹӹ Physical abuse. ӹӹ Neglect. ӹӹ Sexual abuse. ӹӹ Emotional abuse.
COMMUNITY PAEDIATRICS
SAFEGUARDING
FIGURE 3 Head injuries tend to involve the parietal bone, occiput and forehead A
Forehead Nose Chin
Elbows
Palm of hand
These are not mutually exclusive and often co-exist.
Knees
Physical Abuse Physical abuse encompasses any physical harm caused to a child. Figure 3 shows the typical sites of accidental and non-accidental injury (NAI). Figure 4 shows specific non-accidental injuries. This may involve hitting, biting, burning or scalding, shaking, throwing, poisoning, suffocation, drowning, or any cause of physical harm to a child. It includes fabricated or induced illness. Note: ӹӹ These injuries can present as bruises, burns, lacerations, bite marks, petechiae, fractures or neurological signs suggesting intracranial injuries. ӹӹ Specific patterns of bruising are more suspicious than others: ›› Marks that are well demarcated, have the appearance of a specific implement (such as a belt), or look like finger marks from hitting or gripping. ›› Bruises that are not on bony prominences (inner/ posterior thighs, buttocks, trunk, chest, behind or on ears, neck or shoulders, intra-oral), or bilateral. ӹӹ Bruises cannot be aged accurately. ӹӹ In burns, look for splash marks and irregular edges to support a story of accidental burns. ӹӹ There should be a strong suspicion of NAI in spiral, oblique or metaphyseal fractures, but a consistent and good story could explain these, particularly in older children. ӹӹ NAI should be strongly suspected and actively excluded in any non-mobile child or child under 1-year-old with a fracture unless there is a very plausible mechanism of injury.
Shins
Ears — especially pinch marks involving both sides of the ear The ‘triangle of safety’ (ears, side of face and neck, top of shoulders) Inner aspects of arm Back and side of trunk, except directly over the bony spine
Black eyes, especially if bilateral Soft tissues of cheeks Intra-oral injuries Forearms when raised to protect self Chest and abdomen Any groin or genital injury
B Inner aspects of thighs
Soles of feet Typical sites of A) accidental injury and B) non-accidental injury.
Neglect This is the persistent failure to meet a child’s basic physiological and psychological needs and has a negative impact on their health and development. It can include: ӹӹ Poor diet. ӹӹ Meeting health needs. ӹӹ Educational achievement. ӹӹ Hygiene. ӹӹ Protection from danger and prevention of harm.
ӹӹ Protection from witnessing violence. ӹӹ Provision of emotional warmth. Sometimes, neglect is the only concern preceding a child death from abuse, and therefore the significance of neglect should not be underestimated. Every paediatrician assessing children should be alert to signs of neglect of a child’s welfare. One of the best ways to chart wellbeing is through growth parameters,
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Emotional Abuse
FIGURE 4
Bruising Petechiae in between fingers of a slap site
Cigarette burns Iron ‘implement’ mark
Classic NAI injuries: 1. Slapped cheek with space between fingers seen demarcated by petechiae. 2. Pinna bruising. 3. Cigarette burns. 4. Clear demarcation in a shape suggesting use of implement (iron).
so it is vitally important that heights and weights are recorded accurately and, where available, compared along a timeline to ensure that children are growing adequately. Failure to gain weight and to grow may well be due to failure of basic feeding and nutrition.
Sexual Abuse This involves forcing or enticing a child or young person to take part in any sexual activity regardless of whether they are aware of what is happening. This includes any physical contact, both penetrative and non-penetrative, oral sex and prostitution. It may also involve non-contact activities such as looking at or being involved in the production of pornography, being forced to watch sexual activity, or being encouraged to behave in a sexually inappropriate way. In an ever-expanding world of social media, cyber bullying and gang related crime, it is important to explore these issues in depth even if on the surface it seems consensual. There may be grooming, blackmailing or other unlawful behaviours that lead to the sexual activity. In the UK, sexual intercourse is illegal with those under 16-years-old, but with consent, and partners of similar age, power balance, and where both have capacity to consent (no learning difficulties), legal proceedings rarely occur. Any sexual intercourse with a child under 13-years-old is considered statutory rape, even if there is apparent consent. Sexual abuse may present with: ӹӹ A disclosure. ӹӹ Genitourinary symptoms. ӹӹ Unexpected pregnancy. ӹӹ Inappropriately sexualised behaviour in a young child. However, sexual abuse commonly goes unnoticed by anyone and becomes apparent later on in life, often with mental health problems and is then disclosed by the adult.
This is the persistent emotional maltreatment of a child to cause severe and persistent adverse effects to their well-being and emotional development. This may involve: ӹӹ A lack of emotional warmth and love. ӹӹ Constant criticism and being made to feel unworthy, unloved or inadequate. ӹӹ Placing developmentally inappropriate expectations upon them. ӹӹ Bullying, leading to constant fear. ӹӹ Witnessing or hearing of other maltreatment. ӹӹ Exploitation/ corruption of a child. There is some level of emotional abuse in all other categories.
Presentation of Child Abuse Concerns may be picked up through school, nurseries or presenting to a healthcare provider. The most obvious presentation of abuse is a concerning injury without a fitting explanation. The explanation may be implausible, inadequate or inconsistent and must be taken in the context of the child’s developmental age and any background medical conditions. Quite commonly, determining whether it is NAI can be unclear and very challenging. A burn mark in the shape of an iron on the buttocks is obvious. However, a child on a protection plan for neglect with a bruise on their cheek that has a reasonable but not completely obvious explanation is a much harder decision to make and pursue. Other more subtle presentations include: ӹӹ Inappropriate attachment to their caregiver, including attachment disorders. ӹӹ Inappropriate affection towards strangers. ӹӹ Poor school attendance. ӹӹ Poor presentation at school (ill-fitting or dirty clothing, unwashed, smelling of urine/faeces). ӹӹ Persistent infestations with lice. ӹӹ Persistent severe nappy rash with broken skin. ӹӹ Enuresis and/or encopresis. ӹӹ Wide ranging behavioural problems. ӹӹ Poor educational achievement. ӹӹ Early developmental delay. ӹӹ Consistently failing to present for medical appointments. ӹӹ Children can often disclose physical abuse (and occasionally sexual abuse) at nursery or school. The following situations are known to place children at increased risk of abuse: ӹӹ Domestic violence – an important and often underestimated risk. ӹӹ Mental health problems in either parent or caregiver. ӹӹ Parental drug and/or alcohol abuse. ӹӹ Previous maltreatment of other children within the family, or parents themselves. ӹӹ Disability in the child.
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SAFEGUARDING
This highlights the importance of considering safeguarding issues in every child. These more subtle presentations can suggest any category of abuse. It is important to also remember that maltreatment can occur in any setting, without any known risk factors.
Management of Abuse Initial Assessment If a child presents to the ED and there is strong suspicion of physical abuse by their primary caregiver, the child should remain in hospital as a place of safety, until it is deemed safe to return home or alternative arrangements are made. Social services should be involved early. Management of child abuse is
multidisciplinary and follows a clear pathway (Figure 5). It is important to speak alone to any verbal child (usually three to fouryears-old) and document verbatim their account, if they have one. Find out whether they feel happy and safe at home, remembering to avoid using leading questions. One increasingly common way is to ask “If you had three wishes, what would they be?” This process must maintain an open and honest two-way dialogue throughout with the parents/caregivers. Although medical conditions are ruled out simultaneously, this is not the main reason for admission so should not be conveyed as such. The parents must know that NAI is being considered and that social services and the police may be involved. The clinician can explain that it is their legal duty of care to explore this avenue further in any situation where the injury cannot conclusively be explained accidentally. Safeguarding is one of the few circumstances where confidentiality with the child or the parent may have to be broken. The child’s right to be protected from abuse overpowers the right to confidentiality.
FIGURE 5 Suspicion of abuse
Discussion with senior nurse and doctor
Full history and examination (including body map), and acute management of any injuries e.g. fractures/burns
Contact social services and enquire further
Possible police involvement
Consider admission for place of safety, acute management of injury, or further investigations
Investigation and final decision by social services Management of suspected abuse in the Emergency Department.
COMMUNITY PAEDIATRICS
ӹӹ Known maltreatment of animals by parents or carers. ӹӹ Parents who have suffered sexual abuse themselves. ӹӹ Vulnerable and/or unsupported parents, including but not limited to particularly young parents, those with learning difficulties, asylum seekers, and those with significant financial concerns.
Always remember siblings and consider their welfare.
Child Protection Medical Often injuries are noted or disclosed at school or nursery. In this scenario, social services are notified prior to the medical team. They then arrange a “child protection medical” to be carried out. A “child protection medical”, carried out by a senior paediatric doctor, involves: ӹӹ Documenting full and explicit details of the story. ӹӹ Comments on the child’s behaviour, appearance and interaction. ӹӹ A body map drawn with every mark measured and documented (this necessitates fully undressing the child). ӹӹ Details of all carers and school/ nursery noted, including full names and addresses.
Investigations During any hospital admission when NAI is suspected, a child may also have the following investigations: ӹӹ A skeletal survey where all the bones of the body are X-rayed to look for current or old fractures. ӹӹ A CT head to rule out any intracranial bleed or specific patterns of injury associated with NAI –more often in infants. ӹӹ An ophthalmology review to look for ophthalmological signs of “shaken baby” (retinal haemorrhages). ӹӹ Blood testing to check that coagulation is normal and to exclude any metabolic bone disease in a child with fractures.
Social Services Social services will gather information from all sources (for example, primary care physician, school nurse, school teachers, midwife and health visitor) and make a decision as to what is in the best interests of the child. In the UK, this can range from: ӹӹ Social services removing the child (or children) from their environment.
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ӹӹ The child being made subject to a “child protection plan” (a detailed plan identifying how the child is going to be kept safe). ӹӹ The child being made subject to a “child in need plan” (usually for vulnerable children where increased support may be needed but no immediate risk is identified). ӹӹ No further involvement.
Sexual Abuse If sexual abuse is suspected, there are specific pathways to follow. The child should not be examined by an untrained professional. This usually means that if a child presents to the ED and concerns are raised, details of anything disclosed and a general examination of the rest of the body should be documented, including assessment of any risk to immediate health (for example, bleeding after penetrative rape). The story should not be explored in detail.
Sexual Abuse Unit Once any immediate management has been provided, the child should be referred to the local sexual abuse assessment unit if it is within the last 72 hours (post-pubertal) or the last week (pre-pubertal). If the abuse is longstanding or outside this time frame, the examination is carried out by a trained community paediatrician, but does not need to be time-sensitive. At all times, it must be considered who the possible perpetrator is and whether or not home is a place of safety. The local sexual abuse assessment unit will be able to offer emergency contraception, sexually transmitted infection (STI) screening and treatment, HIV prophylaxis, hepatitis B vaccinations, forensic evidence collection, counselling and follow up appointments.
Barriers to Appropriate Safeguarding Management There are many barriers to appropriate recognition of and acting on safeguarding concerns. ӹӹ Fear of missing a treatable condition and then being criticised. ӹӹ Discomfort of medical professionals in believing parents would lie or cause harm to their child, or fear of wrongly accusing them. ӹӹ Cultural beliefs with respect to discipline. ӹӹ A desire to maintain positive professional relationships. ӹӹ Fear of complaints. ӹӹ Stress. ӹӹ Personal safety. ӹӹ Feeling that there was a justifiable reason for the action which led to maltreatment, with no actual intent to cause harm.
Appropriate Placement of a Child Decisions about how and where a child should be cared for can be extremely difficult and must take into account positive as well as negative factors at home. There may be many factors
that have led to parents maltreating their children, e.g. been in care themselves, poor parenting role models, mental health problems, and learning difficulties, but professionals must advocate for the child. In many cases, increasing support to the parents and providing education can help them provide better for their child’s needs. However, if the harm is deemed severe enough, the child/ children may need to be removed and put into foster care. These children are known as “Looked After Children” in the UK.
REFERENCES AND FURTHER READING Development 1 Bellman M, Lingam S, Aukett A. Schedule of Growing Skills II: User’s Guide. Second Edition. London: NFER Nelson Publishing Company Ltd.; 2008. 2 Gardiner M, Eisen S, Murphy C. Training in Paediatrics. Oxford Speciality Training. Oxford UK: Oxford University Press; 2009. 3 Stephenson T, Wallace H, Thompson A. Clinical Paediatrics for Postgraduate Exams. Third Edition. London: Churchill Livingstone; 2003. 4 Shribman S, Billingham K. Healthy Child Programme: Pregnancy and the first 5 years of life. Department of Health. 2009. 5 Weinstock V, Weinstock D, Kraft S. Screening for Childhood Strabismus by primary care physicians. Can Fam Physician. 1998; 44:337-43. 6 Scott O. Vision Testing and Screening in Young Children. 2012. http://patient.info/ doctor/Vision-Testing-and-Screening-in-Young -Children.htm. 7 Ansons A, Davis H. Diagnosis and Management of Ocular Motility Disorders. Fourth Edition. Oxford: Wiley Blackwell; 2014. 8 Waggoner T. New Pediatric Color Vision Test For Three to Six Year Old Pre-School Children. http://colorvisiontesting. com/color5.htm. 9 Cardiff Acuity Test. http://www.cardiff.ac.uk/optometry -vision-sciences/research/research-themes/ visual-rehabilitation/cardiff-acuity-test.
Developmental Delay 1 Shevell M, et al. Practice Parameter: Evaluation of the child with global developmental delay: Report of the quality standards subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2003; 60:367-80. 2 McDonald L, Rennie A, Tolmie J. Investigation of global developmental delay. Arch Dis Child. 2006; 91:701-5. 3 Cleary M, Green A. Developmental delay: When to suspect and how to investigate for an inborn error of metabolism. Arch Dis Child. 2005; 90:1128-32.
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REFERENCES AND FURTHER READING
1 Kim S. Recent update of autism spectrum disorders. Korean J Pediatr. 2015;58; 8-14. 2 Rutter M. Incidence of autistic spectrum disorders: changes over time, and their meaning. Acta Paediatr. 2005; 94:2-15. 3 NICE Guidelines. CG128. Autism diagnosis in children and young people: Recognition, referral and diagnosis of children and young people on the autism spectrum. 2011. https://www.nice.org.uk/guidance/cg128. 4 NHS Immunisation Information. MMR The Facts. Department of Health Publications. 2004. 5 Dyer C. Lancet retracts Wakefield’s MMR paper. BMJ. 2010; 340:c696.
Behavioural Problems 1 NICE Guidelines. CG72. Attention Deficit Hyperactivity Disorder: Diagnosis and management of ADHD in children, young people and adults. 2008. https://www.nice. org.uk/guidance/cg72. 2 McCann D, Barrett A, Cooper A, et al. Food additives and hyperactive behaviour in 3 year olds and 8/9 year olds in the community: a randomised double blinded placebocontrolled trial. Lancet. 2007; 370:1560-7.
Safeguarding
COMMUNITY PAEDIATRICS
Autistic Spectrum Disorder
2 Department for Education. Statutory Guidance: Working together to Safeguard Children. DfE;Ref:DFE-00130-2015; 2015. 3 Maguire S, Mann M. Systematic reviews of bruising in relation to child abuse – what have we learnt: an overview of review updates. Evid. Based Child Health. 2013; 8:255-63. 4 Maguire S, Moynihan S, Mann, M et al. A systematic review of the features that indicate intentional scalds in children. Burns. 2008; 34:1072-81. 5 Maguire S, Cowley L, Mann M, Kemp A. What does the recent literature add to the identification and investigation of fractures in child abuse: an overview of review updates 2005-2013. Evid. Based Child Health. 2013; 8:2044-57. 6 Naughton A, et al. Emotional, behavioural and developmental features indicative of neglect or emotional abuse in preschool children: a systematic review. JAMA Pediatr. 2013; 167:769-75. 7 Core Info – Cardiff Child Protection Systematic Reviews: www.core-info.cardiff.ac.uk. 8 NSPCC. Child Protection Fact Sheet – The definitions and signs of child abuse. NSPCC Inform. 2009. 9 Jutte S, Bentley H, Miller P, Jetha N. How safe are our children? London: NSPCC. 2014.
1 NICE Guidelines. CG89. When to Suspect Child Maltreatment. 2009. https://www.nice.org.uk/ guidance/cg89.
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1.05 ENT
ANDREW HALL
CONTENTS 51 Ear Pathology
51 Hearing Loss 51 Otitis Media 51 Mastoiditis 52 Chronic Otitis Media with Effusion (OME)
52 Nasal Pathology 52 Rhinosinusitis 52 Nasal Polyps 52 Epistaxis
53 Upper Airway Obstruction 53 Stertor and Stridor 53 Airway Foreign Body 53 Choking
55 Infection
55 Periorbital and Orbital Cellulitis 55 Tonsillitis 56 Peritonsillar Abscess (Quinsy) 56 Infectious Mononucleosis (Glandular Fever) 56 Croup 56 Acute Epiglottitis 57 Bacterial Tracheitis (Pseudomembranous Croup)
57 Paediatric Neck Masses 58 References and Further Reading
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Ear Pathology
EAR PATHOLOGY
Investigations
Hearing Loss
Aetiology Hearing loss is broadly broken down into conductive, sensorineural or mixed: ӹӹ Conductive hearing loss (CHL). Sound waves are unable to pass from the opening of the external auditory canal to the inner ear structures. The underlying pathology can involve the external ear canal, tympanic membrane, middle ear or ossicles. ӹӹ Sensorineural hearing loss (SNHL). This affects the vestibulocochlear nerve, inner ear structures (cochlea) or the brain itself. There is either a failure in the conversion of acoustic energy received by the cochlea or a failure of neural transmission to the central nervous system. ӹӹ Mixed hearing loss. This involves concurrent conductive and sensorineural pathology. Causes of hearing loss are summarised in Table 1. Most children with severe congenital hearing loss have a mutation in the Connexin 26 protein on the GJB2 gene. In addition, some form of hearing loss is found in 75% of all children with Down syndrome. This could be due to developmental abnormality of the inner ear or recurrent otitis media with effusion (OME).
Management Management depends on both the underlying cause and the severity of hearing loss. Formal paediatric audiological input may lead to consideration of cochlear implantation in individuals with profound sensorineural hearing loss who do not benefit from a three month trial of hearing aids.
Otitis Media Acute middle ear infections are common in the paediatric population, particularly in those 6-months-old to 2–years-old.
Aetiology The paediatric Eustachian tube is shorter, wider and more horizontal than in adults, making it easier for infection to spread up the tube. Viral infections are more common, but they are difficult to accurately distinguish from bacterial infections on symptoms alone. Bacterial causes include Streptococcus pneumoniae, Haemophilus influenzae and Moraxella catarrhalis, whilst viral causes include respiratory syncytial virus, rhinovirus, adenovirus and parainfluenza virus.
Clinical Features Symptoms include: ӹӹ Otalgia. Children who are unable to vocalise pain may be seen tugging on their affected ear.
TABLE 1: Causes of hearing loss
Congenital.
Acquired.
Conductive (CHL)
Sensorineural (SNHL)
• Down syndrome. • Craniofacial abnormalities e.g. Treacher Collins syndrome.
• Genetic, e.g. Alport syndrome (also associated with nephritis) or Connexin 26 mutation. • Prenatal infection, e.g. toxoplasmosis, rubella, CMV, syphilis.
• Otitis media with effusion. • Wax deposition.
• Perinatal, e.g. birth trauma, hypoxia. • Meningitis. • Ototoxicity, e.g. Gentamycin/Cisplatin.
A buildup of pus behind the tympanic membrane may lead to spontaneous rupture (perforation) that may discharge blood and fluid. This rupture may be associated with a relief of pain. On examination with an otoscope, a red, bulging tympanic membrane or perforation of the tympanic membrane may be evident.
ENT
Early detection and intervention of hearing loss allows maximisation of future language and intellectual development. About one to two children in 1000 have at least moderate permanent hearing loss.
Hearing loss is identified through screening tests, as detailed on p39. It may also be specifically screened when hearing loss is suspected, such as in developmental delay or an accidental ingestion of a toxic dose of gentamicin.
ӹӹ Pyrexia. ӹӹ Systemic illness.
Management Management is based on symptomatic relief. Pain levels should be assessed, and if required, simple analgesia should be prescribed. This is usually all that is required, as most infections are viral and self-limiting. Antibiotic prescribing patterns vary between clinicians. Antibiotic therapy is generally warranted in patients with bilateral otitis media, patients that are systemically unwell, neonates/the very young, and those with persistent non-resolving infection. Penicillins, e.g. amoxicillin, are commonly used.
Complications ӹӹ Tympanic membrane perforation. This may occur secondary to the buildup of pus behind the eardrum, resulting in rupture. This may be associated with a conductive hearing loss and risk of future infections. ӹӹ Chronic otitis media with effusion. ӹӹ Labrynthitis. Infection of the inner ear resulting in vertigo. ӹӹ Mastoiditis. See below. Rarely, intracranial complications are seen from spread of infection, such as abscess formation or meningitis.
Prognosis Full recovery is usually expected, with no long-term sequelae.
Mastoiditis Mastoiditis is a result of infection of the mastoid process and is treated aggressively due to potential intracranial spread of infection.
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Clinical Features Mastoiditis is most commonly seen as a complication of otitis media. It presents with: ӹӹ Significant systemic malaise. ӹӹ Fever. ӹӹ A red, hot, tender swelling over the mastoid.
ear canals can make grommets more difficult to insert and subsequently monitor.
Complications
The pinna itself may protrude anteriorly due to these processes.
Cholesteatoma is a potential complication of untreated OME. This abnormal collection of skin cells grows and expands within the middle ear and mastoid process, potentially damaging the ossicles. If identified, it is treated with surgery.
Management
Prognosis
This condition warrants urgent Ear, Nose and Throat (ENT) review for assessment. Initial treatment is with broad spectrum intravenous antibiotics, but incision and drainage of the mastoid may be required if an abscess has formed. A CT scan may be organised if there is a concern about a possible intracranial abscess or if the child fails to improve with initial intravenous broad-spectrum antibiotics.
OME resolves spontaneously in 90% of children within one year.
Chronic Otitis Media with Effusion (OME) This is a painless, chronic inflammation of the middle ear mucosa leading to the buildup of mucus behind the tympanic membrane. It is therefore also called “glue ear”. It has a bimodal age incidence, peaking at two and five-years-old.
Aetiology OME may be caused by infection (acute otitis media is an important risk factor), or fluid may accumulate in the middle ear secondary to Eustachian tube obstruction or dysfunction. It is the most common cause of hearing loss in children.
NASAL PATHOLOGY Rhinosinusitis Rhinitis (inflammation of the nasal lining), rhinosinusitis (inflammation of the nose and paranasal sinuses) and sinusitis (inflammation of the sinuses) represent a spectrum of disease that can be acute or chronic. A majority of paediatric nasal complaints are related to symptoms of nasal obstruction, rhinorrhoea (runny nose), sneezing and snoring. An important potential complication of acute rhinosinusitis is periorbital cellulitis. Most infective cases of rhinosinusitis are viral and self-limiting, whilst allergic causes usually respond to simple antihistamines.
Nasal Polyps
Symptoms may include school or parental recognition of hearing loss that may be associated with speech and behavioural problems. Intermittent otalgia can be a feature.
Nasal polyps are rarely found on clinical examination in children, and if present, cystic fibrosis and primary ciliary dyskinesia should be investigated. Most commonly, a diagnosis of nasal polyposis is made in error due to failure to recognise the normal anatomy of the inferior turbinate which can be particularly bulky in children.
Investigations
Epistaxis
Evaluation of hearing (audiometry), eardrum compliance (tympanometry) and an assessment of the ear (otoscopy) allow the diagnosis to be made. On otoscopy, there may be a dull appearance to the eardrum, with a fluid level sometimes visible behind the tympanic membrane.
Nosebleeds are commonly encountered in childhood affecting between one-third and two-thirds of all children at some time. It is important to recognise that, in patients less than two-yearsold, non-accidental injury is a possible cause.
Management
Paediatric epistaxis is generally idiopathic and can be managed largely in the outpatient setting. There is generally no underlying systemic vascular or haematological abnormality. Despite this, it is important to take a thorough bleeding history of these patients to assess whether the haematology team needs to be involved in their investigation and management. Topical antiseptic cream in the form of bactroban or naseptin is used as first line therapy. If unsuccessful, nasal cautery of blood vessels can be performed.
Clinical Features
Intervention is recommended in the presence of: ӹӹ Significant symptoms. ӹӹ If there is a persistent hearing loss of 25 dB or more in the affected ear for three months. Insertion of ventilation tubes (i.e. grommets) improves hearing in children with OME for up to 12 months after surgery. A grommet is a hollow tube that punctures the tympanic membrane, and remains in place to prevent the buildup of fluid behind the ear. Another treatment option is hearing aids. This is preferred in children with Down syndrome, where narrow
Management
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Upper Airway Obstruction
UPPER AIRWAY OBSTRUCTION Stertor and Stridor
Stertor This is partial obstruction of the airway with low-pitched snoring produced by vibrations above the level of larynx. Table 2 shows the differential diagnosis. Chronic stertor is often caused by adenoid and tonsillar hypertrophy and may indicate the presence of obstructive sleep apnoea. This requires referral to ENT surgeons for consideration of adenotonsillectomy. It is also important to inquire regarding nasal symptoms as to the possibility of a nasal foreign body.
Stridor This is an audible change in airflow at the level of larynx, trachea or bronchi. Inspiratory stridor indicates turbulent airflow above the vocal cords. Expiratory stridor indicates turbulent airflow in the trachea or bronchi (comparable with wheeze). Biphasic stridor has an inspiratory and expiratory component and is related to pathology located at the level of the vocal cords or subglottis (just beneath the vocal cords).
Airway Foreign Body Airway foreign bodies are commonly encountered in children.
Nasal Foreign Body When identified in the nose, the potential for migration further into the airway necessitates early removal, under anaesthetic if required. A foreign body can usually be removed using simple forceps or by making a seal over the lips, occluding the unaffected nostril, and blowing in the mouth (often done by the mother). This is known as the “kissing technique” or “parent’s kiss” (Figure 1). Unilateral, offensive nasal discharge may be a presenting symptom of a chronic nasal foreign body.
more commonly occurs in the right main bronchus. A chest X-ray (both inspiratory and expiratory films) allows assessment of the foreign body itself and of any associated airway collapse or air trapping-associated obstruction. In chronic obstruction, there may be a secondary infection. Bronchoscopic airway evaluation and removal may be required.
ENT
Paediatric airway obstruction can range from noisy breathing in an otherwise well child to a respiratory arrest. Identification of the approximate level of obstruction from the nose to the bronchi may be achieved by recognising the distinction between stertor and stridor. Upper airway obstruction is a medical emergency that can evolve quickly and is best managed by a multidisciplinary team involving paediatricians, ENT surgeons and anaesthetists.
The differential diagnosis for acute stridor includes croup, epiglottitis, bacterial tracheitis, anaphylaxis and foreign bodies. These will be explored in detail in this chapter. Table 3 summarises the common causes of chronic stridor. Differentiating between acute and chronic causes of paediatric stridor on history and examination alone can be difficult. Where there is diagnostic uncertainty or clinical concern, a referral to the ENT team is appropriate for consideration of microlaryngobronchoscopy to evaluate the airway.
Choking This is usually witnessed in preschool children with a sudden attack of stridor and respiratory distress, secondary to food or foreign body obstruction.
Effective Cough A conscious child with an effective cough (audible cry/speech, loud cough) should be regularly observed and encouraged to cough.
Ineffective Cough If the cough is ineffective (inaudible cry/ speech, unable to breathe, cyanotic), assistance is required. Five back blows FIGURE 1
Distal Foreign Body A child with a history of coughing or choking on feeding should be carefully evaluated for an airway foreign body. Symptoms are dependent on location and can range from audible stridor and respiratory distress to an asymptomatic child. The bronchus is a worrying site for lodging of an inhaled foreign body, and in older children, this
TABLE 2: Differential diagnosis for stertor STERTOR
Acute
Chronic
Obstruction at level of nose/ pharynx.
Tonsillitis.
Enlarged adenoids/tonsils.
Peritonsillar abscess.
Nasal foreign body.
Infectious mononucleosis. Nasal foreign body.
Kissing technique for foreign body removal from nostril. A good seal is made around the mouth, the other nostril is occluded and the parent blows forcefully into the patient’s mouth.
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• From birth, usually resolved by 2-yearsold.
• Stridor worse on feeding/crying.
• Prematurity. • Neuromuscular disorders.
• Aryepiglottic folds “indraw” on inspiration.
• Not required in 90% of cases. • Anti-reflux. • If faltering growthsurgical evaluation.
Age group affected.
Presentation.
Risk factors.
Findings.
Treatment.
Laryngomalacia
TABLE 3: Causes of chronic stridor
• Typically 2-4 months of age.
Subglottic haemangioma
• Varies from observation to ballooning of the stenosis or laryngotracheal reconstruction.
• Narrow subglottis.
• Previous intubation. • Down syndrome.
• Propranolol. • May have adjuvant laser debulking to reduce the size.
• Haemangioma in subglottis.
• Cutaneous haemangiomas.
• Recurrent episodes of • Progressively stridor. worsening stridor. • Poor exercise tolerance. • Barking cough.
• Any age.
Subglottic Stenosis
• May be managed conservatively but if severe surgery can be performed (aortopexy) to relieve pressure on trachea.
• Tracheal compression, e.g. from thymoma.
• Prematurity — congenital deformity of tracheal rings. • Extrinsic pressure — neighbouring blood vessels. • Previous repair of oesophageal atresia.
• Cyanotic episodes.
• First year of life.
Tracheomalacia
• Stridor. • Wheeze. • Difficulty breathing. • Removal of foreign body- in ED or under general anaesthetic.
• Conservative.
• Learning difficulties. • Parental neglect.
• Recurrent chest infection (although significant obstruction presents acutely, with breathlessness and stridor).
• More likely in younger children.
Chronic obstruction with foreign body
• Vocal cord in intermediate position.
• Intra-thoracic surgery/ pathology.
• Altered cry. • Rarely sole cause of stridor.
• Any.
Vocal Cord Palsy
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Infection
FIGURE 2
FIGURE 3
FIGURE 4
ENT
Back blow. The infant is placed over the leg to allow gravity to assist. The centre of the infant’s back is struck firmly with the palm of the hand five times. In an older child, this is done keeping them upright, but bending them forward at the waist.
(Figure 2) should be delivered, followed by five abdominal thrusts in children more than one–year-old (Figure 3), or five chest thrusts in children less than 1– year-old (Figure 4). The patient should be reassessed after each intervention, and the process should be repeated as necessary.
Unconscious Child In a very severe obstruction, the child may become unconscious. In this case, attempt to remove anything seen in the mouth with one finger sweep, and then follow the paediatric basic life support, giving rescue breaths.
INFECTION Periorbital and Orbital Cellulitis Periorbital Cellulitis Periorbital cellulitis is infection and inflammation of the skin and eyelid anterior to the orbital septum (unlike orbital cellulitis). This condition can be secondary to a sinus infection, but can also be due to a minor scratch or insect bite. In its most minor form, there is mild eyelid swelling without any systemic symptoms.
Orbital Cellulitis Progress of periorbital cellulitis can lead to intra-orbital spread of infection
Abdominal thrust. Previously called the Heimlich manoeuvre. The person performing the manoeuvre makes a fist with one hand, and grabs it with the other. They then rapidly pull back and upwards through the abdomen to deliver each thrust.
Chest thrusts. This has a similar technique to chest compressions. Two fingers are placed at the base of the sternum. Five thrusts are then delivered, compressing downward about 1.5 inches.
causing orbital cellulitis, with red flag signs of proptosis (bulging of the eye), limitation of eye movement and chemosis (conjunctival swelling). Colour vision and visual acuity are important to document, as these are sensitive features of optic nerve inflammation. A paramount concern is the potential for optic nerve injury and permanent loss of sight. Intracranial spread of the infection can occur on rare occasions.
lymphadenopathy, high fever and tonsillar exudates. Classically, bacterial infection is with group A streptococcus.
Management
On examination, the tonsils are red and enlarged with possible tonsillar exudate (Figure 5). Cervical lymph nodes may also be enlarged and tender. Simple analgesia, antipyretics and adequate
This condition is best managed with a multi-disciplinary approach involving paediatric, ENT, maxillofacial and ophthalmology teams. Minor cases of periorbital cellulitis usually settle with oral antibiotics. Moderate cases need intravenous therapy. If concerns exist of intra-orbital infection, a computed tomography (CT) scan of the brain/orbit needs to be arranged urgently. Surgical intervention may require either an external or endoscopic approach to drain the pus, depending on the extent and position of the pathology.
Clinical Features Clinical features include: ӹӹ Sore throat. ӹӹ Pain on swallowing (odynophagia). ӹӹ Fever. ӹӹ Headache. ӹӹ Reduced oral intake.
FIGURE 5
Tonsillitis Aetiology Tonsillitis involves inflammation of the tonsils. It may be viral or bacterial in origin. Bacterial causes are more likely in the absence of cough, tender cervical
Bilaterally enlarged tonsils with exudate.
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hydration are required. Antibiotics are not routinely indicated, as the majority of cases are viral.
Management If group A streptococcus is suspected or found on a throat swab, a ten day course of penicillin V should be prescribed. Rarely, severe swelling may precipitate stertor and dysphagia. In such cases, IV antibiotics (e.g. benzylpenicillin) are warranted, and IV fluids may be required for maintenance of hydration. Corticosteroids may be initiated to reduce mucosal oedema and reduce both the stertor and pain associated with swallowing. Tonsillectomy should be considered if the episodes are recurrent (i.e. five or more cases a year), and the episodes are disruptive to everyday functioning.
Peritonsillar Abscess (Quinsy) Clinical Features The patient classically appears toxic with a high temperature and may suffer from trismus (inability to open the mouth), making assessment very difficult. On speaking, patients characteristically have “hot potato voice”, where their vowels are distorted. Due to difficulty swallowing, drooling may also be present. Other features include lethargy, pain on swallowing, and malaise. If there is airway obstruction, cyanosis/breathing abnormalities may also occur. The affected tonsil, soft palate and uvula are pushed towards the centre of the mouth.
bacterial infection. Amoxicillin and ampicillin should not be prescribed as they can cause a generalised, itchy, salmon pink rash. Steroids may be considered in the event of dysphagia to reduce inflammation.
Croup Croup (acute laryngotracheobronchitis) occurs as a result of a viral infection, most commonly parainfluenza and influenza virus in children under the age of two.
Clinical Features Classically, croup presents with a barking cough, which can particularly distress parents. Symptoms are more severe at night, but the onset is insidious over several days with an initial viral coryzal prodrome. Mucosal inflammation results in airway narrowing and increased secretions within the larynx, trachea and bronchi.
Management Treatment of croup is based on severity (Table 4). Most croup is mild and managed in primary care or the ED. Moderate cases require corticosteroid treatment to reduce airway inflammation. In more severe cases, with significant airway narrowing, adrenaline and supplemental oxygen may be given. In the worst cases, intubation and ventilation or an emergency surgical airway may be required.
Acute Epiglottitis
Management
Aetiology
Quinsy is the most common ENT emergency. Treatment typically includes aspiration/drainage of the abscess by the ENT team and intravenous antibiotics.
Infectious Mononucleosis (Glandular Fever)
This condition describes acute severe swelling of the epiglottis and aryepiglottic folds secondary to a Haemophilus influenzae type b (Hib) bacterial infection. This is now very rare due to widespread vaccination. It mainly affects children three to fiveyears-old and can progress rapidly over several hours.
Infectious mononucleosis (glandular fever) can affect children of any age, but is more common in teenagers.
Clinical Features
Aetiology It is caused by the Epstein-Barr virus (EBV). It is sometimes called “kissing disease” as the virus can spread by droplet infection and direct contact.
Clinical Features Patients present with generalised malaise, enlarged exudative tonsils, cervical lymphadenopathy, fever and hepatosplenomegaly.
Investigations To confirm infectious mononucleosis, a Monospot test or EBV serology can be performed.
Management Treatment is largely supportive, with fluids and analgesia, but antibiotics may be recommended to treat any superadded
Epiglottitis is usually rapid in onset, with severe symptoms, including high fever, drooling/dysphagia, and significant distress. There may be stridor, laboured breathing and a muffled/quiet voice.
Management Never do anything to upset a child with epiglottitis as this may precipitate full airway obstruction. This includes examining their throat or ears, taking blood or inserting a cannula (without an anaesthetist being present). Treatment of acute epiglottitis involves protecting the airway as the first priority. This may require intubation or even a surgical airway if there is significant swelling. Broad spectrum antibiotics (e.g. a third generation cephalosporin) should be promptly started, and steroids or adrenaline nebulisers may be needed. Table 5 summarises key differences between croup and acute epiglottitis.
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Paediatric Neck Masses
TABLE 4: Croup assessment (Modified Westley Clinical Scoring System)
Epiglottitis
Voice.
Hoarse.
Muffled/quiet.
1
Coryzal.
Yes.
No.
At rest.
2
Fever.
38.5°C.
Mild.
1
Cough.
Barking, prominent feature.
Moderate.
2
Slight, not prominent feature.
Severe.
3
Appearance.
Well.
Toxic.
Normal.
0
Drooling.
Unlikely.
Yes, very painful to swallow.
Mildly decreased.
1
Treatment.
Severely decreased.
2
None.
0
With agitation/activity.
4
Dependent on severity: Mild: no specific treatment. Moderate: oral steroids. Severe: may require adrenaline, oxygen, intubation, surgical airway.
At rest.
5
Normal.
0
Altered.
5
Steroids, adrenaline nebulisers and IV antibiotics – usually ceftriaxone. Urgent involvement of senior anaesthetist and ENT surgeons with early intubation under general anaesthetic. Occasionally urgent tracheostomy is the only way to secure the airway.
Degree
Points awarded
Inspiratory stridor.
Not present.
0
When agitated/active.
Air entry.
Cyanosis.
Level of consciousness.
Total score 6=severe croup
Bacterial Tracheitis (Pseudomembranous Croup)
ENT
Croup (acute laryngotracheobronchitis)
Symptom/Sign
Intercostal recession.
TABLE 5: Comparison of croup and acute epiglottitis
FIGURE 6
This is caused by Staphylococcus aureus infection and presents with similar clinical features to acute epiglottitis. Bacterial invasion of the trachea results in pseudomembrane formation with purulent secretions unresponsive to conventional management. These patients require broad spectrum antibiotics and may require prolonged intubation, with endoscopic removal of the secretions.
PAEDIATRIC NECK MASSES Most cervical lymphadenopathy in paediatrics is non-malignant. Utilising a structured method of clinical assessment ensures that the correct diagnosis is made whilst rationalising the number of investigations required. Red flags for urgent senior help/ENT referral include: ӹӹ Sepsis. ӹӹ Unwell child. ӹӹ Stridor/airway compromise. ӹӹ Difficulty swallowing. ӹӹ Change in voice quality. ӹӹ Rapid progression.
Cystic hygroma in a newborn baby.
There are a wide variety of causes for palpable neck masses in paediatrics. Key differential diagnoses to consider and subsequent investigations are summarised in Table 6. Neck lumps can be very large as with the example of a cystic hygroma in Figure 6.
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TABLE 6: Causes of paediatric neck lumps Diagnosis
Description
Clinical Features
Key Investigations
Management
Lymphadenitis (Reactive lymph node swelling)
Infective.
• Generalised cervical lymphadenopathy. • Upper respiratory tract infection.
• Elevated white blood cell count secondary to acute infection.
• Consideration of antibiotics, and surgical drainage of any abscess.
Infectious Mononucleosis (Glandular fever)
Infective.
• Teenagers. • Large, tender, bilateral lymphadenopathy. • Sore throat and malaise. • May be associated hepatosplenomegaly and axillary lymphadenopathy.
• Elevated neutrophils secondary to acute infection. • Positive Monospot. • Liver/splenic USS may show hepatitis/splenomegaly.
• Rehydration and supportive treatment.
Mycobacterium tuberculosis
Infective.
• • • •
• Mantoux test. • CXR: apical consolidation/ cavitation/nodules.
• TB treatment. • Avoid surgical excision as it can result in chronic infection.
Atypical Mycobacterium
Infective.
• Classically occurs at 2 to 5-yearsold, likely ingested from soil. • Bright red, enlarged lymph node.
• Mantoux test. • Avoid biopsy as can lead to chronic sinus — remove whole node.
• Spontaneous resolution in most cases but scarring prominent. • Surgical excision of whole node.
Branchial cyst
Congenital.
• Teenagers and young adults. • Located at anterior border of the sternocleidomastoid at the junction of the upper and middle thirds.
• USS will identify a cystic mass.
• Antibiotics to treat any acute infection. • Excision under general anaesthetic.
Thyroglossal cyst
Congenital.
• 90% of midline neck swellings in children. • Embryologically derived from the thyroid gland which descends from the tongue in utero. • Failed atrophy of tract- lump moves on tongue protrusion.
• USS visualises tract and establishes presence of underlying thyroid gland.
• Antibiotics to treat any acute infection. • Excision if recurrent infections.
Lymphatic malformation (cystic hygroma)
Vascular.
• Rare, sponge-like lesions in neonatal period or early infancy. • They can fluctuate in size due to infection and some cause facial disfigurement and airway obstruction.
• USS demonstrates underlying anatomy.
• May need tracheostomy if airway compromised although rare. • Sclerotherapy and possible surgical excision.
Malignancy
Neoplastic.
• Systemic symptoms — fever, weight loss, night sweats. • Often isolated, firm, enlarging, painless lymph node swelling. • Axillary/inguinal lymphadenopathy. • Hepatosplenomegaly.
• Anaemia/Immature blast cells on blood film. Normal blood film does not exclude diagnosis. • Excision biopsy to formally exclude if suspected.
• Dependent on diagnosis. • May include surgery, chemotherapy or radiotherapy.
Painless neck lump. Can develop discharge/abscess. Sinus formation. Associated fever/history of TB risk factors.
REFERENCES AND FURTHER READING Hearing Loss 1 Wilson C, Roberts A, Stephens D. Aetiological investigation of sensorineural hearing loss in children. Arch Dis Child. 2005;90:307-9.
Otitis Media 1 Coker TR et al. Diagnosis, microbial epidemiology, and antibiotic treatment of acute otitis media in children: a systematic review. JAMA. 2010; 304:2161-9.
2 Wald ER. To treat or not to treat. Pediatrics. 2005; 115:1087-89.
Otitis Media with Effusion 1 Browning GG et al. Grommets (ventilation tubes) for hearing loss associated with otitis media with effusion in children. Cochrane Database Syst Rev. 2010 6; CD001801. 2 NICE guideline CG60. Otitis media with effusion in under 12s. http://www.nice.org.uk/guidance/cg60 /resources.
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References and Further Reading
Tonsillitis
1 Cohen-Kerem R et al. Acute mastoiditis in children: is surgical treatment necessary? J Laryngol Otol. 1999; 113:1081.
1 Burton MJ, Glasziou PP. Tonsillectomy or adenotonsillectomy versus non-surgical treatment for chronic/ recurrent acute tonsillitis. Evid Based Child Health. 2009; 4:1291. 2 Wilson JA et al. Tonsillectomy: a cost-effective option for childhood sore throat? Further analysis of a randomized controlled trial. Otolaryngol Head Neck Surg. 2011; 146:122-8.
Rhinosinusitis 1 Jones NS. Current concepts in the management of paediatric rhinosinusitis. J Laryngol Otol. 1999 Jan; 113:1-9.
Nasal Polyps 1 Gysin C, Alothman GA, Papsin BC Sinonasal disease in cystic fibrosis: clinical characteristics, diagnosis, and management. Paediatr Pulmonol. 2000; 30:481ysti.
Epistaxis 1 McGarry G. Nosebleeds in children. Clin Evid. 2006;15:496-9.
Periorbital Cellulitis 1 Baring DE, Hilmi OJ. An evidence based review of periorbital cellulitis. Clin Otolaryngol. 2011; 36:57-64.
Stertor and Stridor 1 Kuo M, Rothera M. Emergency Management of the Paediatric Airway. In: Graham JM, Scadding GK, Bull PD. Paediatric ENT First Edition. Berlin: Springer-Verlag; 2007. 183-7.
Airway Foreign Body 1 Rodray Fo, H., Passali, GC, Gregori D., et al, Management of foreign bodies in the airway and oesophagus. Int. J. Pediatr. Otorhinolaryngol. 2012;76:84. 2 Benjamin E. Harcourt J. The modified ‘Parent’s Kiss’ for the removal of paediatric nasal foreign bodies. Clin Otolaryngol. 2007; 32:120-1.
ENT
Mastoiditis
Peritonsillar Abscess 1 Hsiao HJ, Huang YC, Hsia SH, Wu CT, Lin J.J. Clinical features of peritonsillar abscess in children. Pediatr Neonatol. 2012; 53:366-70.
Glandular Fever 1 Luzuriaga K, Sullivan JL. Infectious mononucleosis N Engl J Med. 2010; 362:1993-2000.
Croup 1 Bjornson C, Russell K, Foisy M, Johnson DW. The Cochrane Library and the treatment of croup in children: an overview of reviews Evid Based Child Health. 2010; 5: 1555d.l
Acute Epiglottitis 1 Acevedo JL, Lander L, Choi S, Shah RK. Airway management in pediatric epiglottitis: a national perspective. Otolaryngol Head Neck Surg. 2009 140(4):548-51.
Bacterial Tracheitis 1 Al-Mutairi B, Kirk V. Bacterial tracheitis in children: Approach to diagnosis and treatment. Paediatr Child Health. 2004; 9: 25.
Choking
Neck Masses
1 ALSG Paediatric Choking Algorithm. 2015. https:// www.resus.org.uk/EasySiteWeb/GatewayLink. aspx?alId=6458
1 Hartley B. Head and Neck Masses. In: Graham JM, Scadding GK, Bull PD. Paediatric ENT First Edition. Berlin: Springer-Verlag; 2007. 111-22.
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1.06
ENDOCRINOLOGY ZESHAN QURESHI, VAITSA TZIAFERI, SADHANANDHAM PUNNIYAKODI, MARYLYNJANE EMEDO AND CLAIRE BRYANT
GLUCOSE REGULATION Type 1 Diabetes Mellitus
CONTENTS 60 Glucose Regulation
60 Type 1 Diabetes Mellitus 64 Diabetic Ketoacidosis 66 Other Types of Diabetes in Childhood 66 Hypoglycaemia
68 Growth
68 Assessment of Abnormal Stature 69 Short Stature 72 Tall Stature 74 Puberty 75 Precocious Puberty 77 Delayed Puberty
Diabetes mellitus (DM) is a condition of elevated plasma glucose. There are two main types: ӹӹ Type 1 diabetes (T1DM). An absolute deficiency of the pancreatic hormone insulin. ӹӹ Type 2 diabetes (T2DM). Failure to appropriately make use of insulin to metabolise glucose. The vast majority of cases in childhood are T1DM, although with the increasing obesity rates, T2DM is becoming more common. The World Health Organisation (WHO) has formulated a set of diagnostic criteria based on blood glucose measurements, dependent on the presence or absence of typical symptoms of the disorder (Figure 1).
Aetiology T1DM is characterised by autoimmune T-cell mediated damage to the β islet cells of the pancreas. The β cells are responsible for insulin production and their destruction leads to insulin deficiency. Normally, insulin acts to reduce blood glucose through stimulating glucose uptake from the blood,
78 Disorders of Sexual Development 80 Adrenal Disorders 80 Adrenal Insufficiency 81 Congenital Adrenal Hyperplasia 83 Cushing Syndrome
84 Thyroid Disorders
FPG> 7.0mmol/L OR Symptomatic
Diagnosis of diabetes (WHO criteria)
84 Hypothyroidism 86 Hyperthyroidism
88 Sodium Disorders 88 Hyponatraemia 88 Hypernatraemia
90 References and Further Reading
2 FPGs > 7.0mmol/L OR 2 random PG > 11.1mmol/L OR Asymptomatic
1 of each of the above OR OGTT-PG>11.1mmol/L 2 hours post ingestion of 75g glucose
86 Calcium Disorders 86 Hypocalcaemia 88 Hypercalcaemia
random PG> 11.1mmol/L
FIGURE 1
HbA1C > 48 mmol/mol (6.5%)
WHO criteria for diagnosis of Type 1 diabetes (FPG=Fasting Plasma Glucose, PG= Plasma Glucose, OGTT=Oral Glucose Tolerance Test). Adapted from “Definition and diagnosis of diabetes mellitus and intermediate hyperglycaemia”: Report of a WHO/IDF consultation.
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ӹӹ Genetic susceptibility. Genetic factors are suggested by the familial nature of DM. Concordance between identical twins has been found to be 30-50%. Those with a first degree relative with diabetes have an approximately six percent risk of developing the disorder compared to the less than one percent background risk. The HLA-DR3 and HLA-DR4 regions of the genome are associated with an increased risk of developing T1DM. HLA-DR2 and HLA-DR5 appear to be protective. ӹӹ Environmental factors. Studies have indicated that exposure to certain viruses (e.g. enteroviruses, cytomegalovirus [CMV] and mumps), early cow’s milk consumption and other dietary factors (e.g. early gluten exposure) may act as triggers in
Inhibition of processes that raise plasma glucose
Stimulation of processes that lower plasma glucose
• Reduced gluconeogenesis (production of glucose from non-glucose substrates) primarily in the liver.
• Increased glucose uptake into fat/ muscle cells.
• Reduced glycogenolysis (breakdown of glycogen to glucose).
• Increased conversion of glucose to glycogen in the liver and muscles.
High blood glucose Raises blood glucose
Glycogen
Promotes insulin release
Glucagon
Glucose Pancreas
Stimulates glycogen formation
Liver
Insulin Stimulates glucose uptake from blood Lowers blood glucose FIGURE 2
Clinical Features Often children are diagnosed with DM following a short history (weeks) of the classic symptoms and signs. A formal oral glucose tolerance test (OGTT) is rarely required for T1DM. Some children may have a first presentation of DM with diabetic ketoacidosis (DKA) and may present extremely unwell. The 4Ts Mnemonic may help remember the classical signs of diabetes: Toilet, Thirsty, Thinner, Tired.
Classic Signs ӹӹ Toilet. Polyuria (+/- secondary enuresis). ӹӹ Thirsty. Polydipsia. ӹӹ Thinner. Weight Loss. ӹӹ Tired. Fatigue.
Additional Signs
TABLE 1: Actions of insulin on glucose regulation
Stimulates glycogen breakdown
children with an underlying genetic predisposition.
Endocrinology
stimulating glycolysis, reducing liver gluconeogenesis and glycogenolysis. Table 1 summarises the role of insulin in glucose regulation. Note that insulin also stimulates triglyceride production from blood lipids and reduces lipolysis. Glucagon is the main counter regulatory hormone to insulin. Its predominant role is to raise blood glucose by stimulating glycogen breakdown in the liver. The interaction of the two hormones is summarised in Figure 2. The insulin deficiency of T1DM leads to elevated glucose concentration in the blood (hyperglycaemia). The cause of the lack of insulin is not entirely clear. It is multifactorial, resulting from an interaction between genetic predisposition and environmental triggering factors:
Tissue cells
Low blood glucose
Endocrine responses to low and high blood glucose.
Promotes glucagon release
ӹӹ Polyphagia. Excess hunger/eating. ӹӹ Recurrent infections. ӹӹ Abdominal pain. As a presentation of DKA.
Investigations Although blood glucose level is sufficient to diagnosis DM, further tests can help establish possible aetiology, possible associations and baseline biochemical parameters before starting any treatment. ӹӹ Full blood count (FBC). There may be anaemia (with chronic disease, poor nutrition or associated pernicious anaemia), or an elevated white cell count (as part of the DM disease process, or an infection). ӹӹ Urea and electrolytes (U&Es). It is important to gauge baseline renal function, as this may become compromised. ӹӹ Liver function tests/Bone profile. This is useful as a baseline parameter. There may be later liver damage. ӹӹ Glycated haemoglobin (HbA1c). This can be used to identify average blood glucose levels over a three month interval. It is a good baseline
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injection several times a day or by continuous infusion via an insulin pump. The types of insulin are summarised in Table 2. Choosing the insulin regimen is dependent on the individual (Table 3). ӹӹ Multiple daily injections (MDI). The multiple daily injection regime is usually first choice for young people who can take ownership of monitoring their blood glucose. It involves adjusting insulin dosage based on glucose readings, meal contents, and lifestyle choices (e.g. exercise). A twice daily injection regime may be considered in those not able to cope with the demands of MDI despite appropriate education and psychosocial support. ӹӹ Insulin pump therapy. Insulin pump therapy is recommended where multiple daily injection
to assess glucose control over the coming months. ӹӹ Thyroid-stimulating hormone (TSH), thyroid auto-antibodies and coeliac autoantibodies. Hypothyroidism and coeliac disease are both associated with T1DM. ӹӹ Anti-islet cell antibodies and antiglutamic acid decarboxylase (GAD) antibodies. These are associated with T1DM, and are particularly useful if the diagnosis is not clear.
Differential Diagnosis The diagnosis is usually clear from the history, but polyuria and polydipsia may be associated with compulsive water drinking or diabetes insipidus.
Management Children with T1DM require exogenous insulin, delivered either by subcutaneous
TABLE 2: Insulin types Insulin type
Common brand names Onset of action
Peak
Duration
Rapid acting.
• NovoRapid, Humalog, Apidra.
• 10-15mins.
• 1 hour.
• 2-5 hours.
Short acting.
• Actrapid, Humulin S.
• 30-60 mins.
• 2-4 hours.
• 8 hours.
Intermediate acting.
• Insulatard, Humulin I.
• 1-2 hours.
• 4-12 hours.
• 12-18 hours.
Long acting.
• Lantus, Levemir.
• 2-4 hours.
• Plateaus.
• 12-24 hours.
regimes have failed, and there is high commitment from the family to use it properly. It may also be considered if there is a strong patient preference, and it matches lifestyle needs. ӹӹ Pancreatic transplantation. In the future, pancreatic transplantation is a potential definitive treatment for T1DM, but has major risks, and requires lifelong immunosuppressive therapy.
Wider Management Children with DM will usually be managed in secondary or tertiary care, by a multidisciplinary team experienced in working with young people and families (Figure 3). Longterm control is monitored by measuring HbA1c levels, which reflect average glycaemic control over the lifespan of an erythrocyte (two to three months). The level recommended for children is usually 7.5% is associated with a greater risk of longterm complications. DM is a chronic condition, requiring active management by the child’s caregivers. Thus one of the most important steps is educating children and their families:
TABLE 3: Insulin regimens Regimen
Insulin
Advantages
Disadvantages/ Challenges
Indication
Multiple daily injections (MDI) aka “basal bolus” regimen.
• A long acting insulin plus a short/rapid acting insulin are given to cover meals.
• It is flexible and can be adjusted according to meals. • It is more physiological.
• This requires more injections throughout the day.
• Good for those with variable diets/exercise patterns. • Good for those comfortable with multiple injections and with adjusting insulin doses.
Insulin pump.
• A continuous subcutaneous infusion of short acting insulin with boluses for meals.
• This improves quality of life. • There is a reduction in severe hypoglycaemic episodes. • It is the most physiological.
• This requires a high level of family commitment.
• When the MDI regimen has failed.
2 injections per day.
• A short/rapid acting insulin mixed with intermediate acting insulin.
• Fewer injections are required per day.
• This is less flexible. • It is more difficult to control blood glucose.
• This is rarely used nowadays. • Good for those unable to give multiple injections throughout the day.
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Ophthalmologist: screening for retinopathy.
FIGURE 3
The Diabetes MDT Specialist Nurse: Liaise closely with the family, providing education at diagnosis and being a regular point of contact.
TABLE 4: Symptoms of hypoglycaemia Stimulation of sympathetic nervous system (sympathomimetic). Psychologist: Adjusting to a complex lifelong condition can be difficult for children and families.
Anxiety.
Endocrinology
Podiatrist: providing foot care advice and screening for neuropathy/ circulatory compromise.
Dietician: to encourage families to provide a healthy diet which optimises growth potential while promoting good glycaemic control.
Tachycardia. Headache. Abdominal pain.
CNS effects (neuroglycopaenic).
Ataxia. Weakness. Confusion.
Paediatrician specialised in diabetes: Regular outpatient review in clinic, in conjunction with other members of the MDT.
Multidisciplinary approach to managing diabetes. (MDT: multidisciplinary team).
ӹӹ Insulin regimes and dietary advice. Some families learn to “carbohydrate count”, titrating insulin dosage to carbohydrate intake. ӹӹ Subcutaneous injection. Instruct on the correct technique, and importance of rotating sites. ӹӹ Blood glucose monitoring. Families should know when to check glucose, and how to respond to high and low glucose. Children should aim for a blood glucose between 4-8 mmol/L, and 38.5°. • CRP > 20mg/dL. • ESR > 40mm/h. • Non-weight bearing. • WCC > 12 x 109/litre. 5 criteria met = 97.5% chance of septic arthritis; 4= 93%; 3= 82%; 2= 62%; 1= 36%; 0= 16%
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joint space narrowing due to effusion and soft tissue swelling precede the appearance of a narrowed joint space secondary to articular cartilage destruction.
Joint Aspiration This is the definitive investigation. Joint aspiration should be carried out under aseptic conditions (to reduce the risk of contamination of a sterile joint) by an orthopaedic surgeon. The aspirate should be sent to the lab for urgent gram staining and microscopy, culture and sensitivity.
Risk Factors ӹӹ ӹӹ ӹӹ ӹӹ ӹӹ ӹӹ
Penetrating injury. Diabetes mellitus. Skin infection, e.g. chickenpox, cellulitis. Immunosuppression. Sickle cell disease. Chronic steroid use.
Causative Organisms
Treatment should be started without delay, to minimise joint damage. After joint aspiration and blood cultures, the joint should be splinted and empirical broad-spectrum intravenous antibiotics should be started whilst arranging urgent surgical drainage and irrigation of the affected joint. A prolonged course of broad spectrum antibiotics are required after drainage and the choice of antimicrobial agent should be tailored according to culture results.
ӹӹ Staphylococcus aureus. This is the most common causative organism affecting children of all ages. ӹӹ Streptococcus pyogenes and Streptococcus pneumoniae. These are less common than S. aureus and also affect all age groups. ӹӹ Haemophilus influenzae. This is uncommon in developed countries due to the Hib immunisation. ӹӹ E. coli and Bacteroides spp. This commonly causes neonatal osteomyelitis as a result of umbilical sepsis. ӹӹ Salmonella. Children with sickle cell anaemia are at higher risk of Salmonella osteomyelitis.
Complications
Clinical Features
ӹӹ Joint stiffness. This may occur secondary to cartilage damage. ӹӹ Septic dislocation. This is due to damage to the joint capsule and bone. ӹӹ Osteonecrosis. This is due to ischaemia. ӹӹ Shortening of the limb. This is due to dislocation and osteonecrosis. ӹӹ Secondary osteoarthritis. This is from damage to the joint capsule.
The presentation of acute osteomyelitis is usually: ӹӹ A markedly painful, immobile limb. Even the slightest touch or manipulation of the affected limb is acutely painful. ӹӹ Febrile illness. ӹӹ Occasionally, a history of a preceding sore throat, skin lesion or injury may be present. ӹӹ Skin changes. The skin overlying the affected site may be swollen, warm and erythematous. ӹӹ Toxaemia. If there is a delay in presentation, the child may exhibit features of toxaemia, for example, tachycardia. Untreated acute osteomyelitis may result in the formation of a subperiosteal abscess due to the infection eroding into the cortex allowing pus to strip the periosteum off the bone. This bone abscess will eventually discharge through a sinus onto the skin surface. At this point, the child is said to have chronic osteomyelitis. Vertebral osteomyelitis (uncommon in the paediatric population) may present with chronic, unremitting back pain, which is worse at rest.
Management
Prognosis With prompt recognition and management, the prognosis is usually good. However, delayed diagnosis may result in joint destruction. This is most likely to occur in the neonates, due to the difficulties with diagnosis.
Osteomyelitis Osteomyelitis is an infection of the metaphysis of long bones.
Aetiology The metaphysis is prone to infection because the area is supplied by the terminal branches of the nutrient arteries. Osteomyelitis can be acute or chronic. Similar to septic arthritis, bacteria can seed in the bone via three portals of entry: 1 Penetration from trauma. 2 Contiguous spread from a nearby soft tissue infection. 3 Haematogenous spread from a distant focus of infection. Common sources of the bacteraemia in children include an infected umbilical cord, a septic tooth, boils and abscesses.
Differential Diagnosis ӹӹ Cellulitis. The infection is limited to the skin only. ӹӹ Septic arthritis. This may be difficult to differentiate clinically although septic arthritis is associated with an acutely hot, swollen, tender joint. ӹӹ Rheumatic fever. Musculoskeletal involvement is usually a polyarthritis. Cardiac, dermatological or abdominal signs and symptoms often co-exist. ӹӹ Sickle-cell crisis. This may be suspected in cases of known sickle cell disease.
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ӹӹ Blood tests. The full blood count may demonstrate a leucocytosis. Acute phase reactants such as ESR and CRP are also likely to be elevated in acute osteomyelitis. Blood cultures prior to antibiotic therapy are mandatory and are positive in approximately 50% of cases. ӹӹ Plain radiographs. These do not demonstrate any abnormality in the early stages of the disease, but should be obtained to exclude any other bony lesions and to provide a baseline to monitor disease progression. After approximately 10 days, subperiosteal new bone formation and localised bone rarefaction may become visible. ӹӹ Magnetic resonance imaging (MRI). This may be useful to distinguish between bone and soft tissue infection. MRI can also detect acute osteomyelitis at an earlier stage by detecting bone changes caused by altered blood supply secondary to acute infection. This imaging modality is the most useful to delineate the degree of soft tissue and medullary involvement. ӹӹ Bone biopsy. This is performed percutaneously through noninfected tissue if the diagnosis remains in doubt or to obtain tissue samples to direct antibiotic therapy.
Management In the acute phase, analgesics should be administered generously and the affected limb should be splinted. Prompt intravenous antibiotic therapy is mandatory. Therapy should begin with ‘best guess’ antibiotics guided by microbiology advice. A prolonged course of antibiotics is required, and the regimen can be changed from intravenous to oral antibiotics as the inflammatory markers begin to settle and the child’s clinical condition improves. If the condition does not begin to respond to antibiotic therapy within 36 hours, or investigations demonstrate an abscess/subperiosteal collection, surgical exploration and drainage is required.
Complications ӹӹ Bone abscess. This may occur if treatment is not early or aggressive. ӹӹ Bacteraemia. The infection may spread to the systemic circulation, as the bone is very vascular. ӹӹ Chronic osteomyelitis. This may occur if inadequately treated. ӹӹ Altered bone growth. This may occur if there is damage to the growth plate. ӹӹ Fracture. A pathological fracture may occur as a result of increased susceptibility from infection. ӹӹ Septic arthritis. This is due to spread of the infection to the joint.
Orthopaedic and Rheumatological Disorders
Investigations
90-95% of cases. However, the disease can recur at any point as walled off nests of bacteria can reactivate. For this reason, long-term follow-up is required to monitor for relapse.
RHEUMATOLOGICAL DISORDERS Transient Synovitis Transient synovitis is a common cause of benign hip pain affecting children between 2 and 12-years-old.
Aetiology The aetiology is unclear, but infection and trauma are thought to be precipitating factors. However, prospective studies have shown infection (usually an upper respiratory tract infection) and trauma to be associated only in 30% and 5% of cases, respectively.
Clinical Features ӹӹ Sudden onset limp with hip and/or knee pain. ӹӹ Absence of rest pain. ӹӹ Decreased range of hip movement, especially internal rotation. ӹӹ The limb is held in the position of greatest comfort, typically flexed, abducted and slightly externally rotated. ӹӹ There may be a preceding or accompanying upper respiratory tract infection. ӹӹ The child is systemically well.
Differential Diagnosis ӹӹ Septic arthritis and osteomyelitis. There is evidence of infection, with significant pain and elevated inflammatory markers. ӹӹ Perthes disease. There is persistent pain with radiographic changes. ӹӹ Slipped upper femoral epiphysis. There are characteristic radiographic changes.
Investigations
Prognosis
The diagnostic workup should include tests to exclude alternative diagnoses and demonstrate confirmatory features. Transient synovitis is a diagnosis of exclusion and septic arthritis should always be considered. ӹӹ Blood tests. White cell count, C-reactive protein and erythrocyte sedimentation rate are usually normal in transient synovitis (but elevated in septic arthritis). ӹӹ Plain radiographs and ultrasound scanning. Plain radiographs do not usually demonstrate any joint abnormality, however a small joint effusion may be seen on ultrasound scanning. If an effusion is noted, it should be aspirated and the fluid sent to the laboratory for microscopy, culture and sensitivity to exclude an infective cause.
Prognosis is variable depending on the number of risk factors and the child’s general condition. Prompt diagnosis and treatment in an otherwise well child results in a full recovery in
The modified Kocher criteria (Box 1, p279) can help to differentiate between septic arthritis and transient synovitis.
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Treatment
Symptoms usually resolve within two weeks and there do not appear to be any long-term complications.
or mild normochromic normocytic anaemia. HLA B27 is positive in 65-96% of cases. ӹӹ Microbiology. Culture of urethral fluids, faeces or throat swabs may identify the causative organism. ӹӹ Joint aspiration. This may be required in the presence of a joint effusion to exclude septic arthritis. ӹӹ Plain joint radiographs. These will be normal in early disease. Later, a periosteal reaction may be evident.
Reactive Arthritis
Management
Reactive arthritis is the most common form of childhood arthritis.
The arthritis should be treated with: ӹӹ Rest. ӹӹ Joint immobilisation. ӹӹ Physiotherapy. ӹӹ Non-steroidal anti-inflammatory drugs. Intra-articular steroid injections are beneficial and systemic steroids may be used in refractory disease. Antibiotics are only used when a causative organism has been identified.
Transient synovitis is a benign, self-limiting condition and is managed conservatively. The mainstay of treatment is rest and simple analgesia.
Prognosis
Aetiology It is an autoimmune condition characterised by transient joint swelling (usually less than six weeks) following an extraarticular infection. There is a strong association with the major histocompatibility antigen HLA B27 and seronegative arthropathies.
Classification Two broad subgroups are recognised: 1 Post enteric form. This is related to gastrointestinal infection with microorganisms such as Salmonella, Campylobacter, Yersinia and Shigella. 2 Post venereal form. This is related to sexual activity and infection with chlamydia and gonorrhoea (rare in children).
Clinical Features Signs and symptoms of reactive arthritis usually develop two to four weeks after gastrointestinal or genitourinary infection. Onset is often acute with malaise, fever and fatigue. Other signs and symptoms are outlined in Table 3.
Investigations ӹӹ Blood tests. Inflammatory markers (CRP, ESR) will be elevated during the active phase of the disease. Full blood count may demonstrate a leucocytosis, thrombocytosis
TABLE 3: Clinical features of reactive arthritis Articular
• Mono or polyarthritis affecting the knees, ankles, feet or hands. • Sacroiliitis. • Heel pain due to calcaneal spur enthesopathy. • Plantar fasciitis.
Urethral
• Urethritis (if related to sexual activity).
Ocular
• Conjunctivitis.
Dermatological
• • • •
Systemic
• Fever. • Malaise.
Keratoderma blennorrhagica. Circinate balanitis. Macules. Pustules.
Prognosis Reactive arthritis is a self-limiting condition. The majority of patients see complete resolution of symptoms within 12 months. One-third will develop recurrent or chronic disease and one-third of these will be functionally limited due to destructive arthritis.
Juvenile Idiopathic Arthritis Juvenile idiopathic arthritis (JIA) is the term used to describe joint inflammation presenting before 16-years-old and persisting for at least six weeks in the absence of infection or other defined cause. It affects one in 1000 children in the United Kingdom.
Classification There are at least seven different forms of the disease as outlined in Table 4.
Investigations JIA is a clinical diagnosis. Investigations are required to aid diagnosis and detect complications. ӹӹ Blood tests. Full blood count may show anaemia, leukocytosis or thrombocytosis in systemic JIA. Erythrocyte sedimentation rate is generally elevated in most forms of JIA. ӹӹ Autoantibodies. Antinuclear antibody (ANA), Rheumatoid Factor (RF) and HLA B27 may be positive in subtypes outlined in Table 4. ANA positivity predicts risk of uveitis. ӹӹ Imaging. Plain radiographs are usually normal in early JIA but are useful to help exclude osteomyelitis, trauma or tumours. Magnetic resonance imaging is most useful to delineate the extent of joint damage and synovitis.
Management Treatment is aimed at improving the quality of life and preserving joint function.
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Orthopaedic and Rheumatological Disorders
TABLE 4: Subtypes, features and blood test abnormalities of JIA Subtype
Clinical features
Blood test abnormalities
Oligoarticular JIA.
• Affects one to four joints. • Joint pain (minimal), stiffness and reduced range of motion. • Five times more common in girls.
ANA positive.
Polyarthritis (RF negative).
• Affects five or more joints. • Can be symmetrical with large and small joint swelling. • Pain and stiffness with minimal swelling.
RF negative.
Polyarthritis (RF positive).
• Symmetrical large and small joint swelling with finger joint involvement.
RF positive.
Systemic JIA.
• Oligoarthritis or polyarthritis with daily spiking of temperature for at least 2 weeks. • Rash, lymphadenopathy, hepatomegaly/splenomegaly or serositis must be present. • More common in girls aged 1 to 10–years-old.
Anaemia, leukocytosis, thrombocytosis and elevated inflammatory markers.
Psoriatic arthritis.
• Asymmetrical arthritis with psoriasis. • Dactylitis or nail pitting is common.
No specific markers.
Enthesitis related arthritis.
• Initially large joint lower limb arthritis and enthesitis (inflammation at the insertion of tendons/ligaments to bone, especially the Achilles tendon), then lumbar or sacroiliac joint involvement. • In addition, for diagnosis to be made, children must have one of; anterior uveitis, HLA B27 positive or family history of HLA B27 related disease.
HLA B27 positive.
Undifferentiated arthritis.
• Arthritis that does not meet the criteria for other forms of JIA.
No specific markers.
ӹӹ Physiotherapy is required to maintain joint mobility. ӹӹ NSAIDs are effective analgesics and can be combined with intra-articular corticosteroid injections. ӹӹ Oral corticosteroids and disease modifying anti-rheumatic drugs (DMARDs) are used in severe/resistant disease.
Complications Complications depend on the subtype, but may include growth failure, anterior uveitis, amyloidosis and osteoporosis.
Prognosis The usual course of oligoarticular disease is permanent remission, whilst this is less common in polyarticular disease. Positive rheumatoid factor is associated with worse outcome.
Ankylosing Spondylitis Ankylosing spondylitis (AS) is a chronic seronegative spondyloarthropathy predominantly affecting the spine and sacroiliac joints. The disease is characterised by progressive ankylosis (stiffening) of affected joints, with initial fibrosis followed by joint ossification. It has a male to female ratio of 3:1 and peak onset is between 15 and 25-years-old.
Aetiology The aetiology is unknown but is thought to be due to a combination of genetic and environmental factors including: ӹӹ A positive family history. ӹӹ Strong association with the major histocompatibility complex antigen HLA B27.
ӹӹ Genitourinary or gastrointestinal infection may trigger the onset of ankylosing spondylitis in genetically susceptible people.
Clinical Features Disease onset is insidious with a course characterised by flares and remissions. Signs and symptoms can be divided into articular and extra-articular features:
Articular Features ӹӹ In children, lower limb oligoarthritis usually precedes spinal involvement. ӹӹ Inflammatory back pain and stiffness worse in the morning and after inactivity. ӹӹ Reduction in all back movements. Schober’s test demonstrates reduced lumbar flexion when the patient is asked to touch their toes from a standing position. ӹӹ Sacroiliac joint ankylosis may be felt as diffuse buttock pain. ӹӹ Achilles tendonitis.
Extra-articular Features ӹӹ Aortic valve insufficiency and cardiac conduction defects. ӹӹ Pulmonary fibrosis. ӹӹ Anterior uveitis. ӹӹ Amyloidosis. ӹӹ Cauda equina syndrome. ӹӹ Osteoporosis.
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Investigations Blood Tests
Prognosis
ӹӹ FBC. This may show a normochromic normocytic anaemia and/or leucocytosis. ӹӹ ESR, CRP. These may be elevated in the context of inflammation. ӹӹ HLA B27. This is strongly associated with ankylosing spondylitis.
Outcomes are generally good in ankylosing spondylitis, with cardiac and neurological complications being the most worrying. Poor prognostic indicators include peripheral joint involvement, early onset, elevated ESR and poor response to NSAIDs.
Systemic Lupus Erythematosus (SLE) SLE is rare in children but may occur in adolescent girls.
Imaging Radiological changes include (Figure 11): ӹӹ Erosions. ӹӹ Sclerosis and ankylosis of the sacroiliac joints. ӹӹ Bony bridges between adjacent vertebrae (syndesmophytes) produce the characteristic “bamboo spine” appearance. ӹӹ Peripheral joint involvement demonstrates radiographic changes similar to rheumatoid arthritis. The mnemonic “LOSS” can be helpful: loss of joint space, osteophyte formation, subchondral sclerosis and subchondral cysts.
Aetiology
Management
Clinical Features
Physiotherapy is the mainstay of treatment and is aimed at maintaining flexibility. No drugs have been shown to modify the course of the disease but NSAIDs are a useful analgesic for symptom control. Surgery is reserved for complications such as fixation of spinal fractures, correction of severe spinal deformity and hip fractures.
Systemic symptoms such as fever, malaise, anorexia and weight loss are common. Other features of SLE are outlined in Table 5. A malar rash is characteristic (Figure 12).
FIGURE 11
Syndesmophytes
Syndesmophytes
Investigations FBC may identify anaemia, thrombocytopenia, or lymphopenia. Renal and liver function should be checked. ESR and CRP may be raised. Complement (C3/4) may be low in active disease. In myositis, creatine kinase may be raised. Autoantibody tests include ANA, anti-dsDNA, anti-Smith, anti-Ro, anti-La, anti-ribosomal P, Anti-RNP, anticardiolipin, anti-histone, and lupus anticoagulant.
Management Management of SLE is largely symptomatic. Medical treatment includes supportive care, lifestyle changes, analgesics and NSAIDs. Management of patients with mild to moderate organ involvement includes oral corticosteroids or steroidsparing agents such as azathioprine and methotrexate. Major organ involvement requires intravenous steroids and biological therapy.
Faecal loading
Oestophyte
Subchondral cysts
SLE is a chronic, inflammatory autoimmune disease characterised by antinuclear antibodies and vasculitis. The cause is unknown but it is thought to be multifactorial involving genetic and environmental elements. SLE runs in families but no specific causative gene has been identified. It has a female preponderance and occurs more commonly in the South-Asian population, associated with the HLA-DR2 and HLA-DR3 alleles. Environmental influences include EpsteinBarr virus and ultraviolet light.
Fused sacroiliac joints
Subchondral sclerosis Early loss of joint space Features of ankylosing spondylitis.
Prognosis Oestophyte
SLE follows a relapsing-remitting course with a five-year survival of 90%. Mortality is often related to cardiovascular and renal disease.
Dermatomyositis Aetiology Dermatomyositis is a systemic connective tissue disorder causing acute and chronic inflammation of striated muscle. It is an ischaemic myopathy resulting in muscle infarction.
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Usual onset is between 5 and 10-years-old. Children present with symmetrical proximal muscle tenderness and weakness, arthritis and fever. Occasionally pharyngeal muscle involvement results in dysphagia. TABLE 5: Features of SLE Skin.
• Malar rash (classic “butterfly” rash over cheeks and nasal bridge). • Photosensitivity. • Alopecia (hair loss). • Livedo reticularis (lace-like purple discolouration of skin due to thrombi in capillaries. • Vasculitis. • Raynaud phenomenon. • Mucosal ulceration – usually affecting the palate. • Sjögren’s syndrome.
Musculoskeletal.
• Myalgia. • Arthralgia. • Myositis.
Renal.
• Painless haematuria and proteinuria. • Glomerulonephritis. • Hypertension.
Pulmonary.
• • • • • •
Pleurisy. Pulmonary embolism. Dyspnoea. Pneumonitis. Fibrosing alveolitis. Pulmonary haemorrhage.
Cardiovascular.
• Accelerated atherosclerosis. • Pericarditis/endocarditis/myocarditis. • Recurrent venous/arterial thrombosis.
Neurological.
• Peripheral neuropathy. • Headaches. • Transient ischaemic attacks (TIAs).
Gastrointestinal.
• • • • •
Nausea and vomiting. Abdominal pain. Oral ulceration. Jaundice. Hepatomegaly.
Haematological.
• • • •
Normochromic normocytic anaemia. Thrombocytopenia. Leukopenia. Splenomegaly.
Two cutaneous manifestations are associated with juvenile dermatomyositis, both of which can precede or occur simultaneously with onset of muscular weakness: ӹӹ The “heliotrope” rash describes a red/purple rash across the upper eyelids. It is associated with periorbital and upper lid oedema and telangiectasia of the eyelid capillaries. ӹӹ Gottron’s papules describe red papules seen classically over the knuckles but can also be seen on the extensor surfaces of the elbows and knees.
Investigations Inflammatory markers (CRP, ESR) and serum creatine kinase may be elevated and muscle biopsy demonstrates inflammatory cell infiltration and atrophy. Several auto-antibody tests have been identified that may be helpful in prognosis and subtyping (e.g. ANA, Anti–Mi-2, Anti-Jo-1, Anti-SRP), but they are not typically needed for routine diagnosis.
Management Standard treatment is with oral corticosteroids, with the dose being tailored over a two year period. Other immunosuppressant agents such as methotrexate and cyclosporin may be required in severe disease. Physiotherapy is helpful to prevent joint contractures.
Sarcoidosis Aetiology Sarcoidosis is a multisystem inflammatory disorder characterised by the development of non-caseating granulomas in affected organs. The aetiology is unknown but it is thought that certain environmental, occupational or infective agents trigger the disease in genetically susceptible individuals.
Orthopaedic and Rheumatological Disorders
Clinical Features
Clinical Features Sarcoidosis is a rare disorder in children and most childhood cases have occurred in children between 13 and 15-yearsold. Sarcoidosis can affect any organ, but frequently manifests in the lungs, skin, lymph nodes, eyes, liver and spleen in children. Accompanying constitutional symptoms such as fever, fatigue and weight loss are common. Children below the age of five years often present with a triad of arthritis, rash and uveitis, without the typical hilar lymphadenopathy and lung involvement seen in adult cases.
Investigations
FIGURE 12 Malar rash in SLE (Image courtesy of d@nderm).
Diagnostic work-up includes serum angiotensin converting enzyme (ACE) levels (elevated in 50% of children with late onset disease) and a chest X-ray to detect hilar lymphadenopathy (Figure 13). FBC, renal function, LFTs, and electrolytes including calcium should all be checked as they may be deranged. The diagnosis can be confirmed by the detection of non-caseating granulomas on biopsy specimen.
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TABLE 6: Manifestations of Marfan syndrome
Both hila are enlarged in keeping with lymphadenopathy
FIGURE 13 Bilateral hilar lymphadenopathy.
Musculoskeletal.
• Arachnodactyly (long fingers). • Tall stature with disproportionately long legs and arms – the span of the arms is greater than the height. • Pectus excavatum (sunken appearance of chest). • Spinal abnormalities – spondylolisthesis, scoliosis. • Generalised joint laxity. • Flat feet. • Mandibular retrognathia (abnormal posterior positioning of mandible). • High arched palate.
Cardiovascular.
• Dilatation of the ascending aorta. • Mitral and aortic regurgitation. • Dissecting aortic aneurysm.
Ocular.
• Lens dislocation. • Retinal detachment.
Skin.
• Striae.
Dural.
• Dural ectasia (enlargement of the dura). • Meningocoele (protrusion of the meninges through a spinal/cranial defect).
Management Corticosteroids are the mainstay of treatment. Other immunosuppressive drugs (e.g. methotrexate) may be required in steroid resistant cases.
Prognosis Long-term prognosis is good, with most children demonstrating significant improvement in symptoms and lung function over time.
Marfan Syndrome Aetiology Marfan syndrome is an autosomal dominant connective tissue disease with characteristic skeletal, dermatological, cardiac, aortic, ocular and dural malformations. It is thought to be due to a defect in the cross-linkage of collagen and elastin secondary to a mutation in the fibrillin-1 gene.
Clinical Features Common features of Marfan syndrome are outlined in Table 6. More formal diagnosis is with the Ghent Nosology.
Management Management should involve a multidisciplinary team including geneticists, ophthalmologists, cardiologists and orthopaedic surgeons. Prophylactic beta-blockers have been used with limited efficacy to reduce the rate of aortic dilatation (by reducing the mean arterial pressure and heart rate). Surgical management is indicated with progressive aortic disease, significant valvular disease, or certain orthopaedic problems such as severe scoliosis or flat feet.
Prognosis Patients with Marfan syndrome can develop severe orthopaedic, cardiovascular and ocular complications, but as treatment and early recognition of complications has
advanced, so has life expectancy. The main cause of death is cardiovascular disease and vascular complications.
SKELETAL DISORDERS Achondroplasia Achondroplasia is the most commonly occurring form of short-limb dwarfism. It affects one in 30,000 births and the incidence increases as paternal age increases. Inheritance is autosomal dominant, although 80% of cases arise due to sporadic mutation.
Aetiology Virtually all cases are caused by a G380R mutation in fibroblast growth factor receptor 3 (FGFR3). FGFR3 is also important in craniofacial, vertebral and neurological development, therefore this mutation has multiple effects in affected individuals.
Clinical Features ӹӹ The characteristic feature is a normal length trunk with disproportionately short limbs, especially proximally. ӹӹ Broad limbs with deep creases. ӹӹ Short fingers. ӹӹ Small face and flat nasal bridge. ӹӹ Enlarged skull vault and frontal bossing. ӹӹ Marked lumbar lordosis when standing. ӹӹ Increased joint laxity.
Differential Diagnosis Other forms of short limb skeletal dysplasia include: ӹӹ Hypochondroplasia. There is less pronounced skeletal disproportion and spinal abnormalities, with the face and skull appearing normal.
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Skeletal Disorders
The average final adult height is 131 cm in males and 124 cm in females.
Investigations
Osteogenesis Imperfecta
Diagnosis is based on the typical features of the condition and characteristic radiographic appearances. If there is clinical suspicion of skeletal dysplasia, a full skeletal survey should be performed. Radiographic features of achondroplasia include: ӹӹ Short bones. ӹӹ Metaphyseal irregularity. ӹӹ Small iliac wings. ӹӹ Narrow pelvic anteroposterior diameter. ӹӹ Translucent proximal femur. ӹӹ Progressive narrowing of the spinal inter-pedicular distance from top to bottom. It may be possible to diagnose short limb skeletal dysplasia in the prenatal period by ultrasonography. If short limb skeletal dysplasia is diagnosed prenatally, plasma can be analysed for the FGFR3 mutation in the mother.
Management Achondroplasia is managed symptomatically. It is recommended that children with achondroplasia are followed up at regular intervals to detect any significant complications. These occur in approximately 10% of children, and may require operative treatment.
Complications ӹӹ Hydrocephalus. There is rapid head growth during the first year of life which needs close monitoring because two percent of children with achondroplasia develop hydrocephalus requiring treatment. ӹӹ Lower limb deformities. Genu varum is common. ӹӹ Progressive thoracolumbar kyphosis.
Orthopaedic and Rheumatological Disorders
ӹӹ Morquio syndrome. The shortening affects the distal limb segments more than the proximal segments. There are widespread associated defects. ӹӹ Epiphyseal dysplasias. The head and face are normal but the epiphyses show characteristic changes on plain radiographs. ӹӹ Proportionate dwarfism. The limbs and trunk are equally affected.
Prognosis Life expectancy is normal in the majority of affected children. However, because of the large head and short limbs, early motor development is often delayed. Intellectual development is normal.
Aetiology Osteogenesis imperfecta is one of the most common inheritable bone disorders, affecting one in 20,000 children. It is characterised by defective synthesis of collagen type 1, resulting in generalised fragility of bones, teeth, ligaments, sclera and skin.
Clinical Features There are four subtypes of osteogenesis imperfecta, showing variations in phenotype. However, common features irrespective of subtype include: ӹӹ Osteopenia. ӹӹ Bone fragility with proneness to fracture with minimal trauma. ӹӹ Laxity of ligaments. Subtypes of osteogenesis imperfecta and their features are outlined in Table 7. In almost all cases, the mode of inheritance is dominant or involves a new dominant mutation irrespective of the phenotypic subtype.
Investigations ӹӹ Severe variants can be diagnosed prenatally by ultrasound scanning. ӹӹ In the postnatal period, diagnosis is made on the basis of the clinical features. In mild cases, non-accidental injury is an important differential diagnosis. ӹӹ Plain radiographs, bone densitometry and genetic testing may all be useful aids to diagnosis. Wormian bones are seen in type 1 disease on skull X-ray.
TABLE 7: Subtypes and features of osteogenesis imperfecta Type I (mild)
Type II (perinatal lethal)
Type III (severe deforming)
Type IV (moderately severe)
• • • • • •
• Multiple fractures in utero. • May be stillborn.
• Blue then white sclera. • Progressively deforming long bones and spine due to fractures. • Short stature. • Dental problems.
• Similar to type I and differentiated on the basis of having white sclera. • Variable short stature.
Blue sclera. Hypermobile joints. Little or no deformity. Fractures occur throughout life. Hearing loss in adulthood. Skull shows multiple Wormian bones (extra bone pieces in the sutures of the skull).
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Management Treatment involves physiotherapy, rehabilitation and surgery. Gentle nursing of infants is paramount to prevent fractures and prompt splinting of fractures is required to limit deformity. Bisphosphonates may reduce bone pain and fracture rate in severe disease. Surgery may be needed for fracture fixation or to correct deformities.
TABLE 8: Neurological causes of pes cavus Static neurological disorders Progressive neurological disorders • • • • •
Stroke. Cerebral palsy. Polio. Spinal nerve root injury. Peroneal nerve injury.
• • • • • •
Charcot-Marie-Tooth disease. Friedreich ataxia. Spinal/brain tumour. Spinal trauma. Muscular dystrophy. Syringomyelia.
Prognosis Life expectancy is normal in type I disease and only slightly reduced in type IV. When there is severe deformity as in type III, the patient may become wheelchair bound.
severity of the lateral curvature can be determined by measuring the angle of the curvature on a plain spine radiograph.
Osteopetrosis
Mild curvature does not require treatment. Braces are utilised for 23 hours daily for moderate curvature. Severe scoliosis (>40°) requires internal fixation or fusion.
Aetiology Osteopetrosis, also known as marble bone disease, is a metabolic bone disease characterised by failure of osteoclast bone resorption secondary to carbonic anhydrase deficiency. This leads to a diffuse increase in bone density and obliteration of the marrow spaces. The end result is skeletal fragility despite an increase in bone mass. The autosomal recessive form presents in children and is more severe than the autosomal dominant form of the disease.
Investigations
Management
Pes Cavus Aetiology Pes cavus can be a normal variant, or due to a congenital bony abnormality or secondary to neurological disorders where the intrinsic foot muscles are weak or paralysed. Detailed family history helps identify any underlying neurological disorder (Table 8).
X-rays are usually diagnostic. They usually show generalised osteosclerosis, although there may be areas of alternating sclerotic and lucent bands. Fractures and osteomyolytic changes may also be seen.
Clinical Features
Clinical Features
Management
Children with autosomal recessive osteopetrosis present with faltering growth, recurrent infections, anaemia and thrombocytopenia (due to bone marrow infiltration). It is often fatal due to overwhelming infection or severe/repeated haemorrhage. The autosomal dominant form usually presents later in childhood with pathological fractures.
Treatment depends on the cause and severity of the deformity. Often no treatment is required. Conservative measures include physiotherapy to loosen tight muscles, orthotic shoes and splints. Surgery is reserved for severe deformity or pain that limits normal activities and is unresponsive to conservative treatments.
Management Treatment of severe disease is with bone marrow transplant, high dose vitamin D and gamma-interferon. Internal fixation of pathological fractures is extremely difficult as the dense bone makes fixation and drilling challenging.
Idiopathic Scoliosis Scoliosis is lateral curvature in the frontal plane of the spine. For thoracic scoliosis, characteristic “humps” can be seen on examining the child’s back when they are bent forward. This is reflective of rotation of the thoracic ribs, from the scoliosis.
Clinical Features Although often asymptomatic, scoliosis may cause cosmetic problems, cardiopulmonary compromise, loss of balance when mobilising and pain from spinal degenerative changes. The
In pes cavus, the longitudinal foot arch is higher than normal. Often, there is an associated clawing of the toes as the weight is taken on the metatarsal heads when walking.
ADDITIONAL PATHOLOGIES Torticollis Torticollis is a symptom rather than a diagnosis. It describes the tilting of the head in one direction, with rotation of the head in the opposite direction. It can be congenital or acquired (Table 9). If it is acquired, it is very important to identify any possible trauma in the history: a C-spine X-ray should be obtained if any doubts, as torticollis may relate to atlantooccipital subluxation. The most common cause is congenital muscular torticollis. It presents within the first few months of life, although if present in newborns, it is also important to consider a clavicular fracture as a possible cause. Congenital muscular torticollis is due to a contracture of the sternocleidomastoid muscle and there is often a fibrotic mass palpable within the substance of the muscle. It is thought to be a result of in utero stretching/compression of
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Additional Pathologies
Congenital
Acquired
• Cervical hemivertebrae. • Soft tissue abnormalities, e.g. unilateral absence of sternocleidomastoid. • Congenital muscular torticollis. • CNS malformations.
• • • •
Atlanto-occipital subluxation. Bone/soft tissue tumour. Cervical lymphadenitis. Sandifer syndrome (from gastro-oesophageal reflux). • Retropharyngeal abscess. • Ocular disorders.
the muscle. It is managed conservatively by passive stretching, stimulation and positioning. In resistant cases, botulinum toxin injection or surgical release of the sternocleidomastoid muscle should be considered. In other cases, the underlying cause needs to be treated (e.g. reflux medication in Sandifer syndrome). Otherwise, a trial of analgesia and a soft collar may be used, with consideration of muscle relaxants and traction in those who have persistent symptoms.
Osgood-Schlatter Disease Aetiology This is an overuse syndrome characterised by small avulsion fractures within the tibial tuberosity due to traction from the patella tendon during forceful contraction of the quadriceps muscles. It usually occurs around the pubertal growth spurt and is associated with physical exertion before skeletal maturation. It is often seen in children who participate in sports such as football and gymnastics and is more common in boys.
Treatment is with rest, analgesia and physiotherapy for quadriceps muscle strengthening. Rarely there is misalignment of the patella requiring surgical intervention. The majority of cases resolve by 30 years of age.
Osteochondritis Dissecans Aetiology Osteochrondritis dissecans is the separation of a small fragment of avascular bone and overlying cartilage from one of the femoral condyles and appears as a loose body in the joint. The disorder is frequently idiopathic but it may result secondary to trauma (microtrauma or repeated minor trauma) or ischaemia.
Clinical Features Osteochondritis dissecans usually presents in the teenage years with intermittent aching or swelling, worsened by activity. Locking, catching and giving way may occur, especially with an intra-articular loose body.
Investigations Plain radiographs may demonstrate a loose body or subchondral crescent sign. CT or MRI can further delineate the pathology and guide management.
Management Physiotherapy and rest is all that is required for small stable lesions. Unstable or large lesions may warrant arthroscopic removal or fixation with pins/screws.
Clinical Features
Genu Varum
Children report gradual onset pain and swelling below the knee, exacerbated by physical activity and relieved by rest. Diagnosis is clinical. Examination of the hip is important as SUFE may present similarly.
Genu varum describes bow legs.
Management Osgood-Schlatter disease is self-limiting and resolves with skeletal maturation. Avoidance of strenuous physical exertion, ice packs, analgesia and quadriceps stretching and strengthening are advised.
Chondromalacia Patellae Aetiology Chondromalacia patellae is softening of the articular cartilage of the patella. It is a significant cause of anterior knee pain in teenage girls.
Clinical Features Chondromalacia patellae presents with anterior knee or retropatellar pain exacerbated by standing up from a sitting position (patella becomes tightly apposed to the femoral condyles) or walking down stairs. There may be a knee effusion present. Diagnosis is clinical.
Orthopaedic and Rheumatological Disorders
Management
TABLE 9: Causes of torticollis
Aetiology Children up to the age of two years may have physiological genu varum, secondary to a tight posterior hip capsule. Pathological genu varum (or genu valgum) should be considered in: ӹӹ Any severe and progressive deformity. ӹӹ Asymmetrical knees. ӹӹ Short stature. ӹӹ Family history of skeletal problems. Causes include rickets and skeletal dysplasias such as achondroplasia. Untreated, the condition may worsen and result in impaired mobility, knee osteoarthritis, meniscal tears and tibiofemoral subluxation.
Clinical Features Genu varum is characterised by painless, symmetrical bowing of the legs, with a tendency for recurrent falls.
Investigation Diagnosis is by plain radiography of the lower limbs and is often the only imaging modality required.
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Management
SUFE
Management of genu varum involves medical treatment of any underlying disease. This condition resolves spontaneously as the child grows and does not usually require any treatment. Corrective surgery is required when medical therapy fails or if deformity is severe.
1 Water A, Helms P, Platt M. Paediatrics: A Core Text on Child Health. Second Edition. Oxford: Oxford University Press; 2006. 2 Alshryda S et al. Intervention for treating slipped upper femoral epiphysis. The Cochrane Collaboration. 2013. www.cochranehandbook.org. 3 Peck D. Slipped Capital Femoral Epiphysis: diagnosis and management. Am Fam Physician. 2010; 82: 258-62. 4 Uglow M, Clarke N. The management of slipped capital femoral epiphysis. J Bone Joint Br. 2004; 84: 631-5.
Genu Valgum Genu valgum describes knock knees.
Aetiology Children between 2 and 6-years-old may have a physiological genu valgum. Typically, these children have ligamentous laxity but the condition does not cause any functional impairment. Pathological genu valgum should be considered if: ӹӹ The deformity is severe and progressive. ӹӹ The lower limbs are asymmetrical. ӹӹ There is a family history of skeletal disorders. Causes of pathological genu valgum include rickets and scurvy. If left untreated, knee instability and osteoarthritis may develop, with a limitation of function.
Clinical Features It is characterised by painless, symmetrical knock knees.
Investigations Like the genu varum deformity, genu valgum can be diagnosed with plain radiography of the lower limbs.
Management For physiological genu valgum, parental reassurance is all that is required as the condition spontaneously resolves around 7-years-old. Although medical control of underlying disease is important, surgery is the only effective treatment for pathological genu valgum.
REFERENCES AND FURTHER READING Trauma/Fractures 1 Soloman L, Warick D, Nayagam, S. Apley’s concise system of orthopaedics and fractures. Fourth Edition. Florida: CRC Press; 2014. 2 Patterson J M. Children’s fractures ‘not to be missed’. Hosp Med. 2002; 63: 426-8.
Developmental Dysplasia of the Hip 1 Loder T, Skopelja E. The epidemiology and demographics of hip dysplasia. ISRN Orthopedics. 2011; 2011: 1-146. 2 Sewell MD, Rosendahl K, Eastwood DM. Developmental dysplasia of the hip. BMJ. 2009; 24;339:b4454. Available from: DOI: 10.1136/bmj.b4454. 3 Storer S, Skaggs D. Developmental dysplasia of the hip. Am Fam Physician. 2006; 74: 1310-6.
Perthes Disease 1 Kim H, Herring J. Pathophysiology, classifications, and natural history of Perthes disease. Orthop Clin North Am. 2011; 42: 285-95. 2 Shah, H. Perthes disease: evaluation and management. Orthop Clin North Am. 2014; 45: 87-97. 3 Perry D, Bruce C. Evaluating the child who presents with an acute limp. BMJ. 2010; 341:c4250. 4 Kim H, Herring J. Pathophysiology, classifications, and natural history of Perthes disease. Orthop Clin North Am. 2011; 42: 285-95.
Septic Arthritis 1 McCarthy J, Dormans J, Kozin S, Pizzutillo P. Musculoskeletal infections in children. J Bone Joint Surg Am. 2004; 86: 850-63. 2 Kocher M, Zurakowski D, Kasser J. Differentiating between septic arthritis and transient synovitis of the hip in children: an evidence-based clinical prediction algorithm. J Bone Joint Surg Am. 1999; 81: 1662-70. 3 Caird M et al. Factors distinguishing septic arthritis from transient synovitis of the hip in children. A prospective study. J Bone Joint Surg Am. 2006; 88: 1251-7.
Osteomyelitis 1 Lazzarini L, Mader T, Calhorn J. Osteomyelitis in long bone. J Bone Joint Surg Am. 2004; 86: 2305-18. 2 Yeo A, Ramachandran M . Acute haematogenous osteomyelitis in children. BMJ. 2014; 348:g66.
Transient Synovitis 1 Nouri A, Walmsley D, Pruszczynski B, Synder M. Transient synovitis of the hip: a comprehensive review. J Pediatr Orthop B. 2014; 23:32-6. 2 Hart, J. Transient synovitis of the hip in children. Am Fam Physician. 1996; 54: 1587-91. 3 Do T. Transient synovitis as a cause of painful limps in children. Curr Opin Pediatr. 2000; 12: 48-51.
Reactive Arthritis 1 Taylor-Robinson D (ed.). Clinical Problems in Sexually Transmitted Diseases (New Perspectives in
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References and Further Reading
4 Williams A et al. Medical treatment of Marfan syndrome: a time for change. Heart. 2008; 94: 414-21.
Juvenile Idiopathic Arthritis
Osteogenesis Imperfecta
1 Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet. 2007; 369: 767-78. 2 Adib N, Silman A, Thomson W. Outcome following onset of juvenile idiopathic inflammatory arthritis: I. frequency of different outcomes. Rheumatology (Oxford). 2005; 44: 995-1001. 3 Oen K et al. Disease course and outcome of juvenile rheumatoid arthritis in a multicenter cohort. J Rheumatol. 2002; 29: 1989-99.
1 Rush E, Plotkin H. Genetics of Osteogenesis Imperfecta. Medscape 2012. http://emedicine.medscape.com/ article/947588-overview.
Ankylosing Spondylitis
1 Altaf F, Gibson A, Dannawi Z. Adolescent idiopathic scoliosis. BMJ. 2013; 346:f2508.
1 McVeigh, C., Cairns, A. Diagnosis and management of ankylosing spondylitis. BMJ. 2006; 333:581. 2 Zochling J, van der Heijde D, Dougados M, Braun J. Current evidence for the management of ankylosing spondylitis: a systematic literature review for the ASAS/EULAR management recommendations in ankylosing spondylitis. Ann Rheum Dis. 2006; 65: 423-32.
SLE 1 D’Cruz DP. Systemic lupus erythematosus. BMJ. 2006; 332: 890-4.
Dermatomyositis 1 Callen J, Wortmann R. Dermatomyositis. Clin Dermatol. 2006; 24: 363-73. 2 Martin N, Li C, Wedderburn L. Juvenile dermatomyositis: new insights and new treatment strategies. Ther Adv Musculoskelet Dis. 2012; 4: 41–50.
Achondroplasia 1 Nahar R et al. Molecular studies of achondroplasia. Indian J Orthop. 2009; 43: 194-6. 2 Wright M, Irving M. Clinical management of achondroplasia. Arch Dis Child. 2012; 97: 129-34.
Osteopetrosis 1 Wilson C, Vellodi A. Autosomal recessive osteopetrosis: diagnosis, management, and outcome. Arch Disease Child. 2000; 83(5): 449–52.
Scoliosis
Pes Cavus 1 Marks R. Midfoot and forefoot issues cavovarus foot: assessment and treatment issues. Foot Ankle Clin. 2008; 13: 229-41. 2 Wicart P. Cavus foot, from neonates to adolescents. Orthop Traumatol Surg Res. 2012; 98: 813-28.
Torticollis 1 Tomczak K, Rosman N. Torticollis. J Child Neurol. 2013; 28(3): 365-78.
Osgood-Schlatter Disease 1 Weiler R, Ingram M, Wolman R. 10-Minute Consultation. Osgood-Schlatter disease. BMJ. 2011; 343:d4534.
Chondromalacia Patellae
Sarcoidosis
1 Hong E, Kraft M. Evaluating anterior knee pain. Med Clin North Am. 2014; 98: 697-717.
1 Shetty A, Gedalia A. Childhood sarcoidosis: a rare but fascinating disorder. Paediatr Rheumatol. 2008; 6: 16.
Osteochondritis Dissecans
Marfan’s Syndrome 1 Online Mendelian Inheritance in Man (OMIM). Marfan’s Syndrome; MFS. [Online] http://omim.org/entry/154700. 2 Tierney E, Feingold B, Printz B. Beta-blocker therapy does not alter the rate of aortic root dilation in pediatric patients with Marfan syndrome. J Pediatr. 2007; 150: 77-82. 3 Matt P et al. Recent advances in understanding Marfan syndrome: should we now treat surgical patients with losartan? J Thorac Cardiovasc Surg. 2008; 135: 389-94.
Orthopaedic and Rheumatological Disorders
Clinical Microbiology). Dordrecht: Martinus Nijhoff Publishers; 1985. 2 Kvien T et al. Reactive arthritis: incidence, triggering agents and clinical presentation. J Rheumatol. 1994; 21: 115-22. 3 Palazzi C, D’Amico E, Pennese E, Petricca A. Management of reactive arthritis. Expert Opin Pharmacother. 2004; 5: 61-70. 4 Selmi C, Gershwin M. Diagnosis and classification of reactive arthritis. Autoimmun Rev. 2014; 13: 546-9.
1 Pascual-Garrido C, Moran C, Green D, Cole B. Osteochondritis dissecans of the knee in children and adolescents. Curr Opin Pediatr. 2013; 25: 46-51. 2 Hixon A, Gibbs L. Osteochondritis dissecans: a diagnosis not to miss. Am Fam Physician. 2000; 61: 151-6.
Genu Varum and Genu Valgum 1 Soloman L, Warick D, Nayagam, S. Apley’s Concise System of Orthopaedics and Fractures. Fourth Edition. Florida: CRC Press; 2014.
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1.20
PUBLIC HEALTH CHRISTOPHER HARRIS
INTRODUCTION
C
CONTENTS 292 Introduction 294 Providers of Health Care Services for Children
294 Primary Care Facilities 294 Secondary and Tertiary Care 295 Other Service Providers
295 Health Promotion for Children 295 Protecting Children’s Health 295 Immunisation 295 Notifiable Diseases 295 Accident Prevention 295 Vulnerable Children
296 References and Further Reading
alculations for worldwide childhood morbidity and mortality are integral to healthcare planning and political direction. A 2013 article in the Lancet reported that 6.3 million children were estimated to die before the age of five, with 51.8% dying of infectious causes and 44% dying in the neonatal period. When compared to mortality rates in 2000, advances in treating pneumonia, diarrhoea and measles were collectively responsible for a reduction of approximately 1.8 million deaths. Projected estimates from this data predict 4.4 million children dying under five years in 2030. Part of the global health agenda is to identify and reduce preventable deaths. Today, mortality is falling, but morbidity is increasing. For example, of the estimated 1.15 million babies with neonatal encephalopathy in 2010, 287,000 died, but a further 412,000 were estimated to survive with neurological impairment. With the provision of modern medicine and suitable intensive care, more babies are surviving, but the resultant medical complications continue throughout their childhood and into adulthood. These complications can reduce quality of life, and even if well managed, represent a major financial cost. More generally, children who would have died from acute infections or due to complications of a medical condition are surviving longer with chronic health concerns. A summary of key causes of mortality, and morbidity are shown in Table 1-2. The cost of providing healthcare worldwide is increasing. Ill health and disease have financial, social and potentially health implications that go beyond the single patient who seeks medical attention. Through public health, it is possible to reduce these burdens of healthcare and better manage the finite resources of individual regions, countries and the world as a whole. Forward planning, health promotion and service provision must evolve to meet the growing and ever changing demands of our diverse populations. As we entered the new millennium, it was recognised that a common framework was needed to provide measurable improvements in health and wellbeing across the developing world. In September 2000, world leaders signed up to the United Nations Millennium Development Goals (MDGs). These were goals designed to focus efforts on a national and international level until 2015. Both progress and solutions
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Introduction
TABLE 1: Causes of mortality in the UK and world (1) – ONS UK 2012, (2)- WHO 2004-14 UK
Worldwide
Leading causes of mortality in 5 to 19-year-olds
Leading causes of mortality in under 5-year-olds
Leading causes of mortality in 5 to 15-year-olds
1 Birth complications and prematurity. 2 Congenital abnormalities. 3 Neoplasm. 4 Trauma. 5 Infections. 6 Metabolic and endocrine disorders.
1 2 3 4
1 Birth complications and prematurity. 2 Pneumonia. 3 Diarrhoea. 4 Malaria. 5 Measles. 6 HIV/AIDs.
1 2 3 4 5 6
Trauma and accidents. Neoplasm. Congenital abnormalities. Infections.
varied dramatically between nations, however, overall the goals were reported as a major success in improving equality across a wide range of issues. In 2015, the MDGs were replaced by the Sustainable Development Goals (Table 3). These are far more wide ranging and took note of concerns levelled at the original goals; that they did not go far enough and they were not specific enough. There continues to be a large focus on paediatric health in these goals, recognising that children are a vulnerable group in the developing world with no voice of their own. Public health officials must work closely with governments and investors to ensure the decisions made have the highest impact at the lowest cost (financial and otherwise) to the public. It is clear that with good public health planning and implementation, the long-term cost to individual countries will be reduced and economic growth strengthened. Public health initiatives are not restricted to medical interventions.
Public Health
Leading causes of mortality in under 5-year-olds
Trauma and accidents. Infections. Nutritional deficiencies. Congenital abnormalities. Neurological causes. Metabolic and endocrine diseases.
TABLE 2: Causes of morbidity in the UK and world (1) – ONS UK 2012, (2)- WHO 2002-14 UK Causes of childhood morbidity
Worldwide causes of childhood morbidity
1 Disability relating to birth/ congenital abnormality. 2 Disability relating to trauma. 3 Disability relating to acute illness. 4 Asthma. 5 Diabetes. 6 Obesity.
1 Disability relating to birth/ congenital abnormality. 2 Disability relating to trauma. 3 Disability relating to acute illness. 4 HIV/AIDS. 5 Chronic infection. 6 Malnutrition.
They also include: ӹӹ Education. ӹӹ Healthy living. ӹӹ Accident prevention. ӹӹ Reducing health inequality. ӹӹ Healthcare provision.
TABLE 3: Millennium and Sustainable Development Goals Millennium Development Goals 1 Eradicate extreme poverty and hunger. 2 Achieve universal primary education. 3 Promote gender equality and empower women. 4 Reduce child mortality. 5 Improve maternal health. 6 Combat HIV/Aids and other diseases. 7 Ensure environmental sustainability. 8 Global partnership for development.
Sustainable Development Goals 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17
End poverty in all its forms everywhere. End hunger, achieve food security and improved nutrition, and promote sustainable agriculture. Ensure healthy lives and promote wellbeing for all at all ages. Ensure inclusive and equitable quality education and promote lifelong learning opportunities for all. Achieve gender equality and empower all women and girls. Ensure availability and sustainable management of water and sanitation for all. Ensure access to affordable, reliable, sustainable and modern energy for all. Promote inclusive and sustainable economic growth, full and productive employment and decent work for all. Build resilient infrastructure, promote inclusive and sustainable industrialisation and foster innovation. Reduce inequality within and among countries. Make cities and human settlements inclusive, safe, resilient and sustainable. Ensure sustainable consumption and production patterns. Take urgent action to combat climate change. Conserve and sustainably use the oceans, seas and marine resources for sustainable development. Protect, restore and promote sustainable use of terrestrial ecosystems, sustainably manage forests, combat desertification, halt and reverse land degradation and halt biodiversity loss. Promote peaceful and inclusive societies for sustainable development, provide access to justice for all and build effective, accountable and inclusive institutions at all levels. Strengthen the means of implementation and revitalise the global partnership for sustainable development.
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Public Health
Local councils, schools, healthcare workers, national governments, international groups and charities work towards achieving the core aims of public health through a variety of measures. The objectives of these groups vary to reflect the differences in global, national and local causes of morbidity and mortality. However, the core aims of public health are: ӹӹ Promoting and protecting health and well-being. ӹӹ Preventing ill health. ӹӹ Prolonging life.
PROVIDERS OF HEALTH CARE SERVICES FOR CHILDREN
TABLE 4: Specialist paediatric care Secondary and Tertiary Care Facility
Role in Child Health
Midwives.
Monitor newborn infants up to 14 to 28–days-old. Ensure good feeding, growth and development.
Emergency Services.
Provide urgent care for sick children. Important role in identifying child protection concerns.
Paediatric Inpatient Wards.
Specialist paediatric care. Provide on-going care for sick children who require admission. Can provide a place of safety for a child.
Paediatric Outpatient Services.
Specialist paediatric care. Children requiring on-going specialist care. Monitoring of illness and development.
Subspecialist Care (e.g. cardiology, endocrinology, surgery and oncology).
Satellite clinics run from a central location. Access to specialised testing, diagnostics and treatment.
Community Paediatric Services.
Developmental assessment. Behaviour assessment. Autism diagnosis and care. Coordinate vaccination strategy. Regular check-ups for looked after children. Assess medical needs for children who require educational support in schools. Child protection.
Primary Care Facilities Primary care physicians and related services form a vital part of the National Childhood Surveillance Service in the UK through set appointments for: 1 Health visitor visits post birth. 2 Baby checks at six weeks. 3 School medical care (school nurses). Unwell children are primarily brought to primary care settings, (either primary care physicians or paediatric specialists in a community setting) who can provide diagnosis, treatment and advice in the majority of cases they see. They can also offer medical care for complex groups of patients, such as migrant children and those with social concerns. These patients often have complex medical and psychosocial needs that require special attention. Therefore, other experts are often involved, such as social services and the community paediatric team. Primary care physicians are able to refer any child, if needed, to a wide range of specialist services such as: ӹӹ Physiotherapy. ӹӹ Occupational therapy. ӹӹ Speech and Language Therapy (SALT). ӹӹ Hearing screening. ӹӹ Parent support groups. ӹӹ Health visitors. ӹӹ Community paediatrics.
TABLE 5: Professional groups involved in childcare Service Provider
Role in Child Health
Schools.
Child surveillance. Identify learning and developmental difficulties. Integrate children with health needs into education. Child protection.
Social Services.
Investigate child protection issues. Care for children when parents are not able or safe to do so, through fostering and adoption placements. Acquire funding to meet health needs of a child (e.g. home alterations).
Local Councils and Governments.
Provide funding for health initiatives and services. Promote healthy living. Provide parks and other leisure activities. Support children’s centres. Road safety and accident prevention.
Charities and Interest Groups.
Promote specific agendas to improve child health, some examples: • Barnardo’s (homeless children). • NSPCC and Childline. • Sporting groups. • Save the Children. • Oxfam.
ӹӹ Secondary/tertiary paediatric care. ӹӹ Community and Adolescent Mental Health Services (CAMHS).
Secondary and Tertiary Care Parents and children may access secondary care services directly or
by referral from another healthcare professional. Schools, police and social services may also seek specialist paediatric advice or referral. Secondary and tertiary care services work closely with primary care services to ensure good patient centred care.
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Protecting Children’s Health
good planning, preventative measures or early identification.
endemic, epidemic and pandemic diseases.
Other Service Providers
Immunisation
Accident Prevention
Since Edward Jenner first discovered the role of cowpox in protecting individuals from smallpox, immunisation has been a key part of global health care. Immunisations are available for a large number of conditions that previously had a high mortality or morbidity rate. They may be given routinely (diphtheria, polio, pertussis, tetanus, meningitis C, pneumococcus, measles, mumps and rubella), in response to increased exposure or outbreak (BCG, measles booster, hepatitis B), or to protect children travelling to an “at risk” area (hepatitis A, typhoid, yellow fever, rabies). In the future, it is hoped that there will be vaccines available for HIV, malaria and many other important diseases. See p150.
The police, fire brigade and medical profession all have a role in accident prevention. Identifying patterns and geographical locations of injuries presenting to an ED might highlight an on-going safety concern. In addition, some countries review all paediatric deaths such as in the UK (Child Death Review Panel) to identify broader risk to the population. The responsible body can then act on these concerns to reduce or remove the risk. Risks identified this way could be: ӹӹ An unsafe piece of equipment in a school playground. ӹӹ Poor visibility at pedestrian crossings. ӹӹ Lack of supervision at a play centre. ӹӹ Lack of a barrier around deep water.
Notifiable Diseases
Vulnerable Children
Some infectious diseases must be reported to the relevant public health authority to ensure that there is a prompt investigation, urgent risk assessment and rapid response to the illness. This has been shown to prevent epidemics and pandemics. It is not necessary to confirm the diagnosis, as this may take days with laboratory testing. If a clinician suspects a notifiable disease, the health protection agency should be informed. They will help with contact tracing and provide prophylaxis medication to any deemed at risk of infection. Examples of notifiable diseases are shown in Table 6. Table 7 lists the differences between
In every society, there are highrisk children for health problems,
It has been recognised that the health and development of infants and children is affected by a number of factors outside of primary or secondary care. Professionals, charities and other groups can work to improve child health, both directly and indirectly. Some examples are summarised in Table 5.
HEALTH PROMOTION FOR CHILDREN Evidence shows that choices made during a child’s development affect health and well-being during adulthood. A key role of public health workers is to promote healthy choices and support a broader agenda for health improvement. Some key initiatives affecting children have been: ӹӹ Promoting breastfeeding through the World Health Organisation (WHO). ӹӹ Smoking cessation support for both parents and children. ӹӹ Encouraging healthy eating and micronutrient recommendations. ӹӹ Increasing the provision and uptake of antenatal and maternity care. ӹӹ Increasing public awareness of mental health issues in children and adolescents. ӹӹ Free healthy school meals. ӹӹ Vitamin D supplementation. All health workers should recognise issues that may precipitate or worsen medical illness. For example, a health professional that sees a child with worsening asthma can support a family trying to resolve damp in their home. Public health officials may promote the benefits of having greater numbers of open spaces and parks for combating obesity in children.
PROTECTING CHILDREN’S HEALTH Some diseases, illnesses or injuries can be prevented or their effects mitigated by
Public Health
Examples of professional groups in secondary and tertiary care are listed in Table 4.
TABLE 6: Some notifiable diseases in the UK Notifiable diseases Meningitis. Poliomyelitis. Anthrax. Cholera. Food poisoning (e.g. salmonella). Hepatitis A and B. E Coli 0157. Malaria. Measles. Mumps. Rubella. Viral haemorrhagic fever (Ebola). Pertussis.
TABLE 7: Useful definitions in public health Word
Definition
Example
Endemic.
A disease regularly found in a population.
Obesity is endemic in the UK.
Epidemic.
A disease that spreads to a large number of people in a population rapidly over a short period.
The measles epidemic in Wales 2013.
Pandemic.
A disease that spreads across different populations.
The HIV pandemic has spread to affect people from all continents.
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TABLE 8: Examples of vulnerable children in the UK Group of Vulnerable Children
Professionals commonly involved
Specific health needs
Children at risk of neglect, emotional, physical or sexual abuse and children who are removed from their parents.
• Community Paediatrician. • Social Services. • Family Courts.
• Emotional support. • Child protection medicals. • Ensuring normal development. • Behavioural support.
Children with disabilities.
• • • • • •
Community Paediatrician. General Paediatrician. Speciality Paediatrician. Physiotherapist. Occupational therapist. Speech and language therapist. • Social services.
• Complex and varied needs. • Coordination of and communication between multiple professionals.
Children seeking or granted asylum.
• Community Paediatrician. • Social Services. • Immigration Services.
• Exposure to tropical diseases. • Emotional support. • Integration.
• Community Paediatrician. • Social Services. • School teachers and nurse.
• Assess needs in school. • Input support required.
Children with special educational needs.
faltering growth or abuse. In the UK, every effort is made to identify these children, anticipate their needs and ensure they receive appropriate care (Table 8).
REFERENCES AND FURTHER READING Mortality and Morbidity 1 Office for National Statistics. Childhood, Infant and Perinatal Mortality in England and Wales, 2013. http://www.ons.gov.uk/ons/rel/vsob1/child-mortality -statistics--childhood--infant-and-perinatal/2013/stb-child-mortality-stats -2013.html. 2 Countdown to 2015. 2010 Countdown to 2010 Decade Report (2000-2010). http://www.countdown2015mnch.org/reports-and-articles/previous-reports /2010-decade-report. 3 The Millennium Development Goals Report 2015. United Nations, Department of Economic and Social Affairs of the United Nations Secretariat; 2015. http://www .un.org/millenniumgoals/2015_MDG_Report/pdf/MDG%202015%20rev%20 (July%201).pdf.
4 Wang H et al. Global, regional, and national levels of neonatal, infant, and under-5 mortality during 19902013: a systematic analysis for the Global Burden of Disease Study 2013. Lancet. 2014;384:957-79.
Services for Children 1 NHS Choices. 2015. Newborn and Infant Physical Exam. http://cpd .screening.nhs.uk/nipe. 2 Knowles RL et al. Surveillance of rare diseases: a public health evaluation of the British Paediatric Surveillance Unit. J Pub Health. 2012;34:279-86.
Health Promotion 1 Piernas C et al. The double burden of under- and overnutrition and nutrient adequacy among Chinese preschool and school-aged children in 2009-2011. Eur. J. Clin Nutr. 2015; 69:1323-9. 2 Liu H, Umberson D. Gender, stress in childhood and adulthood, and trajectories of change in body mass. Soc Sci Med. 2015;139:61-9.
Infectious Diseases 1 Public Health England. 2014. Immunisation against Infectious Disease. https://www.gov.uk /government/collections /immunisation-against-infectious -disease-the-green-book 2 Public Health England. 2014. Notifications of Infectious diseases (NOIDs). https://www.gov.uk /government/collections /notifications-of-infectious -diseases-noids
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RENAL MEDICINE ZESHAN QURESHI, RACHAEL MITCHELL AND STEPHEN D MARKS
CLINICAL MANIFESTATIONS OF RENAL DISEASE Acute Kidney Injury Acute Kidney Injury (AKI) can be defined as a sudden decrease in renal function; elevated urea is followed by an elevated creatinine and a resulting decreasing glomerular filtration rate (GFR). There are often associated difficulties in fluid and electrolyte regulation as well as with blood pressure control. There are numerous definitions for AKI, but one which is widely used is the paediatric RIFLE (pRIFLE) which uses change in creatinine clearance and/or urine output for the definition (Table 1). Note that creatinine clearance is an approximation for the GFR, but is an overestimate (as creatinine is both filtered by the glomerulus, and secreted by the proximal tubule).
Aetiology The causes of acute kidney injury can be pre-renal, renal or post-renal and are summarised in Table 2.
CONTENTS 297 Clinical Manifestations of Renal Disease
297 Acute kidney Injury 299 Chronic kidney Disease 300 Hypertension 300 Nephritic Syndrome 301 Nephrotic Syndrome 304 Renal Masses
304 Renal Disorders
304 Urinary Tract Infections 307 Enuresis 307 IgA Vasculitis [also known as Henoch Schönlein Purpura (HSP)] 308 Haemolytic Uraemic Syndrome 309 Polycystic kidney Disease 310 Nephrolithiasis
311 References and Further Reading
Clinical Features Acute kidney injury can present nonspecifically, particularly in neonates with symptoms such as unexplained crying, restlessness, lethargy, vomiting, or poor feeding. Other features may include reduced urine output and pallor. Most commonly acute kidney injury is related to sepsis or hypovolaemia, in which signs of the cause would be apparent. TABLE 1: Acute Kidney Injury definitions (pRIFLE) Acute Kidney Injury
Creatinine clearance
Urine Output
Risk.
• Decrease by 1g/m2/day
Renal Medicine
Normal
ӹӹ Thyroid function tests. Hypothyroidism may result from loss of thyroxine-binding globulin. ӹӹ Full blood count (FBC). This is to look for associated anaemia.
Periorbital oedema Puffy pale face Swollen lips
Oedema. Test for the presence by showing indentation of the skin (top). The presence of periorbital oedema (bottom) is often an early sign.
Investigations Investigations involve confirming the diagnosis and the severity of the condition, and trying to assess the likely underlying pathology. ӹӹ Assess degree of proteinuria. Multiple tests can be done for this (Table 4). Early morning urine protein:creatinine or albumin:creatinine ratios are increasingly being used. These are used as the amount of creatinine excreted is relatively constant; changes in protein or albumin in relation to creatinine can therefore be a good guide to overall proteinuria. ӹӹ Lipid profile. Nephrotic syndrome is associated with hypercholesterolaemia. ӹӹ Renal function and urinary sodium. This will guide fluid balance. High urinary sodium indicates intravascular fluid depletion (which may otherwise be difficult to identify in oedematous patients).
Investigating possible causes of nephrotic syndrome should be guided by the history. Secondary causes include infection, autoimmune disorders and diabetes mellitus. Investigations may include: ӹӹ Complement level. Hypocomplementaemia with low C3 +/C4 is consistent with post-infectious GN or SLE. ӹӹ Autoantibodies. A positive antinuclear antibody (ANA) and double stranded DNA (dsDNA) points towards systemic lupus erythematosus (SLE). ӹӹ Glucose/glycated haemoglobin (HBA1c). This is to look for evidence of diabetes. ӹӹ Infection markers. This involves looking for evidence of hepatitis, Epstein-Barr virus (EBV), Varicella and human immunodeficiency virus (HIV). Percutaneous renal biopsies are required in those with atypical features, who are less likely to have minimal change disease. Such features include: ӹӹ Age 12-years-old. ӹӹ Gradual onset of oedema. ӹӹ Macroscopic haematuria. ӹӹ Rash or any features of other systemic disease. ӹӹ Hypocomplementaemia. ӹӹ Renal dysfunction. ӹӹ Steroid resistance with failure to respond to steroids by four weeks. ӹӹ Significant hypertension. ӹӹ Confirmation of calcineurin nephrotoxicity. To diagnose a relapse of nephrotic syndrome there must be at least 3+ of proteinuria on urine dipstick testing for three consecutive mornings. To diagnose remission of nephrotic syndrome, urine dipstick must be clear of proteinuria for three consecutive mornings.
Differential Diagnosis Hypoalbuminaemia has a variety of causes and could be due to: ӹӹ Poor intake. Malnutrition.
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ӹӹ Liver disease. There is loss of synthetic function, and therefore reduced albumin production. ӹӹ Excess protein loss. This can be from burns or gastrointestinal losses. ӹӹ Redistributive states (e.g. pregnancy). In such cases, there may be expansion of plasma volume which may also result in relative hypoalbuminaemia. Intermittent swelling may also point to another diagnosis, such as allergy or infection, particularly if it is very localised. Additionally, cardiac failure may present with oedema. Proteinuria is the excess of protein in the urine, usually due to leakage from the kidney, but can be due to excess proteins in the serum, or due to reduced reabsorption at the proximal convoluted tubule. Specific causes include: ӹӹ Orthostatic proteinuria. ӹӹ Glomerular disease. ӹӹ Tubular disease. ӹӹ Renal mass. ӹӹ Chronic kidney disease (with hypertension). ӹӹ Infection.
Complications ӹӹ Infection. Increased leakage through the glomeruli results in loss of regulatory proteins important in the immune system. This results in an increased predisposition to infections, particularly pneumococcal and Haemophilus infections. Pneumococcal peritonitis is a potentially devastating complication. Cellulitis is also more common, particularly if the child is markedly oedematous. ӹӹ Intravascular hypovolaemia. It is important to appreciate that patients can be oedematous, yet simultaneously intravascularly depleted. This is because the fluid is in the interstitial space, not the intravascular space. ӹӹ Hypercoagulability. Patients are more vulnerable to thrombosis, because of factors such as a) loss of anti-clotting factors, b) hyperlipidaemia and c) hyperaggregable platelets.
Management Generally, children are treated with high dose corticosteroids for four weeks, which are subsequently weaned. The length of weaning depends on the risk of side-effects versus the chance of relapse. Most cases of minimal change disease respond within four weeks. Careful monitoring of blood pressure is needed; 25% of children with minimal change nephrotic syndrome have an elevated blood pressure, which may be due to corticosteroids. Other aspects of treatment include: ӹӹ Dietary sodium restriction. This is to reduce fluid retention. High sodium can lead to hypertension and fluid overload. A no added salt diet is recommended. Note that fluid restriction can increase thrombosis risk.
ӹӹ Prophylactic penicillin. This is to reduce infection risk. ӹӹ Fluid management. This can be challenging, particularly if oedema is severe. Fluid restrictions should generally be avoided as it may lead to significant intravascular fluid depletion. Diuretic therapy and/or intravenous albumin infusions may be necessary and should be discussed with a specialist. ӹӹ Vaccination. Pneumococcal vaccination and varicella immunisation should be considered if the child is not already immune, as there is increased risk of infection secondary to immunoglobulin loss. Children with nephrotic syndrome unresponsive to corticosteroids, who have frequent relapses, or have features suspecting more serious pathology, usually require a percutaneous renal biopsy before further treatment. Immunomodulatory agents, such as mycophenolate mofetil, tacrolimus, cyclosporine and intravenous rituximab, may be considered in such cases.
Prognosis Most children who have corticosteroid-sensitive disease will go on to have more than one episode and some will have frequent relapses. However, the majority of patients go into permanent remission around puberty. Children with non-steroid responsive disease have a less satisfactory response to treatment and a worse prognosis.
Renal Masses Renal masses are uncommon, and when identified should promptly be investigated. Possible causes are shown in Table 5.
TABLE 5: Causes of renal masses Unilateral
Bilateral
• Hydronephrosis. • Renal cysts. • Renal tumour (e.g. Wilms’ tumour, which is usually unilateral). • Renal vein or venous thrombosis.
• Renal tumour. • Hydronephrosis. • Renal vein or venous thromboses. • Renal cysts. • Polycystic kidney disease. • Tuberous sclerosis.
RENAL DISORDERS Urinary Tract Infections A urinary tract infection (UTI) is defined as an infection of one or more structures in the urinary system, confirmed on urine culture. However, cases are very often treated before confirmation of a positive culture growth, if there are suggestive symptoms or positive urine dipstick results. In children, UTIs may involve upper structures (pyelonephritis) or lower structures (cystitis). Around one in 10 girls and one in 30 boys will have had a UTI before 16–yearsold. UTI is more common in girls, except for in the first six
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Renal Disorders
Aetiology Atypical UTIs (Box 3) and recurrent UTIs (Box 4) are more likely to result from an underlying abnormality. Upper UTIs are generally more serious than lower UTIs, particularly in the very young. Risk factors include: ӹӹ Constipation. A full rectum can cause pressure on the bladder and prevent emptying. Stasis of urine in the bladder increases risk of bacterial growth and predisposes to infection. ӹӹ Female gender. Structural abnormalities of the urinary tract are more common in boys; therefore, UTIs are more common in males in the first six months of life. Following this, UTIs are more common in females as their urethra is shorter, reducing the distance that bacteria need to travel to reach the bladder. ӹӹ Vesico-ureteric reflux (VUR). VUR is the backflow of urine from the bladder to the upper urinary tract through the ureter to the kidneys. It is usually due to a problem with the valve mechanism in the ureter, which usually prevents abnormal flow. The risk of UTI is increased if the urine in the bladder becomes infected as this can then track backwards. There is a risk that the infected urine may reach the kidney and thus cause pyelonephritis. ӹӹ Congenital anomalies of the kidney and urinary tract (CAKUT). May predispose to infection, for example by causing reflux, or obstruction, and urinary stasis. ӹӹ Previous UTIs.
Box 3: Atypical UTIs • Serious illness. • Poor urine flow. • Abdominal or bladder mass or spinal lesion. • Renal dysfunction (with elevated plasma creatinine and reduced glomerular filtration rate). • Septicaemia. • Failure to respond to treatment with suitable antibiotics within 48 hours. • Infection with non-E. coli organisms.
Box 4: Recurrent UTIs • Two or more episodes of UTI with pyelonephritis. • One episode of UTI with acute pyelonephritis plus one or more episodes of UTI with cystitis. • Three or more episodes of UTI with cystitis.
ӹӹ Encopresis. If a child is incontinent of faeces, bacteria from stools can contaminate the perineal area, predisposing to UTI. ӹӹ Nephrolithiasis. Obstruction of urinary flow can cause stasis of urine therefore increasing the risk of UTI. ӹӹ Uncircumcised boys. There is more likely to be bacteria living under the foreskin of the penis, predisposing to UTI.
Clinical Features UTIs can present non-specifically, particularly in neonates. The following features increase the likelihood of a UTI being present: ӹӹ Changes to the urine such as being bloody (macroscopic haematuria), foul smelling, or cloudy. ӹӹ Increased urinary frequency, including urinary incontinence, especially at night. ӹӹ Pain or burning with micturition (dysuria). ӹӹ Abdominal pain (either flank pain with pyelonephritis, or suprapubic pain with cystitis). ӹӹ Fever and rigors. ӹӹ Nausea and vomiting. ӹӹ Poor growth. ӹӹ Non-specific features in neonates (poor feeding, irritability, sleepiness, jaundice or difficulty breathing).
Renal Medicine
months of life. The most common bacterial cause is Escherichia coli. However, Proteus, Klebsiella, Enterococcus and coagulasenegative Staphylococci are other well-known causes.
Investigations Urine Tests A urine culture is the gold standard investigation to confirm a microbiological diagnosis of UTI. There are several methods of urine collection: ӹӹ Clean catch into a pot. This involves a parent or a nurse patiently waiting for a urine sample by a baby’s side. The baby’s genitals are exposed, and a urine pot is held in prime position to catch urine when it emerges (Figure 3). ӹӹ Mid-stream urine. This is the ideal way to catch urine and is done in older children/adults. The patient is instructed to go to the toilet, start passing urine, and then in the middle of the stream, catch urine in a sterile sample pot. ӹӹ Catheter (either transurethral or ultrasound guided suprapubic). This has the advantage of being able to instantly get a urine sample, to help make a diagnosis. It is also a pragmatic way of obtaining urine if a catheter is being inserted anyway. However, it is invasive, requires expertise to perform, and has the potential to introduce infection. ӹӹ Urine bag. This is rarely used, but involves putting a bag over the genitalia, so that urine is automatically caught when the baby passes it. There is a high risk of a contaminated sample due to skin commensals. White cells and nitrites on dipstick are consistent with an infection. E coli is the most commonly isolated organism, and this will be seen as gram negative rods on urine microscopy (Figure 4). Ideally, it is necessary to get a pure growth of one organism from two urine samples, but this should not delay
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reNal mediciNe
FIGURE 3
Method of clean catch urine collection.
FIGURE 4
abnormal or if there was no third trimester USS. USS should also be considered during the acute illness in any child with a severe or an atypical UTI. Two additional tests are useful in selected cases: ӹ MCUG (micturating cystourethrogram). This demonstrates evidence of posterior urethral valves in boys and/or VUR, which increase the risk of a UTI. ӹ DMSA (99mTc-dimercaptosuccinic acid) scan. This assesses presence of renal scarring, but should only be performed three to six months after a UTI. This is because acute infection changes can be misinterpreted for renal scarring (as they both appear as photopaenic areas).
Management
Gram negative rods detected by microscopy.
treatment, especially if a patient clinically has pyelonephritis.
Blood Tests An elevated white cell count and CRP may suggest infection, but blood tests are not necessary in a well child who does not have atypical features. Blood pressure and renal function should be checked if there are concerns regarding renal damage.
Renal Imaging Further imaging depends on the clinical suspicion of possible renal tract abnormalities or renal complications from the UTI. Non-recurrent typical UTIs that respond well to antibiotics do not require further investigation, unless the child is under 6-months-old. If the child is less than 6–months-old, a renal USS should be performed, especially if the antenatal USS was
Treatment in the acute setting is oral or intravenous antibiotics, depending on the age and severity of UTI. These may have to be adjusted based on urine culture and antibiotic sensitivity results. In children less than three-months-old, in the acutely unwell patient, or those who are persistently vomiting, intravenous antibiotic therapy should be used, along with additional supportive therapy.
Complications Generally, UTIs are easily identified, treated with oral antibiotics and leave no long-term complications. However, potential complications include: ӹ Sepsis. ӹ Renal abscess. ӹ Pyelonephritis. ӹ Acute kidney injury or chronic kidney disease. ӹ Hypertension. ӹ Hydronephrosis.
Prognosis The majority of children are unlikely to get recurrent UTIs after their first episode. However, recurrence is more likely in:
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Renal Disorders
Enuresis Key definitions include: ӹӹ Nocturnal enuresis or bedwetting. This describes involuntary bedwetting occurring during sleep. ӹӹ Daytime enuresis. This specifically refers to those who have reached an age where bladder control would typically have been achieved (around 5-years-old). ӹӹ Secondary enuresis. This is where a previously continent child becomes incontinent. This is always concerning and an organic cause should be excluded.
Aetiology Most affected children are physically and psychologically completely normal, particularly in younger children with nocturnal enuresis. Enuresis is more common in those with a family history. Causes of enuresis include the following: ӹӹ Bladder instability. ӹӹ Bladder neck muscle weakness. ӹӹ Neurogenic bladder. ӹӹ Anatomical abnormalities such as an ectopic ureter. For those with polyuria, there may be a urinary concentration defect. This may be due to an osmotic diuresis from diabetes mellitus or insipidus or a primary renal disorder. Other possible triggers include UTIs and constipation. The commonest cause of secondary enuresis is emotional upset. More generally, enuresis may be due to a child not responding appropriately to bladder sensation. This could be a result of maltreatment or other forms
of stress. However, it could also be due to developmental delay, or a primary neurological disorder such as spina bifida or a spinal cord abnormality.
Investigations Extensive investigations are not generally required, as the diagnosis is mainly clinical. A diary of fluid intake, bedwetting, and toileting may help clarify the history. A urine MC&S may be performed if there is a concern about a UTI, and similarly diabetes mellitus and insipidus may need to be excluded. Further renal and bladder investigations are rarely required, but may include bladder ultrasound (to look for a neurogenic bladder/incomplete emptying), spinal MRI (to look for neurological causes), and urodynamic studies (to look for obstructive/ neurological causes).
Management ӹӹ General advice. General advice should be given as to how common it is, alongside advice about fluid intake and toileting patterns. ӹӹ Reward system. A positive reward system is usually helpful, not for dry nights, but for regular toileting (especially before bed), drinking recommended amounts of fluids during the daytime (and avoiding large volumes before going to bed), and engaging in the management of the condition. ӹӹ Pelvic floor exercises. This helps to improve bladder control. ӹӹ Bell-pad alarms. These are a first line intervention. The key is that the child has to wake up to go to the toilet when the alarm goes off, not with the parents lifting their children. This may not be suitable for all families, but has excellent success rates. Sensors are placed in the bed and in the underwear of the child so that when the child starts to micturate, the alarm goes off and wakes the child. ӹӹ Desmopressin. This can be used in those in whom alarm treatment is not suitable, or for an emergency situation. This is a synthetic vasopressin, which
acts to reduce the amount of water that is excreted in urine. It should be taken one hour before bed and fluid restriction should occur from one hour before until eight hours after taking it. This may be useful when children are going away on school trips or staying over at a friend’s house, as a short term solution. ӹӹ Alternative medications. Failure of desmopressin may warrant tricyclic agents, anticholinergic agents or other specialist medications.
Renal Medicine
ӹӹ Children less than 6-months-old. ӹӹ Female gender. ӹӹ Evidence of underlying renal tract abnormalities [e.g. vesicoureteric junction obstruction, congenital anomalies of the kidney and urinary tract (CAKUT)]. ӹӹ Uncorrected predisposing factors (e.g. constipation). ӹӹ Renal abscess. ӹӹ Hydronephrosis.
Prognosis Most children outgrow bedwetting, but some do not, even with treatment. If a patient is still bedwetting at 18-years-old, they may still outgrow it with support from an experienced clinic.
IgA Vasculitis [also known as Henoch Schönlein Purpura (HSP)] Aetiology IgA vasculitis is an IgA mediated autoimmune disease known to be precipitated by infections and vaccinations, although the pathophysiology is not completely understood. It classically affects children 3 to 10–years-old and is more common in boys.
Clinical Features The most common features of IgA vasculitis are: ӹӹ Purpuric rash. A characteristic raised purpuric rash, over the buttocks and extensor surfaces of the limbs (Figure 5). The child is usually clinically very well (unlike with meningococcal disease). ӹӹ Arthralgia. Pain, which is particularly in the knees and ankles, and which is occasionally associated with periarticular oedema. It usually responds well to simple analgesia. ӹӹ Abdominal pain. Note that this can also be due to intussusception, which is a complication of the disease. The rash is pathognomonic of the disease, but very rarely, a skin biopsy is required to confirm the diagnosis.
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ӹӹ Clotting. This is to ensure there is not a coagulopathy. ӹӹ ASOT/anti DNase B. This would be elevated in post infectious glomerulonephritis. ӹӹ Early morning urine protein:creatinine ratio. This is to look for evidence of renal dysfunction. ӹӹ Complement levels. This will help, for example, in a diagnosis of post infectious glomerulonephritis. ӹӹ Immunoglobulins. These will be important to exclude an immunodeficiency. ӹӹ Autoimmune profile. This will help for example in a diagnosis of SLE.
FIGURE 5
The classical rash of IgA vasculitis (D@nderm).
Differential Diagnosis Other differentials for a purpuric rash are shown in Table 6.
Investigations All children should have the following checked: ӹӹ Blood pressure. Hypertension associated with renal impairment. ӹӹ Urine dipstick. To look for haematuria/proteinuria. ӹӹ Renal function. To identify renal dysfunction. ӹӹ Albumin. Hypoalbuminaemia associated with renal dysfunction/ nephrotic syndrome. These should be monitored in primary care until resolution. Further investigations in atypical cases include: ӹӹ FBC. This is to look for anaemia.
Children with evidence of significant renal compromise (e.g. acute nephritic syndrome, acute renal failure, persistent nephrotic syndrome) may require percutaneous renal biopsy to identify HSP nephritis.
Management Management is generally symptomatic with simple analgesia for pain (avoiding NSAIDs if renal involvement). Children should be discussed with a paediatric nephrologist if there is: ӹӹ Nephrotic range proteinuria (>1g/m2/day). ӹӹ Renal dysfunction. ӹӹ Hypertension. ӹӹ Macroscopic haematuria. Children with significant renal compromise may require immunosuppression for HSP nephritis.
Complications ӹӹ Glomerulonephritis. This is the commonest complication. It is important to check blood pressure, urine dipstick and renal function.
TABLE 6: Differential diagnosis for a purpuric rash Platelets Normal
Platelets Low
Child Well.
• HSP. • Viral infection. • Non-accidental injury.
• Immune Thrombocytopaenic Purpura.
Child Unwell.
• Meningococcal septicaemia. • Non-accidental injury.
• Acute lymphoblastic leukaemia.
ӹӹ GI involvement. Intussusception, protein-losing enteropathy, ileus, haemorrhage. ӹӹ Central nervous system involvement. This can range from self-limiting headaches and mild behavioural changes to CNS vasculitis. ӹӹ Orchitis. ӹӹ Pulmonary vasculitis.
Prognosis Initial attacks of IgA vasculitis can last several months and children may have recurrences. IgA vasculitis generally has a benign course, particularly in those that present at a young age, but renal involvement can be a serious long-term complication. Therefore all patients need to have follow up, and repeat renal function tests.
Haemolytic Uraemic Syndrome Haemolytic Uraemic Syndrome (HUS) is a triad of: ӹӹ Microangiopathic non-immune (Coombs –ve) haemolytic anaemia. ӹӹ Thrombocytopaenia. ӹӹ Acute kidney injury.
Aetiology Typical diarrhoea-associated HUS is caused by exposure to E. coli 0157, also known as Shiga Toxin Producing E. coli (STEC). Shiga toxin binds to glomerular endothelial cells, making them thrombogenic, and activating an inflammatory cascade. This can cause platelet activation, and increased von Willebrand Factor (vWF) activity, which results in microthrombi formation, and a consumptive thrombocytopaenia. The microthrombi lodge in arterioles and capillaries, with two important effects: ӹӹ Ischaemia. The kidneys and brain are most dependent on high blood flow, and therefore are most likely to be damaged. ӹӹ Microangiopathic haemolytic anaemia. The microthrombus destroys the red blood cells in its path as it becomes lodged in the smaller vessels.
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Clinical Features Diarrhoea associated (or typical) HUS is the most well recognised variant. Presentation is with bloody diarrhoea, severe abdominal pain and vomiting. Other features may include pallor, lethargy, jaundice, petechiae, convulsions, pneumonia and signs of renal injury (e.g. oliguria, fluid overload).
Investigations Haematological Tests ӹӹ FBC and reticulocyte count. Haemoglobin level will guide need for transfusion. Reticulocyte count is elevated in haemolysis. Thrombocytopaenia may be identified. ӹӹ Blood film. This is primarily to look for fragmented red cells from haemolysis. ӹӹ Group and Save/Coomb’s test. There is a high probability of transfusion being needed. A positive Coomb’s test implies possible immunemediated haemolytic disease, whereas a negative test is consistent with HUS. ӹӹ Lactate dehydrogenase (LDH). Elevated due to increased cell turnover secondary to haemolysis. ӹӹ Haptoglobins. This is reduced in haemolytic anaemia. ӹӹ Coagulation screen. This is required to rule out a coagulopathy. In HUS, the fibrinogen, D-Dimer, international normalised ratio (INR) and activated
partial thromboplastin time (APTT) are generally normal, as coagulation factors are not consumed.
Biochemistry Tests ӹӹ Renal function and electrolytes. Renal dysfunction is an important feature. Urea may also be elevated from haemolysis. ӹӹ Renal USS +/- percutaneous renal biopsy. This is to identify any renal damage, and particularly in diarrhoea negative disease, biopsy may be needed to confirm diagnosis. ӹӹ LFTs. This is to check for liver damage. ӹӹ Amylase/lipase. This is to check for pancreatic damage. ӹӹ Glucose. There may be hyperglycaemia (from pancreatic involvement) or hypoglycaemia (from infection, or liver disease). ӹӹ CRP. This is elevated from generalised inflammation and the predisposing infection. ӹӹ Urinalysis. Proteinuria and haematuria may be present.
Infection ӹӹ Stool culture. This is to look for possible initial infective cause, if diarrhoea is present. ӹӹ ASOT titres/anti-DNase B. This is to demonstrate previous streptococcal infection. ӹӹ Thomsen-Friedenreich antigen. This is to demonstrate previous pneumococcal infection. ӹӹ HIV serology. This is if HIV is suspected.
Management Management is supportive. Antibiotics should be avoided unless the patient appears clinically septic. In many countries, including the UK, HUS is a notifiable disease. Management includes: ӹӹ Renal support. Fluid resuscitation and electrolyte replacement are required. If there is significant renal dysfunction, dialysis may be necessary. Renal monitoring needs to be regular because of the risk of
deterioration. Those that progress to end-stage renal failure may require renal transplantation. ӹӹ Neurological support. Seizure prophylaxis is required if neurological signs are present. ӹӹ Haematological support. Some children may require red blood cell and/or platelet transfusion.
Renal Medicine
Beyond STEC, other pathogens, such as Shigella, have been known to cause a similar picture. HUS due to the Thomsen-Friedenreich antigen is usually associated with pneumococcal infection. There is increasing recognition of genetic predisposition to atypical HUS variants. Atypical HUS is thought to relate to chronic, uncontrolled activation of complement, leading to platelet activation, leukocyte activation and endothelial cell damage. Generally, it has a worse prognosis. Other causes include drugs (e.g. calcineurin inhibitors), malignancy, SLE and glomerulonephritis.
Historically, severe cases of HUS were treated with plasmapheresis, but intravenous eculizimab (a monoclonal antibody that inhibits complement) is beginning to prove beneficial in early clinical trials for atypical HUS.
Complications Complications are wide ranging, but may include acute or chronic renal failure, seizures, stroke, cardiac problems, hypertension and haematological abnormalities.
Prognosis Although patients can present very unwell, complete renal recovery is usual in diarrhoea associated HUS, although all forms of HUS can be associated with chronic kidney disease. HUS is rarely associated with neurological sequelae (cerebral vasculitis, cerebrovascular accidents and seizures), and these are the greatest cause of mortality.
Polycystic Kidney Disease Polycystic renal disease is inherited, either in the autosomal recessive or autosomal dominant form.
Autosomal Recessive Polycystic Kidney Disease The autosomal recessive form is often diagnosed on antenatal USS, associated with oligohydramnios and pulmonary hypoplasia (Potter sequence). The kidneys are enlarged, cystic (Figure 6), and easily palpable in the abdomen. Many infants do not survive the neonatal period due to pulmonary complications. For those that survive, renal failure, with or without hepatic failure, is inevitable in early childhood. Management is
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FIGURE 6
Further blood and urine tests are vital to further investigate to find the cause.
Imaging ӹӹ Renal USS. This is the first investigation of choice, and will identify the majority of renal stones, particularly if they are large. ӹӹ Abdominal X-ray. These may help identify radiopaque stones and stones at the PUJ. The radiopacity of a stone on X-Ray can be an indicator of its aetiology (Table 7). ӹӹ Low dose CT-DMSA. As CT is much more sensitive to tissue attenuation, almost all stones are radiopaque, although based on composition, the density varies considerably.
Blood Tests
Normal kidney (left) compared to polycystic kidney (right).
supportive, with aggressive control of hypertension and renal function. Renal transplant, with or without liver transplant, may be required in childhood.
Autosomal Dominant Polycystic Kidney Disease The autosomal dominant form of polycystic kidney disease can be detected antenatally or in childhood but more commonly presents in adulthood. The earlier the presentation, the more severe the disease. There may be multi-organ involvement, with the development of intracranial aneurysms, hepatic cysts and mitral valve prolapse. Aggressive management of hypertension and other cardiovascular risk factors is a key factor influencing disease progression. In view of the inheritance pattern, there are often difficult discussions to be had with asymptomatic family members about screening.
Renal calculi are uncommon in children, but should be considered in those presenting with abdominal pain, loin pain or haematuria. Renal calculi may also present with passage of the stone itself, or as a UTI.
ӹӹ Urea & electrolytes (U&Es). It is important to know if there is any associated renal dysfunction. ӹӹ Calcium, phosphate, magnesium, oxalate, urate, parathyroid hormone (PTH) and alkaline phosphatase (ALP). These can be an indicator for the likely type of stone present (Table 7). Note oxalate can also be measured in blood. ӹӹ Full blood count (FBC) and coagulation. These are important to ensure it is safe to carry out any form of surgical procedure.
Investigations
Urine Tests
Imaging is essential to determine if there is a renal calculus and its location.
ӹӹ Urine dipstick. This will identify the presence of any red or white
As renal calculi are rare, all children presenting with them should have a work-up to exclude urinary tract or metabolic abnormalities.
Clinical Features
TABLE 7: Aetiology of stones and their appearance on X-Ray Stone Type
Likely Aetiology
Appearance on X-ray
• Magnesium ammonium phosphate.
• Infection.
• Poor radio-opacity.
• Ammonium urate.
• Infection.
• Radiolucent.
• Calcium oxalate.
• Non-infective.
• Radio-opaque.
Nephrolithiasis
• Calcium Phosphate.
• Non-infective.
• Radio-opaque.
Aetiology
• Uric Acid.
• Non-infective.
• Radiolucent.
The most common reasons for renal stones are infection, metabolic, genetic and medication (Table 7). The commonest stones seen in children are those containing calcium-phosphate.
• Cystine.
• Genetic.
• Poor radio-opacity.
• Xanthine.
• Genetic.
• Radiolucent.
• Stones caused by drugs.
• Drug related, e.g. ciprofloxacin, loop diuretics.
• Radiolucent.
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References and Further Reading
Management Simple
Complications Complete obstruction will eventually lead to hydronephrosis and renal failure. Stones may also become infected and lead to sepsis.
Prognosis Most stones are small and pass without complications.
REFERENCES AND FURTHER READING Urinary Tract Infection
In such cases, the stone is small. Supportive measures include analgesia and increased fluid intake to help the stone to pass more quickly. Antibiotics may be given if passage of the stone takes longer or is associated with a UTI. Longterm, optimal fluid intake is recommended to prevent recurrence. There is limited evidence to suggest that any other dietary changes are of benefit.
Complicated A child with a larger stone, or one that blocks urine flow and causes great pain, may need to be hospitalised for more urgent treatment. Early referral for intervention is needed to prevent hydroureteronephrosis. These treatments include: ӹӹ Extracorporeal shock wave lithotripsy (ESWL). Shock waves are delivered to the kidney to break down the stone into smaller pieces which can be passed more easily, as shown in (Figure 7). ӹӹ Ureteroscopy. Surgical removal or lithotripsy can occur via a ureteroscope, a tube like structure which inserts into the child’s urethra and slides into the ureter via the bladder. ӹӹ Percutaneous nephrolithotomy. Surgical removal of the stone may be necessary through a small incision in the back. Occasionally lithotripsy is needed first, to break the stone down into smaller pieces that can be more readily removed.
FIGURE 7
1 Craig JC et al. Effect of circumcision on incidence of urinary tract infection in preschool boys. J Pediatr. 1996; 128:23–7. 2 Jodal U. Ten-year results of randomized treatment of children with severe vesicoureteral reflux. Final report of the International Reflux Study in Children. Pediatr Nephrol. 2006; 21:785–92. 3 McGillivray D et al. A head-to-head comparison: “cleanvoid” bag versus catheter urinalysis in the diagnosis of urinary tract infection in young children. J Pediatr. 2005; 147:451–6. 4 NICE guideline 47. Feverish illness in children: assessment and initial management in children younger than 5 years. 2007. http://www.nice.org.uk/guidance/cg47/ 5 NICE guideline 54. Urinary tract infections in children. 2007. http://www.nice.org.uk/guidance/cg54/ 6 Zorc J et al. Clinical and demographic factors associated with urinary tract infection in young febrile infants. J Pediatr. 2005; 116:644–8.
Renal Medicine
blood cells, and demonstrate the urinary pH, which is important to determine which type of stone is likely to be present. The presence of nitrites with leucocytes is suggestive of infection. ӹӹ Urine microscopy, culture & sensitivity (MC&S). All urine should be sent for culture to look for the presence of a UTI. ӹӹ Stone analysis. If a stone is passed, the urine and stone itself should be sent for analysis.
Chronic Kidney Disease 1 Hodson E et al. Growth hormone for children with chronic kidney disease. Cochrane Database of Systematic Reviews. 2012; Issue 2. http://onlinelibrary.wiley.com /doi/10.1002/14651858.CD003264.pub3/pdf. 2 Levey AS et al. A simplified equation to predict glomerular filtration rate from serum creatinine. J Am Soc Nephrol. 2011;A0828, 2000.
Polycystic Kidney Disease Kidney stones
1 Mayo Clinic. Normal and Polycystic Kidneys. 1998-2015. http://www.mayoclinic.org/diseases -conditions/polycystickidneydisease/multimedia /normal-and-polycystic-kidneys/img-20006896.
Glomerulonephritis 1 Infokid Website. Glomerulonephritis. 2013. http://www .infokid.org.uk/glomerulonephritis 2 UNC Kidney Center. Glomerular disease. 2012. http://unckidneycenter.org/kidneyhealthlibrary /glomerular-disease Mechanism of action of lithotripsy.
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Kidney Stones 1 National Institute of Health. Medline Plus: Lithotripsy Procedure. 2011. http://www.nlm.nih.gov/medlineplus /ency/imagepages/19246.htm 2 European Association of Urology. Guidelines on Urolithiasis. , 2014. http://uroweb.org/wp-content /uploads/22-Urolithiasis_LR.pdf.
Enuresis 1 NICE Quality Standard 70. Nocturnal enuresis (bedwetting) in children and young people. 2014. http://www.nice.org .uk/guidance/qs70
vero-cytotoxin producing Escherichia coli in children. 2012. http://webarchive.nationalarchives.gov.uk/20140714084352 /http://www.hpa.org.uk/webc/HPAwebFile/HPAweb _C/1309968515827. 2 Medscape. Paediatric HUS. http://emedicine.medscape .com/article/982025-overview.
Miscellaneous 1 National Kidney Foundation. KDOQI Guidelines. 2002. http://www2.kidney.org/professionals/KDOQI/guidelines _ckd/toc.htm.
HUS 1 The Health Protection Agency. The management of acute bloody diarrhoea in children potentially caused by
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1.22
RESPIRATORY MEDICINE CHRISTOPHER GRIME
RESPIRATORY DISORDERS Asthma Asthma is a clinical diagnosis characterised by reversible airflow limitation.
Aetiology
CONTENTS 313 Respiratory Disorders
313 Asthma 317 Preschool Wheezing Disorders 318 Bronchiolitis 319 Pneumonia 321 Cystic Fibrosis 323 Pertussis (Whooping Cough) 324 Tuberculosis 325 Primary Ciliary Dyskinesia 326 Bronchiectasis 327 Pneumothorax
327 Important Adjuncts in Respiratory Management
327 Tracheostomies 328 Oxygen Therapy
329 References and Further Reading
Three main features give rise to the asthmatic phenotype: ӹӹ Airway hyperresponsiveness. This is an exaggerated bronchial smooth muscle contraction to a wide range of stimuli. The triggers vary according to the individual, but may include cold air, house dust mites or smoke. ӹӹ Bronchial inflammation. There is widespread inflammation in the bronchioles, with infiltration by eosinophils, T lymphocytes and mast cells. This is associated with oedema, smooth muscle hypertrophy, mucus plugging and epithelial damage. Some of these changes may be chronic but they are more pronounced during an asthma exacerbation. ӹӹ Airflow limitation. This is usually reversible, either spontaneously or with treatment, but there may be some underlying chronic changes. Asthma is generally characterised by classical helper T cell type 2 (Th2) pathology with increased cytokines such as interleukin 4, 5 and 13 (IL-4, IL-5 and IL-13), which are thought to drive symptoms. The pathology of asthma is characterised by goblet cell hyperplasia and infiltration of inflammatory cells such as CD4+ T cells, eosinophils and mast cells.
Clinical Features As with most conditions, a clear clinical history is often all that is needed, particularly with young children. Symptoms are induced by weather changes, ill health and exercise. Nocturnal symptoms and improvement on bronchodilation are even more suggestive of the disease. ӹӹ Wheeze. Classically, in asthma, the wheeze is an expiratory airflow sound resulting from narrowed and inflamed airways. It can be heard at the bedside or require auscultation. However, the volume of the sound can be misleading. A
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ӹӹ
ӹӹ
ӹӹ
ӹӹ
ӹӹ
quieter wheeze may be suggestive of severely narrowed airways. Paradoxically, in this setting, the volume of the wheeze would increase in response to bronchodilation. Coughing. Bronchoconstriction is often accompanied by severe bouts of coughing. For some children, this can be the main symptom, particularly at night. Shortness of breath. Poorly controlled asthma in a child may present with shortness of breath, showing increased respiratory effort on minimal exertion. Exercise induced symptoms. Children will often have exacerbations while exerting themselves. It is good practice to have a reliever inhaler nearby during sport and for some children, to administer bronchodilators before exercising. Atopic nature. Most childhood asthma is associated with an element of atopic disease such as eczema, rhinitis, hay fever or a family history of atopy. Managing these diseases will often improve asthma symptoms. Chest wall remodelling. Rarely, in severe disease, children can develop pectus carinatum (protrusion of sternum and ribs, also known as
pigeon chest). Harrison’s sulcus (a horizontal groove along the costal margin) may also be seen in chronic disease.
children over 5–years-old, when lung function can be measured. ӹӹ Allergy test. Skin prick test to aeroallergens or specific IgE to allergens suggests an atopic nature, which could be useful when considering aggravating factors in the environment.
Examination findings include: ӹӹ Wheeze. Be aware that this may be absent in severe disease, where there is insufficient air flow to generate wheeze. ӹӹ Reduced air entry. ӹӹ Hyperexpanded chest. ӹӹ Use of accessory muscles/increased work of breathing.
If very severe and referred to a tertiary centre, a child may undergo further tests such as a bronchoscopy, formal lung function test and exhaled nitric oxide test (found to be increased with inflammatory conditions) before more novel therapies are considered.
Investigations The combination of classic symptoms with demonstrable variable airflow obstruction make the diagnosis. ӹӹ Obstructive pattern to lung function. Asthma is an obstructive lung disease demonstrated on lung function by showing a reduced forced expiratory volume in one second (FEV1) with relatively preserved forced vital capacity (FVC) and a reduced FEV1/FVC (40mm, it is most likely that the child will require surgery. ӹӹ If the AP diameter is 38°C, and C. Predisposing cardiac condition. Infective endocarditis is an infection of the heart valves and/ or endocardium, often associated with valvular vegetations and/or thrombus formation. It is notoriously difficult to diagnose and, although rare in children, should be excluded in a child with pyrexia of unknown origin. Clinicians use the modified Duke’s criteria to guide diagnoses of infective endocarditis (p31). Although a recent dental procedure may be an event during which bacteria enter the blood stream, it is not a criteria for diagnosis. Splinter haemorrhages occur from micro-emboli originating from the cardiac vegetation. Although often present in infective endocarditis, they are rare in children, are non-specific and do not alone form a criterion for diagnosis.
3
Accurate diagnosis often with limited, non-specific clinical history available, particularly in neonates. Awareness that this may be the first presentation of congenital anomalies of the kidneys or urinary tract, such as dysplastic kidneys, vesico-ureteric reflux or posterior urethral valves that increase the risk of infection. Careful consideration of potential complications of infection such as renal scarring.
This patient has clear evidence of a UTI, as evidenced by two pure growths of E. coli in the urine, with an associated elevated urinary WCC. It is an atypical urinary tract infection, because although this organism is a common cause of UTIs, the patient has failed to respond to antibiotics after 48 hours. NICE guidelines (p306) would suggest that this scenario requires an acute USS, and both an MCUG and a DMSA scan should be strongly considered once the acute infection has resolved. A MCUG – Incorrect. This contrast scan evaluates the presence of structural abnormalities (such as posterior urethral valves) and vesico-ureteric reflux. In an atypical UTI, it is important to consider vesico-ureteric reflux as the cause, as it may increase the risk of future infections. However, it should be performed after the acute infection has resolved, as it will not significantly alter acute management.
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B DMSA – Incorrect. This radionuclide scan evaluates renal function. It can be used acutely to delineate pyelonephritis from cystitis, but acute changes can resolve as the infection is treated. The recommendations are to wait for three to six months following acute infection to evaluate potential renal scarring. C Renal USS – Correct. USS will show anatomical abnormalities, such as hydroureteronephrosis (which may be due to posterior urethral valves in boys, for which an ablation procedure would be needed). Prophylactic antibiotics may also be initiated, depending on the abnormality identified. D Abdominal X-ray – Incorrect. This is not indicated in further evaluation of a UTI, although it may potentially be useful in detecting radiolucent calculi if nephrolithiasis is suspected. E CT-KUB – Incorrect. Although a CT-KUB would provide sophisticated imaging of the kidney and the renal tract, the radiation exposure limits its use to isolated cases of suspected renal trauma or renal calculi.
Key Point UTIs in children less than 3-months-old or acutely unwell should be taken very seriously and they invariably need admission to hospital, intravenous antibiotics and further investigations. Male infants with posterior urethral valves can present with UTIs, although some cases are diagnosed in utero due to antenatal ultrasound scanning. Q2 Which of the following would most strongly suggest an atypical UTI (rather than a typical UTI)? The correct answer is D. Abdominal or bladder mass. It is important to evaluate whether an infection is an atypical UTI, since this may mean more serious pathology and suggest a need for further evaluation. A Failure to respond to 24 hours of antibiotics – Incorrect. A 48-hour trial of antibiotics would be needed before determining an atypical infection, as it can take up to two days to see a response to therapy. B A pure growth of E. coli (105 organisms/mL) – Incorrect. E. coli is frequently seen in UTIs; therefore, isolating E. coli by itself does not merit further investigation. C A mixed growth of organisms in two separate urine samples – Incorrect. A mixed growth indicates a likely contaminant; therefore, if a UTI is still suspected, another urine sample should be sent. Ensure that this is a clean catch sample to minimise the risk of contamination again and that the sample is taken directly to the microbiology laboratory without delay. This is a common problem with primary care or out-of-hours samples, as the delay between collection and analysis can lead to artefactual increases in organism counts.
D Abdominal or bladder mass – Correct. This indicates possible significant pathology. For example, the mass could be a multicystic dysplastic kidney, severe hydronephrosis or an unusual tumour. E CRP of 64 mg/L – Incorrect. A high baseline CRP does not suggest an atypical infection. However, it is a useful marker of response to treatment on subsequent blood tests. Other concerns of an atypical UTI would be renal dysfunction, as this indicates either baseline renal pathology or acute kidney injury secondary to the UTI.
Key Point Take the time to ensure that the best possible urine sample is collected. In older children, a mid-stream urine sample should be collected. In babies, a clean catch sample should be collected, or if that fails an “in-out catheter”, or a suprapubic aspiration under ultrasound guidance can be used. Once the sample is collected, it should be sent to the laboratory as soon as possible to avoid inaccurate results. Q3 Which of the following increases the likelihood of a urine sample being contaminated? The correct answer is C. “Pad” sample being taken (compared to a clean catch sample). Assessing whether a urine sample shows an infection or is a contaminant requires evaluation of the following: a) Mechanism by which the sample was obtained b) Presence of WBC and epithelial cells in the urine c) Quantity and name of the organism(s) that grow(s) on culture d) Replication of result by a second urine sample taken concurrently e) Time taken to get the sample to the laboratory. In general, a clean catch sample, with no epithelial cells, an elevated WCC, leukocytes/nitrites on a dipstick and a pure growth of one organism in multiple urine bottles will strongly suggest a UTI. A No epithelial cells reported on microscopy (compared to epithelial cells being present) – Incorrect. Epithelial cells are contaminants that should not normally be present in significant amounts in the urine. Therefore, if epithelial cells are present, the sample is more likely to be contaminated. B WCC 3+ being reported on microscopy (compared to white cells not being present) – Incorrect. An elevated WCC suggests an inflammatory response in the urine, which could potentially be caused by a UTI. It can also be a sign of glomerulonephritis.
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ANSWERS
Key Point The urine dipstick itself is less reliable in babies than it is in adults. First, it is more difficult to get clean catch specimens, and other methods of obtaining urine are liable to contamination. Second, nitrites are less likely to be positive in children who are not yet continent of urine. This is because in order for bacteria to produce nitrites, urine has to be held in the bladder for at least 30 minutes. Therefore, initial microscopy results are very important to consider. IMPORTANT LEARNING POINTS
• UTIs are usually mild and respond well to short courses of oral antibiotics. • The diagnosis can only be confirmed by urine cultures, although be wary of misleading results due to contamination. Clean catch specimens minimise the risk of false positives. • For inpatients, it is important to follow up the results of any urine culture tests. It is very common for these results to be missed, leading to patients continuing on ineffective antibiotics, despite a test result demonstrating antibiotic resistance. • In cases with severe, recurrent or atypical infections, be aware of possible complications and the need for further investigations.
ANSWER TO CASE 36
A 9-year-old boy presenting with primary nocturnal enuresis Q1 Which of the following would you want to do in further assessing this child? The correct answers are A. Full abdominal examination, B. Ask about John’s fluid intake, C. Plot height and weight, D. Dipstick urine, and E. Take a social history and find out how John is getting on at school. Nocturnal enuresis or bedwetting describes the involuntary wetting that can occur during sleep. The causes of bedwetting are not fully understood. They can include sleep arousal difficulties, polyuria and bladder dysfunction, and often bedwetting can run in families. Bedwetting has a negative impact on self-esteem; therefore, its treatment is very important for both the physical and mental wellbeing of the child.
[ CHAPTER CHAPTER Clinical TITLE Cases: Standard MARKER ]
C “Pad” sample being taken (compared to a clean catch sample) – Correct. Pad samples are liable to contamination from the perineal area and from the genitalia, although sterile pads which are removed as soon as micturition has occurred reduce the risk of contamination. D Pure growth of a single organism (compared to mixed growth of several organisms) – Incorrect. This is suggestive of an infection with a single organism; therefore, this makes a contaminant less likely. Contamination from skin/faeces usually contain multiple organisms: this reflects the organisms normally present at that site. E The same organism grown in two separate urine samples (compared to being grown in just one sample) – Incorrect. The same contaminant is unlikely to grow in two separate “contaminated” samples; therefore, this makes a contaminant less likely. F The presence of both nitrites and leukocytes on a dipstick (compared to neither or just one) – Incorrect. Leukocytes and nitrites increase the likelihood of a UTI being present, particularly when both are positive.
A Full abdominal examination – Correct. This would be an important step in assessing the child for organic causes. Although the child may claim to have a normal bowel habit, palpable faeces may indicate constipation as the underlying cause of urinary incontinence. B Ask about John’s fluid intake – Correct. It would be important to do this, to check that John is not drinking excessively at night, or that he is not restricting his fluid intake in an attempt to stop the bedwetting. Fluid intake should be adequate and should vary depending on time of day, ambient temperature and amount of physical activity. Regular toileting should be encouraged and children should avoid caffeinated drinks. C Plot height and weight – Correct. This is important to do during any consultation with a child. Growth is an important marker of general well-being. It can sometimes be the first indicator that something is wrong, and there may be an organic secondary cause. D Dipstick urine – Correct. It is helpful to perform a urine dipstick to ensure there is no evidence of renal disease. E Take a social history and find out how John is getting on at school – Correct. This is fundamental in this scenario. Bedwetting can be the first sign of bullying, problems at school or maltreatment.
Key Point Primary nocturnal enuresis is common and usually does not have an organic cause, particularly in younger children. In contrast, secondary enuresis (where a previously continent child becomes incontinent) should always be thoroughly investigated.
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Clinical Cases: Standard
Q2 John’s mother is very keen to work hard to get his problems under control. There are no problems at school and John’s family is very supportive. Assuming that all the initial investigations are normal and John is clinically well, what would be the first line management for John? The correct answer is E. Referral to an enuresis clinic for a bell pad alarm. Treatment for bedwetting should focus on the fact that it is not the child’s fault. Drinking habits should be reviewed. Bell pad alarms/reward systems are extremely effective, and desmopressin may be effective medical therapy in selected cases. A Desmopressin – Incorrect. In the case of John, whose mum seems keen to try other measures, desmopressin is not the first-line treatment. It can be used for social reasons (such as sleepovers), or in children where other treatment has failed, older children, or if the other treatment options are not suitable for the parents. B Referral for counselling – Incorrect. In children with underlying psychological disturbances, or in whom bedwetting is secondary to maltreatment, counselling and other forms of support may be necessary. The question clearly states, however, that this is not an issue for John. C Fluid restriction – Incorrect. Adequate fluid intake is important and fluid restriction, even in the evening, should be avoided. Regular toileting should be encouraged and children should avoid caffeinated drinks. D Trimethoprim to treat presumed UTI – Incorrect. There were no features to suggest infection in this scenario. E Referral to an enuresis clinic for a bell pad alarm – Correct. In families where this is acceptable, this would be the first line treatment, combined with reward systems. The key is that the child has to wake up to go to the toilet when the alarm goes off — parents should not be lifting their children. This would not be suitable for all families, but in those who are willing, there are excellent success rates. Sensors are placed in the bed and in the underwear of the child so that when the child starts to wet, the alarm goes off and wakes the child.
Key Point For nocturnal enuresis, desmopressin may be required, but generally conservative measures such as bell pad alarms and reward systems are effective.
IMPORTANT LEARNING POINTS
• Enuresis is common, particularly nocturnally, and in younger children. • Although secondary enuresis may have a secondary cause, primary enuresis is something children usually grow out of. • First-line treatment for nocturnal enuresis usually involves conservative measures, such as reviewing drinking habits, regular toileting and bedside alarms. This is extremely effective.
ANSWER TO CASE 37
A 3-year-old girl presenting with bloody diarrhoea and anuria Q1 What would you do first in this scenario? The correct answer is A. IV fluid resuscitation. This child sounds very unwell and is in hypovolaemic shock secondary to gastroenteritis. The initial management of an acutely unwell patient is always the same: resuscitate using an airway, breathing and circulation approach. The question tells you that her airway is patent by proxy of the fact that she is responding to her mother. “B” has already been assessed and she is on oxygen. The next step of management is “C” – circulation. A IV fluid resuscitation – Correct. The question clearly states that she is peripherally shut down, tachycardic and anuric. A 20 mL/kg fluid bolus is needed to try to restore circulating volume. She should be monitored carefully for signs of fluid overload. In the presence of renal dysfunction, care must be taken in administering further fluid boluses. B IV antibiotics – Incorrect. The fluid bolus is the priority, but IV antibiotics should be considered in any shocked child with potential septicaemia. However, there is evidence of worsening prognosis by giving antibiotics in HUS, which is a potential diagnosis at this stage. C Oral fluid challenge – Incorrect. Although fluids are what this child needs, an oral fluid challenge would not act quickly enough; therefore, IV fluids should be given. D Paracetamol – Incorrect. Managing pain is less important than treating hypovolaemia. E Surgical opinion for her abdomen – Incorrect. This child has probable gastroenteritis and requires fluid resuscitation. There is nothing to suggest that there is any other intraabdominal pathology requiring surgical intervention, but a detailed abdominal examination is needed. However, even if the abdominal examination revealed surgical concern (e.g. clinical suspicion of perforation), in the first instance, stabilisation with a fluid bolus should be commenced immediately.
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ANSWERS
The management of any acutely unwell child, regardless of the possible underlying diagnosis, should start with resuscitation, using an ABC approach. Q2 What is the most likely diagnosis? The correct answer is E. Haemolytic Uraemic Syndrome. This anuria is consistent with acute kidney injury. Of note, there are also low platelets and the history of a recent visit to a farm certainly raises the possibility of E. coli 0157 infection. This picture of severe gastroenteritis with bloody diarrhoea, acute renal failure and thrombocytopenia is classic of HUS. A Glomerulonephritis – Incorrect. The history is not typical here and would be unlikely to present so acutely, with a shocked child in acute renal failure. B Henoch-Schönlein Purpura – Incorrect. The child with HSP is typically very well with normal renal function. HSP is also associated with abdominal pain, arthralgia and a typical purpuric rash. C Chronic kidney disease – Incorrect. Although we cannot definitely say that the renal failure is new, the presentation is acute and secondary to HUS. Of note, the urea is elevated in proportion to the creatinine, again suggesting a more acute picture. The low haemoglobin may be a result of the microangiopathic haemolytic anaemia seen in HUS. D Gastroenteritis – Incorrect. Although, superficially, this child does have gastroenteritis, there are clues in the history
that this is more complicated. Pure gastroenteritis would be unlikely to cause this degree of renal failure, and it does not cause thrombocytopaenia. This, combined with the possible E. coli 0157 exposure, makes HUS the most likely overriding diagnosis. E Haemolytic Uraemic Syndrome – Correct. This is a rare condition that tends to occur in outbreaks due to exposure to E. coli 0157, often following exposure to infected farm animals.
Key Point Patients with HUS usually present as severely unwell. Suspect this diagnosis particularly if there has been possible contact with E. coli 0157. IMPORTANT LEARNING POINTS
• HUS is defined by the triad of acute kidney injury, thrombocytopenia and microangiopathic haemolytic anaemia. It typically presents with bloody diarrhoea, abdominal pain and vomiting.
[ CHAPTER CHAPTER Clinical TITLE Cases: Standard MARKER ]
Key Point
• It is a rare diagnosis, and gastroenteritis is a much more common cause of bloody diarrhoea. The important clues are on the blood test results (renal function, platelet count, haemoglobin), and the severity of the illness. • Management is generally supportive, with fluid and electrolyte replacement. Referral to a specialist unit is required, as the child may require dialysis and intensive monitoring. In severe cases, seizure prophylaxis and blood products may be required.
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2.02
CLINICAL CASES: INTERMEDIATE ALL AUTHORS
CONTENTS 439 Case 1
443 Case 10
A 5-year-old girl presenting with an acute neck swelling
439 Case 2
445 Case 11
A 6-hour-old girl presenting with bilious vomiting
A 10-year-old boy with newly diagnosed diabetes
A 3-year-old boy presenting with possible appendicitis
440 Case 3
445 Case 12
A 14-year-old girl presenting with delayed puberty
441 Case 4
A 12-day-old baby presenting with poor feeding and vomiting
441 Case 5 A 5-year-old boy presenting with a headache
442 Case 6
A 13-month-old girl presents with severe pallor
442 Case 7 A 4-year-old presenting with thrombocytopenia
443 Case 8
A 4-month-old boy presenting with severe respiratory distress
443 Case 9 A 1-year-old boy presents with an itchy rash
A baby born at 32 weeks gestation
445 Case 13 A n 11-month-old presenting with possible developmental delay
446 Case 14
A 22-month-old presenting with delayed gross motor skills
446 Case 15 A 5-day-old baby with a genetic condition picked up on newborn screening
447 Case 16 A 9-year-old boy presenting with tight foreskin
448 Case 17
A 2-year-old presenting with bony deformities
448 Case 18
A 12-month-old presenting with faltering growth
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QUESTIONS
450 Case 22
An 11-year-old boy presenting with haematuria
A 2-year-old presenting with a blue episode
449 Case 20
451 Case 23
A 5-week-old girl presenting with respiratory distress and poor weight gain
A 5-year-old girl presenting with a purpuric rash
450 Case 21 A 4-year-old boy presenting with nephrotic syndrome
CASE 1
A 5-year-old girl presenting with an acute neck swelling
A
previously fit and well 5-year-old girl is reviewed in ED with a progressively enlarging, left-sided neck swelling. She reports having a sore throat and has a fever of 38°C, but observations are otherwise normal.
Questions Q1 What clinical feature(s) below would be of concern and prompt further investigation? A Night sweats B Coryzal symptoms C Hepatosplenomegaly D Stridor E Weight loss Q2 On examining the child, what finding(s) would suggest acute infection? Select all that apply. A Overlying erythema B Multiple, bilateral cervical lymph nodes. C Solitary, painless neck mass D Fluctuant mass E Fever Q3 If a non-infective pathology is suspected, which three of the following should be organised as first-line investigations? A MRI scan of the neck B Ultrasound scan C Blood film D FBC E Excision biopsy Q4 With regard to neck masses in children, mark the following statements true or false. A The most common histological feature on biopsy is reactive hyperplasia
B Most palpable lymph nodes over 0.5 cm will require an excision biopsy C Branchial cysts most commonly present in the neonatal period D Supraclavicular swellings are common in children E Neck swellings are rarely associated with infectious mononucleosis
Clinical cases: Intermediate
449 Case 19
For answers see page 452
CASE 2
A 10-year-old boy with newly diagnosed diabetes Jamie is a sporty and active 10-year-old. His mother noticed that recently his clothes have seemed too big for him, and he was always complaining of feeling tired. Jamie was very embarrassed to admit that he had wet the bed on two occasions this week. Observations are within normal limits. On examination, Jamie is very thin, with signs of recent weight loss. Jamie’s urine dipstick shows 4+ glucose, 2+ ketones, 1+ leucocytes and is negative for nitrites. A new diagnosis of diabetes is suspected.
Questions Q1 Which of the following best fits with a diagnosis of Type 1 diabetes in this child? A A high blood glucose, only if it is seen following a formal oral glucose tolerance test B A random blood glucose >11.1 mmol/L C The presence of polyuria and polydipsia D 4+ of glucose on urine dipstick E The presence of ketonuria Q2 Regarding the aetiology of diabetes, mark the following statements true or false. A All diabetes in children is type 1 B Type 1 diabetes mellitus is an autoimmune condition caused by damage to pancreatic alpha cells
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C Pancreatic damage is mediated by T lymphocytes D Type 1 diabetes can be associated with other autoimmune conditions such as Addison’s disease, coeliac disease and hypothyroidism E HLA-DR3 and HLA-DR4 are associated with an increased risk of developing diabetes At hospital, it is confirmed that Jamie does have diabetes. He has a blood glucose level of 26 mmol/L. He is admitted to the paediatric ward to establish his insulin regime and to allow his mother to learn important principles for management of his condition. He is then discharged home on the same day and then regularly visited at home by the community nurses over the next few weeks. Q3 Regarding insulin, mark the following statements true or false. A Insulin is usually derived from the pancreas of pigs B Injections must be confined to a certain location to ensure a consistent dose-response relationship C Insulin is usually given as one injection per day D Most children will require relatively small amounts of insulin in the months following diagnosis E Insulin should be given to keep blood glucose within the normal range at all times
Time of day
Breakfast (08:00) Pre
Mon.
Tues.
Wed.
Q4 In addition to insulin administration, which of the following will also cause reductions in blood glucose? Select all that apply. A Growth hormone secretion B Glucagon C Use of corticosteroid medication D Consumption of alcoholic drinks E Febrile illnesses A few weeks after discharge, Jamie is awarded “man of the match” in his five-a-side team game, but his mother notices he appears withdrawn and quiet on the way home and he says he feels “funny”. She checks his blood glucose and finds it is 2.8 mmol/L. Q5 Which would be the next most appropriate action to manage Jamie’s hypoglycaemia? A IM glucagon B Offer Jamie a sugary drink C Withhold his insulin for the rest of the day D Call an ambulance E Give him a sandwich It is now one year since Jamie’s diagnosis, and he is making good progress, with no other emergency presentations to hospital. He is now giving some of his injections by himself
Lunch (13:00) Post
Pre
Dinner (18:00) Post
Pre
Post 7.2
Blood Glucose
11.7
4.1
3.8
Insulin Dose (units)
4 (short acting)
5 (short acting)
6 (short acting)
Blood Glucose
10.1
6.1
Insulin Dose (units)
5 (short acting)
6 (short acting)
Blood Glucose
12.9
Insulin Dose (units)
5 (short acting)
For answers see page 453
10 (long acting)
3 (short acting) 6.0
4 (short acting)
Q6 What conclusion can you draw from the above glucose readings? (normal range 4-7 mmol/L) A Jamie must be snacking overnight B Jamie should skip breakfast C Checking Jamie’s blood glucose overnight for few nights will be helpful D Jamie should cut out sugary food from his diet
5.8
7.1
6.1
with his mother’s supervision. He is seen in clinic by his consultant for his annual review.
Bedtime (21:00)
11.9 5 (short acting)
11 (long acting) 9.0 12 (long acting)
CASE 3
A 14-year-old girl presenting with delayed puberty A 14-year-old girl has primary amenorrhoea. On further questioning, the girl says she is tired and feels cold all the time. Her general development is normal and she attends mainstream school. She is an academically able child and is expected to do very well in her exams the following year. Observations are within normal limits. On examination, she is on the 50th centile for height, her BMI is 16.5 kg/m2 (11.1 mmol/L. Type 1 diabetes is diagnosed by a random blood glucose, with a formal oral glucose tolerance test rarely being required. Children with Type 1 diabetes are also usually symptomatic at the time of diagnosis. Type 2 is becoming increasingly common amongst children, as the prevalence of obesity is rising. A A high blood glucose only if it is seen following a formal oral glucose tolerance test – Incorrect. A formal glucose tolerance test is rarely required to make the diagnosis in children, but can be performed in borderline cases. B A random blood glucose >11.1 mmol/L – Correct. According to WHO criteria, diagnosis can be made in a symptomatic child such as Jamie, with a random plasma blood glucose >11.1 mmol/L. This is not to indicate that any child with a blood glucose >11.1 mmol/L should be considered to have diabetes. Physiological stress, such as that encountered during an acute asthma attack, or gastroenteritis with release of endogenous corticosteroids, can induce transient hyperglycaemia, even in children who do not have diabetes. C The presence of polyuria and polydipsia – Incorrect. Although excessive urination and thirst should lead to consideration of diabetes mellitus, this is not diagnostic
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and other conditions such as habitual drinking and diabetes insipidus must also be considered. D 4+ of glucose on urine dipstick – Incorrect. This indicates that the renal threshold of reabsorbing glucose has been exceeded; however, this is not a diagnostic criterion for diabetes. E The presence of ketonuria – Incorrect. This also occurs during fasting states, not solely in diabetes.
Key Point Diabetes in children and young people is usually diagnosed by means of a clinical history, and a random blood glucose. A formal OGTT is rarely required. Q2 R egarding the aetiology of diabetes, mark the following statements true or false. A All diabetes in children is type 1 – False. The majority of cases are type 1; however, type 2 diabetes is increasing in prevalence, particularly in obese and south Asian populations. B Type 1 diabetes mellitus is an autoimmune condition caused by damage to pancreatic alpha cells – False. Type 1 diabetes is an autoimmune condition due to autoimmune damage to the insulin secreting beta cells of the pancreas. Alpha cells are responsible for secretion of glucagon, which raises blood glucose. The clinical syndrome of diabetes is only seen once over 80% of beta cells have been destroyed. C Pancreatic damage is mediated by T lymphocytes – True. Beta cells in the pancreas are damaged by T lymphocytes. D Type 1 diabetes can be associated with other autoimmune conditions such as Addison’s disease, coeliac disease and hypothyroidism – True. Children are screened for these conditions following diagnosis. E HLA-DR3 and HLA-DR4 are associated with an increased risk of developing diabetes – True. HLA-DR2 and DR5 appear to be protective. It has been determined that the HLA region on chromosome 6 is the predominant factor in determining genetic predisposition to developing type 1 diabetes.
Key Point Type 1 diabetes is a condition resulting from immune mediated destruction of pancreatic beta cells. It is of multifactorial causation, with environmental factors inducing the clinical syndrome in those with a genetic susceptibility. Q3 R egarding insulin, mark the following statements true or false. A Insulin is usually derived from the pancreas of pigs – False. Insulin is usually synthetically produced. Most children are
on insulin analogues where the insulin molecule is slightly modified in order to achieve faster (rapid-acting analogues) or slower (long-acting analogues) action. B Injections must be confined to a certain location to ensure a consistent dose-response relationship – False. Rotation of injection sites is an important practice. This avoids complications such as lipohypertrophy or lipodystrophy. Rates of absorption vary between sites, with quickest absorption being from the adipose tissue of the abdomen and slowest from the thighs. C Insulin is usually given as one injection per day – False. A combination of insulin types will be required, with the usual regime consisting of a long acting insulin to suppress gluconeogenesis (e.g. Lantus) and shorter acting boluses (e.g. Novorapid) to cover meals. Most children will be on regimes requiring 3-4 separate daily injections. D Most children will require relatively small amounts of insulin in the months following diagnosis – True. This is known as the “honeymoon period”, as beta cell function has not yet been completely exhausted. Children usually require 1unit/kg/day of insulin in the long term; however, approximately 3-6 weeks following initiation of treatment, their blood glucose may be manageable on as little as 0.5units/kg/day. E Insulin should be given to keep blood glucose within the normal range at all times – False. Overly tight control of blood glucose increases the risk of hypoglycaemia. Therefore, mild hyperglycaemia is tolerated acutely.
Key Point Insulin requirements are determined by a combination of residual beta cell function, glucose consumption and energy expenditure. Q4 In addition to insulin administration, which of the following will also cause reductions in blood glucose? The correct answer is D. Consumption of alcoholic drinks. Maintaining glucose levels requires careful attention not just to diet, but also health and lifestyle choices. Any new medications (e.g. corticosteroids) may have an effect on glucose levels or the effect of insulin, and this needs to be considered when adjusting doses. A Growth hormone secretion – Incorrect. GH is a counter regulatory hormone, with actions opposing insulin, increasing blood glucose concentration. GH secretion increases during puberty, accounting for increased insulin requirements at this stage. B Glucagon – Incorrect. Glucagon is secreted by the alpha cells of the pancreas. It causes glycogenolysis and gluconeogenesis, thus increasing glucose levels.
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ANSWERS
Key Point Young people will transition into a more active role in the management of their condition as they become more mature and independent. It is important that they are educated on an ongoing basis about factors which affect their blood glucose to enable them to make well informed lifestyle choices. Q5 W hich would be the next most appropriate action to manage Jamie’s hypoglycaemia? The correct answer is B. Offer Jamie a sugary drink. Hypoglycaemia can be a medical emergency, but in a well child, oral glucose is the first line management. A IM glucagon – Incorrect. In an alert, conscious and cooperative child, this is not the recommended first step. Glucagon may be the next appropriate action if Jamie was unable or unwilling to take anything orally. B Offer Jamie a sugary drink – Correct. This can be repeated after 10-15 minutes if there are ongoing concerns. C Withhold his insulin for the rest of the day – Incorrect. This would be dangerous. Blood glucose levels should be closely monitored, with appropriate adjustment of insulin doses. D Call an ambulance – Incorrect. This may be appropriate if, for example, there were concerns regarding Jamie’s level of consciousness. Additionally if a child significantly overdoses on insulin, or if they ingest excess oral hypoglycaemic agents, urgent medical review would be required. A glucose infusion may be required. E Give him a sandwich – Incorrect. Simple carbohydrates, such as juice, glucose gel or jam, are the priority as they immediately raise blood glucose. Complex carbohydrates, such as bread, should be given after this, to help maintain the blood glucose level.
Key Point Most children with diabetes will suffer a hypoglycaemic episode at some point. Recognition of symptoms and sensible stepwise management protocols are essential principles that families will need to learn to manage their child’s diabetes safely. First-line management in a well child is oral simple carbohydrates, like a sugary drink.
Q6 What conclusion can you draw from the above glucose readings? The correct answer is C. Checking Jamie’s blood glucose overnight for few nights will be helpful. Maintaining a diary of blood glucose recordings can be an invaluable tool for troubleshooting, particularly in cases where glycaemic control appears to be suboptimal. Occasionally, a continuous glucose sensor can be inserted under the skin to measure interstitial glucose for few a days. The most common indications for this are: frequent/ unpredictable hypoglycaemic episodes and hypoglycaemia unawareness. A Jamie must be snacking overnight – Incorrect. Jamie has high blood glucose readings prior to eating in the morning, and this can be for a variety of reasons apart from food intake overnight. Counter-regulatory hormone release, e.g. cortisol, in the morning (Dawn phenomenon) can account for high morning blood glucose. B Jamie should skip breakfast – Incorrect. If blood glucose is high, a correction dose should be offered, taking into account the additional carbohydrates consumed. C Checking Jamie’s blood glucose overnight for a few nights will be helpful – Correct. Jamie may need to adjust his night time insulin dose. Morning hyperglycaemia is usually associated with glucose release from the liver overnight. Growth hormone and sex steroid hormones during puberty can cause morning hyperglycaemia as well (dawn phenomenon). Hyperglycaemia may also be the result of nocturnal hypoglycaemia, and subsequent secretion of excess counter-regulatory hormones. This is called the Somogyi phenomenon. D Jamie should cut out sugary food from his diet – Incorrect. In general, dietary guidelines are similar for children with diabetes as they are for the entire population. A healthy diet will consist of approximately 50% carbohydrates, 30-35% fat and 10-15% protein. A large proportion should consist of foods with a low glycaemic index: these foods release their glucose into the bloodstream at a slower rate than high glycaemic index foods. Carbohydrate counting (carb counting) is used to match the amount of insulin given at mealtimes to the amount of carbohydrate eaten.
Clinical cases: Intermediate
C Use of corticosteroid medication – Incorrect. Steroid ingestion increases blood glucose levels. D Consumption of alcoholic drinks – Correct. It is important for young people who drink alcohol to be aware of this effect, as it can result in unpredicted hypoglycaemia. E Febrile illnesses – Incorrect. Febrile illness is associated with hyperglycaemia, due to the actions of stress (counterregulatory) hormones.
Key Point Even in well controlled diabetes, it is still important to check blood glucose levels. This ensures objective information is available for any changes in insulin therapy. Carbohydrate counting is used to match insulin dose to carbohydrate consumption.
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IMPORTANT LEARNING POINTS
• Type 1 diabetes in children is an autoimmune condition that can be diagnosed with a random plasma blood glucose >11.1 mmol/L. • Management requires the child’s caregiver to have a good understanding of factors which affect their child’s blood glucose (e.g. exercise or intercurrent illness) and how to make appropriate insulin dosage adjustments. • Hypoglycaemic episodes can usually be managed with a sugary drink/snack, with no need to attend the hospital.
ANSWER TO CASE 3
A 14-year-old girl presenting with delayed puberty Q1 W hich of the following findings are consistent with the likely underlying diagnosis? The correct answers are A. Lanugo hair, C. Bradycardia, D. Obsessional thoughts and E. Social withdrawal. Although nothing has been said about food avoidance or fear of gaining weight, this child is likely to have anorexia nervosa. Anorexia nervosa has both psychological manifestations, and physical manifestations as a result of inadequate oral intake. The low BMI and amenorrhoea strongly suggest the diagnosis. She is also tired and hypothermic, common findings in anorexia nervosa. A Lanugo hair – Correct. Patients with anorexia nervosa are commonly found to have fine, downy lanugo hair on examination. B Hypertension – Incorrect. Patients with anorexia nervosa are often hypotensive, not hypertensive. C Bradycardia – Correct. Sinus bradycardia occurs in up to 95% of cases of anorexia nervosa. D Obsessional thoughts – Correct. Obsessional thoughts are common in patients with anorexia nervosa and are not limited to food. E Social withdrawal – Correct. Patients with anorexia nervosa may become withdrawn from their social circle or lose interest in activities they previously enjoyed.
Key Point Common findings in patients with anorexia nervosa are prepubertal sexual characteristics, lanugo hair, low BMI and signs of systemic failure, such as hypotension, hypothermia and bradycardia.
Q2 Which of the following initial investigations would be helpful in this patient? The correct answers are A. LH and FSH concentrations, B. Thyroid function tests and E. Chromosomal karyotyping. Although, clinically, it may appear that this is a clear case of anorexia nervosa, investigations are required to assess other potentially treatable causes. This includes hypothyroidism, Turner’s syndrome and hypogonadotrophic hypogonadism. A LH and FSH concentrations – Correct. This will help to identify if the hypothalamo-pituitary-gonadal axis is intact and exclude primary gonadal failure. If LH and FSH are low, it implies the problem is hypothalamic/pituitary in origin (as seen in anorexia nervosa), whereas if they are high/normal, the problem is more likely to be primary gonadal failure. B Thyroid function tests – Correct. Children with hypothyroidism may present with delayed puberty. They do not always present with typical weight gain. C MRI brain – Incorrect. This is not a key initial investigation, although is very important in patients where there is a suspicion of a pituitary tumour. D Adrenal MRI – Incorrect. An adrenal mass would present with precocious puberty, not delayed. E Chromosomal karyotyping – Correct. Any girl with primary amenorrhoea should have her karyotype checked to rule out Turner’s syndrome. Other rarer conditions, such as complete androgen insensitivity syndrome (i.e. a male genotype XY with a female phenotype because of androgen insensitivity) can present with primary amenorrhoea.
Key Point • All girls with delayed puberty should have a karyotype done to exclude Turner’s syndrome. • Baseline LH and FSH measurements are useful first-line investigations to differentiate primary from secondary gonadal failure. Q3 Which management option is best? The correct answer is E. Referral to child and adolescent mental health services The mainstay of treatment for anorexia nervosa is with child and adolescent mental health services. It is also important to treat anorexia nervosa from a medical perspective. There may be associated bradycardia or hypotension. Introducing feeding may be associated with refeeding syndrome, and so electrolytes need to be closely monitored. Severe cases will require admission to hospital for medical stabilisation and refeeding.
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ANSWERS
Key Point Patients with suspicion of anorexia nervosa should be referred to mental health services. The urgency of referral depends on the rate of weight loss and evidence of systemic failure. IMPORTANT LEARNING POINTS
• Delayed puberty may be the first presentation of anorexia nervosa. • Delayed puberty is more likely to have a pathological cause in girls than in boys. • All girls with delayed puberty need to undergo chromosomal analysis to exclude Turner’s syndrome, even if they have no other features of the disease. • Anorexia nervosa cases should promptly be referred to mental health services. If there is systemic failure, or very rapid weight loss, referral has a greater urgency, and inpatient admission may be warranted.
ANSWER TO CASE 4
A 12-day-old baby presenting with poor feeding and vomiting Q1 What is the most likely diagnosis? The correct answer is B. Congenital adrenal hyperplasia. The differential for poor feeding and vomiting in a neonate includes infection, sepsis, congenital heart disease and bronchiolitis. However, the presence of hyponatraemia and hyperkalaemia should always prompt clinicians to rule out CAH.
A Fulminant sepsis – Incorrect. Sepsis is an important differential diagnosis because of lethargy and irritability. However, given i) no temperature, ii) negative cultures and iii) normal inflammatory markers, it is less likely than CAH. It is important to note that the absence of fever and negative blood cultures do not rule out sepsis. Therefore, even though it is not the top differential, this baby needs to be empirically treated for sepsis. B Congenital adrenal hyperplasia – Correct. Hypoglycaemia, hyponatraemia, hyperkalaemia and dehydration in the neonatal period suggest CAH. Hyperpigmentation of scrotal skin is due to elevated ACTH concentration, which also fits with the diagnosis. This is the salt losing form of CAH. C Gastro-oesophageal reflux disease – Incorrect. GOR may present with vomiting, particularly after feeds. However, it would not cause the electrolyte derangement noted in this case. D Heart failure – Incorrect. Heart failure may present with poor feeding, but in this case there were normal antenatal scans, and a chest X-ray with no signs of heart failure. Additionally, the electrolyte derangement doesn’t fit with heart failure.
Clinical cases: Intermediate
A Watch and wait; follow up in 6 months – Incorrect. Anorexia nervosa needs to be actively managed from the point of diagnosis. B Start on antidepressants – Incorrect. Even if there was associated depression, talking therapies are first-line therapy, not medication. C Pubertal induction using oestrogen preparations – Incorrect. In this case, inducing puberty using oestrogens does not solve the underlying diagnosis of anorexia nervosa. The patient has delayed puberty due to hypothalamic dysfunction secondary to anorexia. D Start on GnRH agonist – Incorrect. This paradoxically suppresses gonadotrophin production, reducing FSH and LH levels. GnRH agonists are used in management of central precocious puberty. E Referral to child and adolescent mental health services – Correct. Urgency of the referral in patients depends on evidence of systemic failure and rate of weight loss.
Key Point The possibility of CAH should be considered in infants presenting with diarrhoea, vomiting and hypoglycaemia, especially when associated with hyponatraemia, hyperkalaemia, hypovolemia and metabolic acidosis. Q2 Which of the following tests is most useful in establishing the diagnosis in this clinical scenario? The correct answer is C. 17-hydroxyprogesterone. It is important to collect the correct blood samples before starting corticosteroid replacement. It is often difficult in the dehydrated, shocked neonate to collect blood. 17-hydroxyprogesterone is one of the precursors accumulating in 21-hydroxylase deficiency, the commonest form of the disease. Other useful tests are ACTH (high), renin (high) and cortisol (inappropriately low). A urine steroid profile is also diagnostic. In countries with newborn CAH screening, 17-hydroxyprogesterone is measured in a blood spot. A Karyotyping – Incorrect. Karyotyping may be required if there is doubt over gender, but it is not diagnostic. B Specific gene testing – Incorrect. Gene testing is not routinely done in CAH. However, it is useful if the parents are planning for future pregnancy. C 17-hydroxyprogesterone – Correct. 17OHP is a precursor in the adrenal biosynthetic pathway that is very high in 21-hydroxylase deficiency, the most common form of CAH.
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D Pelvic ultrasound – Incorrect. Pelvic ultrasound is useful in infants with disorders of sexual development in order to establish the presence or absence of the uterus. However, as bilateral descended testes were noted on examination, the scan is unnecessary.
Key Point A hormone profile is essential to diagnose CAH. An elevated 17-hydroxyprogesterone level is diagnostic of 21-hydroxylase deficiency. Q3 W hich of the following may be important aims in the treatment of this condition? The correct answers are A. Normalisation and maintenance of normal electrolytes, B. Glucocorticoid replacement, C. Mineralocorticoid replacement and D. Supporting normal growth and sexual maturation. CAH requires lifelong treatment to prevent hospital admissions and to maximise quality of life. Cortisol and aldosterone replacement is key. This also prevents the elevated ACTH (as a result of a “negative feedback” loop from low cortisol/aldosterone) that leads to accumulation of steroid precursors and subsequent androgen production. A Normalisation and maintenance of normal electrolytes – Correct. Electrolytes need to be monitored regularly, especially in the first year of life. Babies with the salt-wasting form of CAH usually need sodium oral supplementation in the first year of life. B Glucocorticoid replacement – Correct. Replacement with glucocorticoids is aimed to replace the lack of cortisol and to suppress the excess production of cortisol precursors. Oral corticosteroids have their limitations because cortisol is a hormone with a diurnal pattern that it is almost impossible to mimic with medication. Doses also need to be increased during an acute infection, surgery or trauma. Hydrocortisone needs to be given three to four times a day, which can make lifelong adherence to treatment challenging. C Mineralocorticoid replacement – Correct. Children with the salt-wasting form of CAH need mineralocorticoid replacement (fludrocortisone). Monitoring of BP, electrolytes and renin is required to ensure adequate treatment. Unlike with corticosteroids, these doses do not need to be increased during infections. D Supporting normal growth and sexual maturation – Correct. CAH can lead to precocious puberty. It can also lead to tall stature, and (from premature closure of epiphyseal plates) short stature.
Key Point CAH requires life-long adherence to treatment as there is no cure. Good control is signified by normal growth, normal sexual maturation and normal electrolytes. IMPORTANT LEARNING POINTS
• It is important to suspect CAH in all neonates presenting with hypoglycaemia, hyponatraemia and hyperkalaemia. • CAH is a life-threatening condition that requires lifelong replacement with corticosteroids +/mineralocorticoids. • CAH is most commonly due to 21-hydroxylase deficiency. Female infants classically present at birth with genital ambiguity, whilst boys usually present in the second week of life with adrenal crisis.
ANSWER TO CASE 5
A 5-year-old boy presenting with a headache Q1 In this case, which clinical features suggest the presence of raised intracranial pressure? Select all that apply. The correct answers are A. Early morning headaches, B. Unsteadiness and C. Persistent vomiting. Headaches in children are a common presenting symptom in primary care. The most common causes are benign headaches and migraines. The key to this question is to identify “red flag” symptoms and signs that suggest a more sinister pathology, including features that may indicate a brain tumour or raised ICP. Symptoms of raised ICP in children include headache, vomiting, new squint or visual disturbance, delayed walking, unsteadiness and drowsiness. Rapidly rising ICP can lead to hypertension, bradycardia and reduced conscious level. This is known as the “Cushing’s response” and is a neurological emergency. Measures should be immediately taken to reduce ICP (intravenous hypertonic sodium chloride or mannitol can lower ICP in an emergency, and corticosteroids reduce oedema surrounding the tumour). A Early morning headaches – Correct. Headache that is worse in the morning, present on waking or aggravated by sneezing, coughing or straining should raise concerns about raised ICP. However, remember that children with raised ICP do not always follow this pattern, as symptoms may occur at any time of the day or night. Very young children may not even complain of a headache.
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ANSWERS
Key Point Raised ICP should be considered in all children with persistent vomiting or headache without additional features to support an alternative diagnosis. In raised ICP, headache and vomiting is classically worse in the morning on waking. Q2 O n examination, there is evidence of ataxia and pastpointing. Bilateral papilloedema is present on fundoscopy. What is the most likely underlying diagnosis? The correct answer is D. Brain tumour. Early morning headache, vomiting and papilloedema are characteristic of raised ICP. This triad of red flags in a previously well child should suggest the possibility of a brain tumour. A Severe hypertension – Incorrect. Severe hypertension or “malignant” hypertension must be excluded as a cause of chronic/severe headache. However, in this case, the BP is normal. B Post-infectious acute cerebellar ataxia – Incorrect. It is important to exclude causes of acute cerebellar ataxia, but the absence of a preceding prodromal illness or varicella infection (a common cause of post-infectious cerebellar ataxia) make this diagnosis unlikely. C Cerebral abscess – Incorrect. This is unlikely in a previously well child without a fever, but a lack of fever does not exclude a cerebral abscess, so it still should be considered in the differential diagnosis. D Brain tumour – Correct. The presence of ataxia, pastpointing and gait disturbance suggest a tumour in the posterior fossa of the brain (affecting the cerebellum).
Tumours in this location commonly cause obstruction of the CSF flow, leading to hydrocephalus and raised ICP. The most common tumours affecting the posterior fossa are medulloblastomas and astrocytomas. E Viral gastroenteritis – Incorrect. There are no additional symptoms, such as fever or diarrhoea, to support this diagnosis.
Key Point Brain tumours, although rare, are an important differential diagnosis in children presenting with headaches with or without vomiting. Q3 What would be your next investigation? The correct answer is A. CT or MRI scan.
Clinical cases: Intermediate
B Unsteadiness – Correct. Delayed walking or unsteadiness are features of raised ICP because of effects on coordination and motor function. C Persistent vomiting – Correct. Raised ICP should be considered in children with persistent vomiting without additional supporting features to suggest gastroenteritis (e.g. diarrhoea, fever or an infected contact), even in the absence of a headache. D Intermittent headaches – Incorrect. Intermittent headaches are common in children and young people. The vast majority are benign. Headaches in children less than 4-years-old are rare and should be taken seriously. Headaches that are severe enough to wake a child from sleep, present on waking, are persistent (>4 weeks) or associated with confusion or other neurological signs should be urgently referred to a paediatric specialist. When assessing children with headaches in the absence of red flag features, it is essential to give safety net advice in case symptoms continue or get worse.
Brain imaging is essential to confirm the diagnosis of a space-occupying lesion and to look for the presence of hydrocephalus. A CT or MRI scan – Correct. This will depend on availability, as an MRI scan is more difficult to arrange in an emergency setting. Young children also require a general anaesthetic for an MRI scan as it requires them to lie still for a prolonged time. When there is strong suspicion of raised ICP, children will often undergo a CT scan first, as this can be done quickly. B Lumbar puncture – Incorrect. LP is contraindicated in children with reduced GCS, seizures, focal neurological signs and signs of raised ICP. In the presence of elevated ICP secondary to a brain tumour, an LP could cause cerebral herniation. Children diagnosed with certain brain tumours will require an LP at a later stage, once stabilised, in order to look for malignant cells that have spread to the CSF. C Blood culture – Incorrect. There are no clinical features to suggest an underlying infectious aetiology. D Varicella serology – Incorrect. There are no clinical features to suggest varicella infection, although this would be considered in a general screen for a patient with cerebellar symptoms when the diagnosis is less clear. E EEG – Incorrect. There is no evidence that this patient has had seizures, and subclinical seizures are unlikely to present with any of the other features mentioned in isolation.
Key Point The presence of raised ICP should warrant immediate paediatric referral for brain imaging.
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Q4 Th e CT scan confirms an intracranial lesion. How would you manage this child in ED? The correct answer is E. Referral to the nearest neurosurgical centre. This child has evidence of raised ICP which may be caused by a large tumour directly infiltrating the brain or by the tumour obstructing CSF flow. A Corticosteroids – Incorrect. Corticosteroids will reduce oedema surrounding the tumour, thereby reducing ICP. This should only be given after discussion with neurosurgeons, since, if surgery is being done imminently, corticosteroids may increase risk of bleeding. In the presence of raised ICP, mannitol or hypertonic sodium chloride may also be used. B Intravenous aciclovir – Incorrect. There is no indication for antiviral treatment, as the pathology is malignancy. C Discharge with parental advice to return if symptoms are worsening – Incorrect. There are clearly symptoms and signs of raised intracranial pressure that require prompt recognition and management. If treatment is delayed, it could lead to cerebral herniation (coning), coma or death. D Lumbar puncture to relieve the symptoms of raised ICP – Incorrect. Lumbar puncture is contraindicated in this instance, as a sudden reduction in pressure may precipitate cerebral herniation. E Referral to the nearest paediatric neurosurgical centre – Correct. Urgent referral to the on-call paediatric neurosurgeon is required for management of raised ICP. If supportive measures and corticosteroids alone are not able to control ICP, emergency neurosurgical intervention may be required.
Key Point The most important initial management of a patient with a brain tumour is to safely control raised ICP. Emergency management of airway, breathing and circulation is required for children with reduced GCS, seizures or “Cushing’s response”, together with transfer to a specialist paediatric neurosurgical centre. IMPORTANT LEARNING POINTS
• Early symptoms of brain tumours in children can mimic those of common benign paediatric conditions. • Symptoms and signs include headache, vomiting, visual symptoms and signs (including papilloedema, nystagmus and squint), motor symptoms and signs (including ataxia, weakness, swallowing difficulties and speech disturbance), behavioural change/drowsiness and seizures.
ANSWER TO CASE 6
A 13-month-old girl presents with severe pallor Q1 What is the most likely underlying diagnosis? The correct answer is B. Iron deficiency anaemia. The key features here are the dietary history and the fact the child is found to be well, with no worrying features on examination. A Acute lymphoblastic leukaemia – Incorrect. ALL is an important differential diagnosis for anaemic symptoms in children. Children with leukaemia will often present more unwell with additional features including pallor, tiredness, pain and lymphadenopathy. It is unusual, but possible, for children with ALL to present with an isolated anaemia. It is therefore essential for children presenting with abnormal FBC parameters to obtain an urgent blood film. B Iron deficiency anaemia – Correct. Iron deficiency anaemia is the commonest cause of anaemia in an otherwise well child. There is often a history of excessive cow’s milk intake in the older infant or inadequate dietary variety. Proteins in cow’s milk disrupt iron absorption and whole milk proteins can cause an enteropathy, thus causing chronic blood loss. C Aplastic anaemia – Incorrect. This is an important differential but very rare. It is usually associated with other abnormalities in the blood parameters. D Coeliac disease – Incorrect. Coeliac disease may result in a microcytic anaemia but is also associated with weight loss, abdominal distension, irregular bowel habits and diarrhoea. E B12 deficiency – Incorrect. B12 deficiency is associated with a macrocytic anaemia, not a microcytic anaemia.
Key Point Iron deficiency anaemia is the commonest cause of anaemia in children. The anaemia is not usually severe, but can be mild or moderate. Q2 What would be your next investigations? Select all that apply. The correct answers are B. Iron studies and C. Blood film. A Bone marrow aspirate – Incorrect. A bone marrow aspiration would not be indicated in this child at this stage. If the anaemia fails to respond to a course of adequate iron therapy or there are other abnormalities in the blood count, then a bone marrow aspirate may be performed at a later stage. B Iron studies – Correct. A low serum ferritin, low iron and elevated total iron binding capacity (TIBC) will help to confirm iron deficiency anaemia. C Blood film – Correct. A blood film examination is essential to look at cellular morphology. The most important
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Key Point Iron deficiency anaemia is confirmed by verifying a low serum ferritin, low iron, and an elevated TIBC. However, ferritin may be in the normal range if there is concurrent illness, as ferritin is elevated in acute inflammation. Q3 W hat would be your first-line management for the treatment of the above condition? The correct answer is C. Discharge, commence oral iron supplementation and observe. Whilst local guidelines and policies may vary, the general principles are that a period of observation in hospital, along with adequate documentation of successful toleration of oral iron therapy, are important in children with this severe an anaemia. A Observation as an outpatient and advise – Incorrect. Such a low Hb level necessitates treatment. B Admit for blood transfusion – Incorrect. Normally, it is possible to avoid blood transfusion in the case of iron deficiency, regardless of how low the haemoglobin is. This child has anaemia but no heart failure and she is well compensated. If done, transfusion should be done cautiously. C Discharge, commence oral iron supplementation and observe – Correct. The degree of anaemia necessitates oral iron supplementation, and improvement of diet. D Admit for IV iron supplementation – Incorrect. IV iron is rarely used, as it has caused deaths by anaphylaxis. It would be reserved for cases where oral supplementation was not feasible or achieved. E Referral to gastroenterology for urgent endoscopy – Incorrect. This child has a good consistent history for dietary iron deficiency, so endoscopy is not indicated.
Q4 What feature is not typical of this condition and should prompt rapid referral to a specialist team? The correct answer is A. Black tarry stools. The presence of significant GI bleeding would be a very different entity and requires prompt endoscopic evaluation and gastroenterologist review. A Black tarry stools – Correct. Black tarry stools suggest blood loss and could be suggestive of a Meckel’s diverticulum. This should prompt urgent assessment by gastroenterologists. Iron supplements may cause black stools, but not tarry stools. B Poor diet – Incorrect. Iron deficiency occurs in the context of an inadequately balanced diet. If the family report a wellbalanced diet, other causes should be looked for. C Normal serum ferritin – Incorrect. Serum ferritin is an acute phase reactant so it may be falsely elevated with intercurrent viral infections. A normal serum ferritin does not exclude iron deficiency, which is why measuring iron and TIBC is helpful. D Severe pallor – Incorrect. The degree of pallor will be linked to the degree of the anaemia, not the underlying cause. E Exclusively breastfed – Incorrect. After six months of age, exclusive breastfeeding can lead to iron deficiency. Cow’s milk and human milk both have low iron contents but the bioavailability of iron from human milk is higher. Infants who are fed on formula milks are less likely to develop severe iron deficiency anaemia, as formula milk is supplemented with iron.
Clinical cases: Intermediate
diagnosis to rule out is ALL, where a blood film may reveal lymphoblasts. D Parvovirus screen – Incorrect. Parvovirus can cause aplastic anaemia, but it is not the most likely cause, particularly given that platelets and WBC counts are normal. It will also usually be associated with a low/normal reticulocyte count, as it affects the bone marrow. E Endoscopy – Incorrect. Whilst an endoscopy may be required in adults presenting with severe iron deficiency anaemia, in children with a compatible history, dietary iron deficiency is most likely.
Key Point Black tarry stools are suggestive of blood loss and further investigation needs to be carried out to rule out GI bleeding, e.g. from a Meckel’s diverticulum. IMPORTANT LEARNING POINTS
• Iron deficiency anaemia is the most common cause of anaemia in childhood. The majority of cases are dietary in origin. It can result in severe anaemia. • Exclusive breastfeeding after six-months-old or excessive cow’s milk consumption in infants are risk factors for development of iron deficiency. • Admission and supportive care during the initial stages of managing severe anaemia is important, even if supplementation is oral.
Key Point Children with severe iron deficiency anaemia do not necessarily need a blood transfusion, even if the haemoglobin is very low. In general, they should be started on oral iron supplementation and observed closely to ensure this is tolerated.
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ANSWER TO CASE 7
A 4-year-old presenting with thrombocytopenia Q1 What is the most likely underlying diagnosis? The correct answer is B. Immune thrombocytopenic purpura. Petechiae and bruising have a broad differential, with many sinister causes such as meningococcal sepsis, ALL and non-accidental injury. A Acute lymphoblastic leukaemia – Incorrect. Children with leukaemia will often present as more unwell, with additional features including pallor, tiredness, pain and lymphadenopathy. It is unusual, but possible, for children with ALL to present with an isolated thrombocytopenia. It is therefore essential to obtain a blood film for any children presenting with thrombocytopenia. B Immune thrombocytopenic purpura – Correct. ITP is characterised by rapid-onset bruising and petechiae in an otherwise well child. There is often a history of a recent viral infection or immunisations. C Henoch-Schönlein purpura – Incorrect. HSP is a clinical diagnosis based on the identification of certain clinical features including an evolving palpable purpuric rash, joint pain and periarticular oedema. There may also be abdominal symptoms and renal involvement. It is a vasculitic disorder and may be precipitated by an URTI. Thrombocytopenia is not characteristic; conversely the platelet count may be elevated. D Systemic lupus erythematosus – Incorrect. In older children and teenagers, thrombocytopenia is more often associated with autoimmune diseases and rheumatological conditions, such as SLE. Additional symptoms and signs include a characteristic facial “butterfly” rash, fatigue and arthralgia. E Meningococcal sepsis – Incorrect. Early meningococcal infection can be difficult to recognise. Children presenting acutely unwell with a fever and non-blanching rash should be treated empirically with broad-spectrum intravenous antibiotics.
Key Point ITP is the most common cause of thrombocytopenia in children. Diagnosis of ITP is made by exclusion of other more serious conditions, particularly ALL. Q2 What would be your next investigation? The correct answer is B. Blood film. A blood film is key to ensuring leukaemia is not missed.
A Bone marrow aspirate – Incorrect. Bone marrow aspirate is not indicated in children diagnosed with classic ITP. It is an invasive test, often requiring general anaesthetic use. However, it may be considered after discussion with a paediatric haematologist when atypical features are present or if thrombocytopenia is unusually prolonged. B Blood film – Correct. Blood film examination is essential to look at cellular morphology. The most important diagnosis not to miss is ALL where a blood film may reveal lymphoblasts. C No further investigation – Incorrect. ALL needs to be ruled out. D Autoantibody screen – Incorrect. Further investigations are only indicated in older children, or in patients with chronic ITP when autoimmune disorders and other rare conditions may be the cause of the thrombocytopenia.
Key Point If atypical features of ITP are present, it is recommended that the patient is referred to a paediatric haematologist for a bone marrow aspirate. Q3 What would be your first-line management for the treatment of the above condition? The correct answer is A. Observation and advice. The natural disease course for ITP is spontaneous resolution; therefore, in most cases, no specific treatment is needed. A Observation and advice – Correct. The majority of children with ITP undergo spontaneous resolution within 6-8 weeks. Initial management should not depend on platelet count alone and is influenced by the presence of complications including significant haemorrhage. The parents should be advised to attend the ED if the child sustains a head injury. B Intravenous immunoglobulin – Incorrect. Only in the case of severe symptoms and significant haemorrhage is intravenous immunoglobulin considered. This treatment has not been shown to influence the overall disease duration. C Corticosteroids – Incorrect. Corticosteroids are only indicated in specific cases, e.g. significant haemorrhage. This treatment has not been shown to influence the overall disease outcome. Diagnosis of ITP must be certain before giving corticosteroids as this can have major adverse consequences should the patient turn out to have underlying leukaemia. Many specialists recommend BMA prior to starting corticosteroid treatment for this reason. D Splenectomy – Incorrect. Splenectomy is very rarely required and only considered in cases of chronic ITP (>6 months duration) with impaired quality of life. E Platelet transfusion – Incorrect. Due to the disease aetiology, transfused platelets will be quickly consumed. Therefore, transfusion should be reserved for patients with lifethreatening haemorrhage.
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Initial management of ITP consists of observation, reassurance, parental education and support. The vast majority of cases resolve spontaneously, and significant haemorrhage is rare. Q4 What features are not typical of this condition? The correct answer is A. White cell blasts on blood film. Atypical features may necessitate alternative treatment, or an alternative diagnosis. ALL is important to always rule out with a blood film. A White cell blasts on blood film – Correct. The presence of white cell blasts on blood film is not a feature of ITP and is suggestive of leukaemia. This should prompt immediate referral to a paediatric haematologist for further management. B Spontaneous resolution in 6-8 weeks – Incorrect. The natural course of uncomplicated ITP is for the disease to spontaneously resolve within 6-8 weeks. C History of a preceding febrile illness – Incorrect. ITP is often associated with a recent URTI. D Platelet count 10 x 109/L – Incorrect. Platelet count can fall to extremely low levels with ITP. It is common for the platelet count to be 38.5°C, inability to weight-bear, white cell count >12 x 109/L), which gives around a 90% chance of septic arthritis. The preceding trauma may be a red herring or may have caused a sterile haemarthrosis that has subsequently become infected. D Transient synovitis – Incorrect. Differentiating septic arthritis from transient synovitis can be difficult. In the above case, there is no preceding illness and enough positive modified Kocher criteria are present to warrant concern regarding septic arthritis. E Osgood-Schlatter disease – Incorrect. Osgood-Schlatter disease is caused by inflammation of the patella ligament at its insertion onto the tibial tuberosity at the knee. There is often a painful, palpable bump over the tibial tubercle. It predominantly affects sporty, male children between the ages of 9 to 16.
Key Point Septic arthritis should be excluded in a child with preceding trauma as a haemarthrosis provides a rich medium in which organisms can grow. Q2 W hat additional investigations might be helpful in establishing a diagnosis? The correct answers are A. X-ray right knee, B. CRP, C. ESR and D. Ultrasound right knee. Radiographs of the affected joint and baseline bloods including CRP are the first line investigations of choice in any acutely swollen joint.
A X-ray right knee – Correct. An AP and lateral X-ray of the affected joint is sensible to ensure no underlying fractures are missed. The lateral view may also demonstrate a lipohaemarthrosis. B CRP – Correct. CRP is a useful marker of underlying inflammation. C ESR – Correct. ESR is also an important inflammatory marker, and should be performed in addition to CRP. D Ultrasound right knee – Correct. If there is diagnostic doubt, the ultrasound can confirm the presence of an effusion. Any synovial reaction identified is consistent with infection. Ultrasound can also be used to guide aspiration if needed. This would not be appropriate for pus in the knee as this requires a washout but may be useful in other rheumatological conditions. Ultrasound may also be useful to identify features of autoimmune disease associated with an effusion, such as synovial thickening although this depends on the skill of the ultrasonographer. An ultrasound should not delay knee washout where there is a high suspicion of septic arthritis. E X-ray right hip – Incorrect. Given the history and clear knee pathology, an X-ray of the hip is unnecessary providing the child has no evidence of hip pain upon examination.
Clinical Cases: Difficult
ANSWERS
Key Point Septic arthritis classically results in elevated inflammatory markers (CRP, ESR, white cell counts), pyrexia and an inability to weight-bear. The more factors present, the more likely the diagnosis. Q3 What is the next best stage in management of this patient? The correct answer is D. Refer to orthopaedic team for knee aspiration/washout and then commence broad-spectrum antibiotics. Aspiration of the joint under sterile conditions is the definitive way to diagnose an infected joint. Antibiotics started prior to aspiration can eradicate organisms prior to culture making definitive diagnosis and targeting of antibiotic therapy difficult. A Immediately commence broad-spectrum antibiotics – Incorrect. Although sepsis campaigns advocate the immediate commencement of intravenous antibiotics, in the case of joint infections early administration before aspiration can prevent accurate identification of a causative organism. B Admit and observe the patient – Incorrect. The patient has a high probability of having a septic joint according to Kocher’s modified criteria and, without treatment, is likely to deteriorate. C Immediately commence broad-spectrum antibiotics and then aspirate the knee – Incorrect. Early antibiotic therapy can prevent accurate diagnosis of the causative organism.
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Clinical Cases: Difficult
D Refer to the orthopaedic team for knee aspiration/ washout and then commence broad-spectrum antibiotics – Correct. Given the patient’s age, an anaesthetic will be necessary. If there is frank pus aspirated under anaesthetic, a formal washout of the joint is needed. If there is no pus, as much fluid as possible should be aspirated and sent for urgent MC&S. Following aspiration, broad-spectrum antibiotics should be immediately commenced.
Key Point Aspiration of a joint should be performed before commencing antibiotics as antibiotic therapy can eradicate organisms prior to culture. Q4 G iven the patient’s age, what are the most likely causes of septic arthritis? The correct answers are A. Staphylococcus aureus and E. Haemophilus influenzae. Different organisms are more likely to affect each specific age group due to different exposures. Confirmation is via aspiration of joint fluid under sterile conditions and culture. A Staphylococcus aureus – Correct. This is common in all age groups and is the most common organism to infect joints. B Neisseria gonorrhoea – Incorrect. This primarily occurs in sexually active teenagers. C Group B streptococci – Incorrect. This primarily occurs in neonates and children under one. D Gram negative bacilli – Incorrect. This primarily occurs in neonates and children under one. E Haemophilus influenzae – Correct. This is seen in 1 to 5-year-olds.
Key Point Staphylococcus aureus is the most common causative organism in joint infections. IMPORTANT LEARNING POINTS
• Any child with a temperature and inability to weight-bear or with a swollen joint should be assessed for septic arthritis. • A history of trauma does not exclude a subsequent septic arthritis. • The child should undergo aspiration and joint washout prior to commencing antibiotics to allow identification of the infecting organism.
ANSWER TO CASE 2
An 11-year-old boy presenting with a wrist injury Q1 What two pieces of information from the history are most important for the above scenario from the list below? The correct answers are A. Hand dominance and B. Mechanism of injury. A Hand dominance – Correct. Knowing the hand dominance of the child is important for practical reasons. The child and parent will need advice on casting and/or resting the injured side. This may require alterations to their school work, such as no sports or stopping writing for a short period. B Mechanism of injury – Correct. In any child with a fracture, knowing the underlying mechanism of injury is vital for understanding both how to treat the fracture and to ensure there are no child protection issues. C Family history – Incorrect. This is unrelated to fracture management, apart from in rare cases of pathological fractures where the underlying cause may have a genetic aetiology. D Time of last meal – Incorrect. This is important in the context of urgent surgery, but doesn’t apply in the above case. E Weight – Incorrect. The weight of the child has no bearing on management.
Key Point Understanding the link between mechanism of injury and likely pathology is vital to determining whether there are any child protection concerns. Q2 How would you assess the function of the anteriorinterosseous nerve branch of the median nerve? The correct answer is C. Ask the patient to make an “ok” sign with their index finger and thumb. Accurate examination and documentation of neurovascular status following a fracture are vital so that no nerve or arterial injuries are missed. The same should be documented following any reduction of the fracture. A Ask the child to spread their fingers, whilst the examiner tries to push them together – Incorrect. This tests the motor function of the ulnar nerve. B Ask the patient to extend their wrist – Incorrect. This tests motor function of the radial nerve. C Ask the patient to make an “ok” sign with their index finger and thumb – Correct. The anterior interosseous branch of the median nerve innervates flexor pollicis longus, pronator quadratus and the radial half of flexor digitorum profundus.
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ANSWERS
Key Point A full neurovascular examination should be conducted distal to any fracture. Nerves are rarely fully transected (neurotmesis) but instead are often compressed (neuropraxia) leading to a transient loss of function or paraesthesia which usually recovers in weeks to months. Q3 How would you manage this child? The correct answer is D. Below elbow Plaster of Paris for 2-3 weeks. The anatomy and biomechanics of children’s bones are unique, resulting in different fracture patterns, different healing mechanisms and different management compared to adults. Children’s fractures have significantly lower rates of non-union. Adult fractures take four to six weeks to heal, whereas children’s take approximately half this time. A Closed reduction and percutaneous pinning – Incorrect. The injury is closed, extra-articular and is minimally displaced. In an 11-year-old child, this should heal quickly and is likely a stable injury. Operative intervention should be reserved for a) extra-articular injuries that are i) angulated or ii) associated with a deformity, and b) intra-articular injuries that are displaced. B Open reduction and internal fixation – Incorrect. Operative intervention is not indicated. C Futura splint for eight weeks – Incorrect. If the child is comfortable and the greenstick fracture is stable, a Futura splint for 2-3 weeks is an option, as it will immobilise the joint. However eight weeks is too long. D Below elbow Plaster of Paris for 2-3 weeks – Correct. This is the appropriate management of a stable greenstick fracture, as the child is uncomfortable due to swelling and soft tissue injury. The fracture will heal over a 2-3 week period and a fiberglass below elbow cast allows the wrist to be protected if the child is likely to be active. Repeat X-ray at this time is unnecessary in stable undisplaced greenstick fractures and the child may return to have the plaster removed at the hospital or the parents may be given instructions to remove it themselves if they wish. E Above elbow Plaster of Paris for 2-3 weeks – Incorrect. While the time period is correct, immobilising the elbow joint is unnecessary in a stable greenstick fracture and can lead to problems with stiffness upon removal of the cast.
Key Point Undisplaced extra-articular fractures do not require operative intervention unless there is visible deformity or angulation, although this should be judged on a case-bycase basis. IMPORTANT LEARNING POINTS
• In any paediatric fracture, determining the mechanism of injury and understanding patterns of injury is vital to exclude any child protection issues. • Neurovascular status should be carefully documented both pre and post any reduction. In the child with an upper limb injury, neurological status is best done by demonstrating movements, and then asking the child to copy them.
Clinical Cases: Difficult
D Test sensation over the anatomical snuff box area – Incorrect. This tests the sensory modality of the radial nerve. E Test sensation over the regimental badge area – Incorrect. This tests the sensory modality of the axillary nerve.
• Paediatric fractures heal quickly. Casts should be used to protect the fractured area from further trauma and to hold any unstable injury in a reduced position. Casts are associated with complications, such as joint stiffness; therefore, they need to be removed in a timely manner.
ANSWER TO CASE 3
An 8-year-old boy presenting with groin and knee pain Q1 What is the most likely differential diagnosis? The correct answer is C. Perthes’ disease. Perthes’ disease is due to lack of blood flow to the growing femoral head and subsequent avascular necrosis. Hip, knee, groin pain and limp are common presentations in the 5 to 12-years-old age range. A Developmental dysplasia of the hip – Incorrect. Congenital developmental dysplasia of the hip may be picked up before the child begins to walk by an observant parent. In later childhood, developmental dysplasia of the hip presents insidiously with an abnormal gait and excessive shoulder sway with minimal if any hip pain. Trendelenburg’s sign is positive. B Slipped upper femoral epiphysis – Incorrect. SUFE is the posterior-inferior displacement of the upper femoral epiphysis through the epiphyseal plate. It usually occurs in tall, overweight boys during the adolescent growth spurt. C Perthes’ disease – Correct. This child is displaying the signs and symptoms of Perthes’ disease. Classically, it presents with insidious onset hip pain radiating down the thigh, associated with a limp. All hip movements are reduced and painful, especially internal rotation and abduction. Leg length discrepancy and muscle wasting may occur in severe disease.
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D Transient synovitis – Incorrect. This presents more acutely, and is associated with minor trauma or viral infection. Pain is usually absent at rest and the limb is held in the position of greatest ease; usually flexed, abducted and slightly externally rotated. There will be no leg length discrepancy. E Septic arthritis – Incorrect. Septic arthritis is a purulent infection of the joint space. It presents acutely, usually with fever, an exquisitely painful joint and an inability to weight bear.
Key Point Thorough history and examination, taking into account the child’s age is paramount when a child presents with a limp. A diagnosis of transient synovitis should only be made after careful consideration of more sinister pathology. Q2 Which two of the following are risk factors for this condition? The correct answers are C. Low birth weight and E. short stature. The exact cause of Perthes’ disease is still unknown. A number of risk factors have been identified, such as low birth weight, short stature, family history, delayed skeletal age, and low socioeconomic status. A Breech presentation – Incorrect. Breech presentation predisposes to developmental dysplasia of the hip, not Perthes’ disease. Other risk factors for developmental dysplasia of the hip include family history, female sex, maternal oligohydramnios and first born child. B Obesity – Incorrect. Associated with an increased risk of SUFE. C Low birth weight – Correct. Risk factors for Perthes’ disease also include a family history, low socioeconomic class and delayed skeletal age. D Female – Incorrect. Perthes’ is four times more common in boys. E Short stature – Correct. Conversely, SUFE is more common in tall boys.
Key Point Childhood pathologies presenting with a limp are common and often share similar history and examination findings. Therefore, an awareness of the risk factors for these conditions can go a long way in narrowing down the differential diagnosis and preventing unnecessary investigations. Q3 W hich of the following are the two most appropriate initial investigations? The correct answers are A. AP and lateral X-ray of the hip, and D. FBC, CRP, ESR.
Plain hip radiographs and baseline blood tests are standard initial investigations in the child presenting with new joint pain. Blood tests are primarily to exclude a septic cause or malignancy and radiographs may demonstrate features of Perthes’ disease. A AP and lateral X-ray of the hip – Correct. Plain radiographs are the most appropriate initial imaging study and the mainstay in the diagnosis of Perthes’ disease. A limping child is a common presenting complaint with several differential diagnoses. Many of the causative conditions have characteristic X-ray appearances suggestive of a particular diagnosis. Radiological features of Perthes’ disease include collapse of the femoral head, sclerosis and widening of the joint space. Later, the femoral head becomes fragmented and irregular. B CT scan – Incorrect. Not typically used in the diagnosis of Perthes’ disease. C MRI scan – Incorrect. This would not be an initial investigation. However, MRI does have a role to play in the diagnosis of Perthes’ disease if the history, physical examination and other imaging studies do not identify the source of the pathology. MRI is very sensitive at detecting early disease devoid of plain radiographic features. D FBC, CRP, ESR – Correct. Elevated white cell count, CRP or ESR may suggest septic arthritis or osteomyelitis. If these tests are within their reference ranges, it strengthens the suspicion of Perthes’ disease. E Ultrasound – Incorrect. This investigation is of limited value in the diagnosis of Perthes’ disease, but it may identify joint effusion, which would help in the broader evaluation of a limping child.
Key Point For any child with an atraumatic limp, have a low threshold for performing a hip X-ray and check baseline inflammatory markers. Septic arthritis is the most worrying differential, and although there is no perfect diagnostic test, normal inflammatory markers in an apyrexial patient makes it much less likely. Q4 How should this child be treated? The correct answer is A. Surgical containment of the femoral head. The main aim of treatment is to keep the femoral head contained in the acetabulum, and maintain good hip movement. This is compromised in Perthes’ disease by the changes in shape of the femoral head. In younger children, containment can usually be achieved conservatively, with observation, activity restriction, partial weight bearing, traction and physical therapy. In older children, conservative management is more likely to fail, and femoral and/or pelvic osteotomies (removing part of the bone) may be necessary.
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ANSWERS
Mid-parental height calculation is based on the calculation of the mean parental height correcting for sex as the mean sex difference is around 13 cm. Therefore the following formulae are suggested: ӹӹ Boys’ sex-corrected height = (Father’s height + mother’s height + 13) / 2 ӹӹ Girls’ sex-corrected height = (Father’s height + mother’s height - 13) / 2 Charts like that given simplify this process (Figure 2b). Note that this chart is for boys, and there is a separate chart for girls. Parent Height Comparator Father’s height cm ft/in 6′7″ 200 6′6″ 195
190
185
180
175
170
A 3-year-old boy presenting with short stature Q1 Using the centile chart, please calculate the centile of the midparental height. The correct answer is B. 50th centile.
6′3″
5′9″ 75th
6′1″
5′7″
50th
Centile chart.
155
165
160
5′2″ 25th
5′5″
5′1″
5′5″
5′3″
5′7″
5′2″
170
5′4″
5′1″
155
5′
5′4″
FIGURE 2B
175
5′6″
5′9″
5′3″
180
5′8″
5′11″
5′6″
160
5′11″ 5′10″
5′8″
IMPORTANT LEARNING POINTS
ANSWER TO CASE 4
91st
6′4″
5′10″
165
• Where diagnosis remains uncertain, MRI scans can be used, and early orthopaedic input should be sought.
6′5″
6′
A simple aide-memoire to remember the treatment of Perthes’ disease is “half a dozen, half a head”. So, if the patient is more than 6-years-old or has more than half the femoral head involved, surgical containment treatment should be considered.
• Remember that a child presenting with features suggestive of transient synovitis may in fact be an early presentation of Perthes’ disease. Viral illnesses, such as upper respiratory tract infections, are very common in childhood: this may be a “red herring” in the history.
Mid-parental 6′ centile
6′2″
Key Point
• Any patient 4 to 8-years-old presenting with atraumatic hip or knee pain should be investigated for Perthes’ disease.
Mother’s height ft/in cm 185 6′1″
Clinical Cases: Difficult
A Surgical containment of the femoral head – Correct. Mild to moderate degree Perthes’ disease is said to be present if there is less than 50% involvement of the femoral head with preservation of joint space. In younger children management is likely to be conservative with bed rest and traction. Severe disease occurs when there is more than 50% involvement of the femoral head and usually mandates surgical treatment. B Intravenous antibiotics – Incorrect. Antibiotics are not used in the management of Perthes’ disease. Instead, they are indicated in infective pathologies, such as septic arthritis and osteomyelitis. C Bed rest and traction – Incorrect. Indicated in mild to moderate Perthes’ disease in a child younger than 6-years-old. D Fixation of the epiphysis with threaded pins/screws – Incorrect. This is the appropriate management of slipped upper femoral epiphysis with minor displacement of the femoral head. E Conservative management with observation only – Incorrect. Conservative management may be appropriate for mild Perthes’ disease. However, such conservative treatment includes bed rest, traction, analgesia and regular physiotherapy to increase muscle strength, not observation alone.
4′11″ 9th
150
4′10″ 4′9″
145
Key Point Mid-parental height should always be corrected for the gender of the child. Q2 Which of the following suggest a pathological cause for short stature? The correct answers are A. Discrepancy between height and weight, B. Discrepancy in height centile and mid-parental centile, and C. Poor height velocity. Accurate measurements and record keeping are paramount in the assessment of growth in children. Healthcare providers should always refer children to paediatricians when there is discrepancy
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Clinical Cases: Difficult
between height and weight centiles, when height falls two centiles below or above the mid-parental height or when there is evidence of poor growth rate. A Discrepancy between height and weight – Correct. When there is a discrepancy between height and weight, one needs to rule out endocrine causes of short stature, such as GH deficiency, thyroid disorders and Cushing’s syndrome. Children with GH deficiency, Cushing’s syndrome or hypothyroidism are usually short and fat. Children with no endocrine cause of obesity are tall and obese. B Discrepancy in height centile and mid-parental centile – Correct. The greater the distance from the mid-parental height, the higher the possibility that height is abnormal. In cases where one parent is short, one needs to consider conditions inherited by autosomal dominant manner (e.g. skeletal dysplasia) that may have been undetected in the parent. C Poor height velocity – Correct. Height velocity is calculated by the increase in height over a period of time (at least 6 months, best over one year). The denominator consists of the difference in decimal age.
Key Point Endocrine problems often present with an obese child who is short for his family. Q3 W hich of the following is/are likely to yield useful information as to the cause of short stature? The correct answers are A. Birth weight, B. Neonatal hypoglycaemia, C. Neonatal jaundice and D. Floppy episodes during illness. Past medical history in the majority of children with short stature is unremarkable, but sometimes there are clues present, as described below. A Birth weight – Correct. Children born SGA who fail to catch up can be offered GH treatment. Additionally, being born SGA might be part of a syndrome. On the other hand, in the context of tall stature, a large birth weight may be associated with an overgrowth syndrome, such as BeckwithWiedemann. B Neonatal hypoglycaemia – Correct. GH deficiency can present in neonatal life with hypoglycaemia. C Neonatal jaundice – Correct. Pituitary hormone deficiencies can be related to conjugated hyperbilirubinaemia (ACTH, GH) and unconjugated hyperbilirubinaemia (TSH). D Floppy episodes during illness – Correct. Floppy episodes during illness might suggest episodes of hypoglycaemia.
Key Point A detailed neonatal history may provide important clues for the identification of the aetiology of short stature. Q4 Which investigation(s) should be requested for this patient? The correct answers are A. Thyroid function tests, B. Insulinlike growth factor 1, and C. Coeliac screen. Baseline tests are helpful to rule out conditions such as growth hormone deficiency, hypothyroidism, coeliac disease and Turner’s in girls. Growth hormone is particularly of concern in this case, given that: i The child is short with a disproportionately high weight. ii There is a history of neonatal hypoglycaemia. iii Loss of height is occurring in the second to third year of life (a growth hormone driven phase). Other tests that are likely to be requested are a FBC, ESR, CRP, LFTs, bone profile, urea and electrolytes, and Vitamin D levels. This may identify chronic illness. If a pituitary lesion is suspected, then wider pituitary function tests should be checked, along with an MRI of the pituitary. A bone age X-ray is also helpful to see whether this is delayed or normal. In females, chromosome testing is required to rule out Turner’s syndrome. A Thyroid function tests – Correct. Hypothyroidism can present with short stature in children especially when longstanding. B Insulin-like growth factor 1 – Correct. GH acts on its receptor on the liver to produce IGF-1. IGF-1 is a surrogate marker for GH action. It acts on the growth plates to promote linear growth. C Coeliac screen – Correct. Coeliac disease may manifest with poor growth, thus tissue transglutaminase and anti-endomysial antibodies should be checked. Children with coeliac disease are usually underweight but this is not an absolute rule. Gastrointestinal manifestations may not always be present or reported therefore most clinicians include coeliac screening in the first line investigations for short stature. D IGFBP-3 – Incorrect. GH circulates in a ternary complex with IGF1, IGFBP-3 and ALS (acid labile subunit). IGF1 is related to nutritional status, therefore in underweight children, IGFBP-3 might be helpful. However, it is not routinely checked.
Key Point Thyroid function tests, IGF-1, coeliac screen, FBC, ESR, CRP, LFTs, bone profile, urea and electrolytes, and vitamin D levels are useful first-line investigations in the assessment of short stature. In girls, a karyotype should always be included to rule out Turner’s syndrome.
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ANSWERS
• Accurate height and weight measurements should be obtained over multiple time points in all children with suspected weight or height abnormalities. The trend in both of these should be looked at from birth and compared to the mid-parental height. • There is more likely to be a pathological cause for short stature if i) growth velocity is poor, ii) height is significantly lower than mid-parental height centile, iii) if there is a discrepancy between height and weight centiles, and iv) if there are clues suggesting chronic illness in the history. • GH deficiency might present with transient hypoglycaemia in the neonatal period or infancy and then short stature later on (the second or third year of life).
ANSWER TO CASE 5
after a urine dipstick test so a negative history is not always helpful. E Watery diarrhoea and sweating – Correct. The presence of loose, watery stool may be caused by a neuroblastoma that secretes vasoactive intestinal peptide.
Key Point Abdominal malignancies are often associated with specific features: ◆◆ Haematuria – Wilms’ tumour ◆◆ Hypertension – Wilms’ tumour and neuroblastoma ◆◆ Urinary obstruction – rhabdomyosarcoma affecting the renal tract/bladder ◆◆ Watery stools and sweating – neuroblastoma Q2 Which two of the following diagnoses are most likely?
An 18-month-old girl presenting with an abdominal mass
The correct answers are B. Neuroblastoma and D. Wilms’ tumour.
Q1 Which of the following might suggest that the abdominal mass may be a tumour?
The presence of a well-defined mass and hypertension is strongly suggestive of cancer, most likely Wilms’ tumour or neuroblastoma.
The correct answers are B. Rapid change in size of abdominal mass, C. Presence of systemic symptoms such as fever, tiredness and weight loss, D. Presence of blood in the urine, and E. Watery diarrhoea and sweating.
A Constipation – Incorrect. Constipation is common in children and can cause abdominal distension, pain and rarely a left-sided mass (which is sometimes “indentable”). However, there is no history of infrequent stools in this child. B Neuroblastoma – Correct. Neuroblastoma commonly arises in the adrenal glands, abdomen or thorax. It can present with an isolated abdominal mass, or can be associated with pain, sweating, pallor, diarrhoea and hypertension. C Intussusception – Incorrect. Intussusception is caused by the “telescoping” of one segment of small bowel into another. It causes acute paroxysms of pain often associated with vomiting. An abdominal mass may be present however the clinical presentation of this child is not consistent with intussusception. D Wilms’ tumour – Correct. Wilms’ tumours commonly present with a painless palpable or visible mass in an otherwise well child. Other features include hypertension and haematuria. E Splenomegaly – Incorrect. It should (but not always) be possible to distinguish between an enlarged spleen and renal mass by palpation. An enlarged spleen, unlike an enlarged kidney, is not ballotable and it is not possible to palpate above it. Splenomegaly, however, is not associated with hypertension.
Doctors responsible for examining infants and children must be able to recognise the presence of an abdominal mass, as this may indicate underlying malignancy. Some tumours may only present with an abdominal mass (with no other symptoms). History and examination can help discriminate between potential malignant and benign pathology although all need investigation. The age of the child is also important as some tumours are more common in very young children (e.g. neuroblastoma and hepatoblastoma) and others are more common in older children (e.g. germ cell tumours). A Severe pain – Incorrect. Painful masses are more suggestive of an abscess/abdominal collections, unless the mass has caused abdominal obstruction. B Rapid change in size of abdominal mass – Correct. Again, a mass that is rapidly increasing in size is more suggestive of malignancy, although it may also represent an abdominal collection/abscess. C Systemic symptoms such as fever, tiredness and weight loss – Correct. Although systemic symptoms are not always present, fever, tiredness and weight loss may suggest underlying malignancy. D Presence of blood in the urine – Correct. Children with tumours affecting the renal tract (e.g. Wilms’ tumour or rhabdomyosarcoma) may have microscopic or macroscopic haematuria. Microscopic haematuria is only diagnosed
Clinical Cases: Difficult
IMPORTANT LEARNING POINTS
Key Point Malignancies may present in a child that is otherwise asymptomatic.
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Q3 How would you further investigate this patient?
ANSWER TO CASE 6
The correct answers are A. Urinary homovanillic acid (HVA) and vanillylmandelic acid (VMA), C. Abdominal ultrasound, D. Urine dipstick and E. Chest X-ray.
Q1 Which of the following results is/are likely to be found?
One of the most helpful initial investigations is an abdominal ultrasound scan. Ultrasound can reveal which organ the mass is arising from before being confirmed on more detailed imaging such as CT or MRI. A Urinary homovanillic acid (HVA) and vanillylmandelic acid (VMA) - Correct. Urine levels of VMA and HVA (metabolites of catecholamines) are elevated in most cases of neuroblastoma. B Laparoscopy – Incorrect. Laparoscopy is an invasive test which is not indicated, especially before imaging. Following diagnosis of an abdominal tumour on imaging and tumour markers, a biopsy may be planned, but this is not always the case. C Abdominal ultrasound – Correct. This is the most useful initial investigation to determine the underlying cause. It will reveal which organ the mass originates from and provide diagnostic information before more detailed imaging can be arranged (CT or MRI). Ultrasounds are accessible, do not expose the child to radiation and do not require sedation. D Urine dipstick – Correct. Some tumours affecting the renal tract may cause microscopic haematuria (including Wilms’ tumour and rhabdomyosarcoma). E Chest X-ray – Correct. A chest X-ray is required to look for the presence of lung metastases.
Key Point Differentiating between organomegaly and abdominal tumours can be difficult based on examination findings alone. Investigations including ultrasound and tumour markers provide useful initial diagnostic information. IMPORTANT LEARNING POINTS
• Remember that otherwise well children with an asymptomatic abdominal mass may have a renal tumour. • The location of the mass and presence of additional symptoms and signs can be useful for distinguishing between different tumour types. However, definitive diagnosis is always based on histology of the tissue. • Diagnostic work-up includes imaging (ultrasound, CT/MRI), blood tests (FBC, U&E, LFT, clotting and tumour markers) and urine dipstick (for haematuria and urinary catecholamines). • Suspected abdominal tumours in children should be referred to a paediatric oncology centre for specialist management.
A 28-day-old baby with projectile vomiting
The correct answers are A. Hyponatraemia, C. Hypochloraemia, and E. Acidic urine. The likely diagnosis in this case is pyloric stenosis. There is projectile, non-bilious vomiting, in a baby that is eager to feed. This classically presents with a hypochloraemic metabolic alkalosis. In this case, initial blood gas showed the following: Na Cl K HCO3 pH
133 mmol/L (133-146) 92 mmol/L (96-111) 3.3 mmol/L (3.5-5.5) 31.2 mmol/L (22-26) 7.52 (7.35-7.45)
A Hyponatraemia – Correct. The characteristic biochemical change is a hypochloraemic metabolic alkalosis. This is mainly due to loss of gastric fluids (HCl contains hydrogen and chloride), along with smaller amounts of sodium and potassium. Typically, patients with pyloric stenosis are dehydrated with elevated serum pH and bicarbonate levels. B Hyperkalaemia – Incorrect. There is loss of potassium from gastric fluids. C Hypochloraemia – Correct. Due to loss of chloride from gastric fluids. D Metabolic acidosis – Incorrect. A metabolic alkalosis is the typical finding; this is due to loss of the hydrogen ion in lost gastric secretions. E Acidic urine – Correct. In pyloric stenosis, there is a paradoxical aciduria. Initially, the urine is alkaline to compensate for the metabolic alkalosis. As the condition progresses and dehydration worsens, the kidneys prioritise sodium conservation. As sodium is reabsorbed, potassium and hydrogen ions are excreted (due to the Na/K/H pumps in the kidney) resulting in “paradoxical” aciduria.
Key Point The typical biochemical finding in babies with pyloric stenosis is a hypochloraemic metabolic alkalosis with paradoxical aciduria. Q2 What is/are the most appropriate next step/steps to confirm the diagnosis? The correct answer is B. Test feed. If a palpable “olive” mass was found on examination, no further investigation would be needed. If the abdominal examination is normal, a test feed is the first-line investigation to confirm the
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ANSWERS
A Ultrasound scan – Incorrect. This is not the first line investigation. However, an ultrasound is often done in most centres to confirm the diagnosis. The following three criteria are needed to confirm pyloric stenosis on ultrasound: i) a pyloric muscle thickness >4 mm, ii) a pyloric muscle length >18 mm, and iii) failure of fluid passage beyond the pylorus despite vigorous gastric peristalsis. B Test feed – Correct. A test feed should be performed prior to any other investigation. The baby’s stomach should be empty. This can be achieved by inserting a nasogastric tube into the stomach and draining/aspirating the contents. The baby is then given a small feed of Dioralyte. At the same time, the examiner places their hand in the epigastrium, below the liver edge. The dextrose feed allows the baby’s abdominal muscles to relax and the pylorus to contract. The pyloric tumour can be rolled under the fingers of the palpating hand. Occasionally, gastric peristalsis can be seen during the examination. If the pylorus is palpable, no further tests are needed. If, however, the test feed was not conclusive, an ultrasound scan is recommended. This is often performed by the paediatric surgeon rather than in the referral centre where an ultrasound is a more popular test due to lack of expertise in feeling the abdominal mass after a test feed. C Contrast study – Incorrect. Contrast is usually reserved for cases where ultrasonography was inconclusive. The “string sign” is the diagnostic term given to the delayed passage of a small amount of contrast through a thin, long pyloric canal. D CT scan – Incorrect. This test is not indicated for pyloric stenosis, as sufficient diagnostic information can be yielded from ultrasound/contrast studies. E Endoscopy – Incorrect. This invasive procedure is not required for diagnosis.
Key Point A positive test feed is sufficient to make the diagnosis of pyloric stenosis. This is usually carried out by the paediatric surgeon or an experienced paediatrician. Q3 A fter the diagnosis of “pyloric stenosis with moderate dehydration” has been confirmed, which of the following should you do? The correct answers are A. Insert a nasogastric tube and B. Calculate and commence maintenance fluids. A Insert a nasogastric tube – Correct. A fine (6F or 8F) nasogastric tube should be inserted. Gastric contents should actively be aspirated in regular intervals, in the interim the tube should be allowed to freely drain into a bag.
This minimises the risk of aspiration and allows accurate replacement of gastric fluid losses. B Calculate and commence maintenance fluids – Correct. Meticulous fluid management in babies with pyloric stenosis extremely important. There are three aspects to fluid management: a fluid bolus (as the baby is dehydrated), maintenance fluids (as being kept nil by mouth), and replacement fluids (to replace nasogastric losses). C Insert a catheter – Incorrect. Insertion of a urinary catheter in infants can be traumatic and a source of infection and should be reserved for very sick babies. Therefore it is not routinely done. D Allow the baby to formula feed – Incorrect. Babies with pyloric stenosis are very hungry, but it is very important to keep the infant fasting, along with a correctly placed nasogastric tube. Both actions insure an empty stomach and minimise the risk of aspiration. E Place the baby on the emergency operating list – Incorrect. The standard treatment for pyloric stenosis is surgery, namely a pyloromyotomy. It is, however, very risky to subject a patient with a significant electrolyte/fluid imbalance to general anaesthetic. Therefore, surgery should only be undertaken once fluid and electrolyte imbalances have been resolved and the blood tests have normalised. This can take 48 hours or longer.
Clinical Cases: Difficult
diagnosis of pyloric stenosis, followed by an ultrasound if the diagnosis is still unclear. Many centres will do an ultrasound regardless, to confirm the diagnosis.
Key Point Electrolyte imbalances in a baby can be dangerous. The first step in managing pyloric stenosis is active fluid resuscitation.
IMPORTANT LEARNING POINTS
• Pyloric stenosis is the commonest surgical cause of nonbilious vomiting in infants. • It can be diagnosed clinically, or after a test feed. Otherwise, ultrasound is required. • Pyloromyotomy is the definitive treatment for the condition, but the operation should only be performed once adequate fluid resuscitation has been achieved.
ANSWER TO CASE 7
A 1-day-old boy with desaturations after feeds and drooling Q1 Which steps/investigations would be useful in the acute management of this baby? Select all that apply. The correct answers are B. Chest and abdominal X-ray, and C. Nasogastric tube. The nasogastric tube is inserted first. Typically, there is some resistance in passing it and caution must be taken not to perforate the oesophagus.
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A Chest X-ray only – Incorrect. A CXR would show the potential tell-tale sign of a curled NG tube in the upper pouch of the oesophagus. However, it is likely from the history (polyhydramnios and drooling saliva) that this baby has an oesophageal atresia/tracheo-oesophageal fistula (OA/TOF), so radiology should also include the abdomen to look for stomach and other anomalies. B Chest and abdominal X-ray – Correct. To rule out TOF/ OA, the tip of the NG needs to be seen in the stomach. TOF/ OA can be associated with multiple other gastrointestinal abnormalities (duodenal atresia, malrotation, anorectal malformation), so it is therefore necessary to include the abdomen in the radiograph. C Nasogastric tube – Correct. The curling of the NG tube in the upper pouch is diagnostic of the condition. Once the diagnosis is established on radiographs, the NG tube should be removed and a Replogle tube should be inserted. A Replogle tube can provide continuous suction; an NG tube cannot provide that. D HIDA scan – Incorrect. This test is used for investigation of liver tract abnormalities. This is unrelated to children with TOF/OA. E Renal ultrasound scan – Incorrect. Whilst it is true that renal anomalies are commonly associated with TOF/OA, there is no role for a renal ultrasound scan in the “acute” management of this condition. A renal ultrasound scan should be booked as a routine test after the baby recovers from surgery.
Key Point A high index of suspicion of TOF/OA should be kept when dealing with a baby with an antenatal history of polyhydramnios.
abdominal distension, an increase in intra-abdominal pressure and desaturation of the neonate. If respiratory distress should occur, then it is advisable to intubate the patient and pass the tip of the endotracheal tube past the fistula. B A cross matched unit of blood should be ordered as soon as possible – True. This baby is likely to require urgent surgery; it is therefore necessary to have blood available. C Surgery can be undertaken in a day or two after the baby stabilises – False. If the patient had an isolated TOF/OA, deferring surgery 24-48 hours might be advantageous, but in the case of a baby with both a TOF/OA and an anorectal malformation, it is important to proceed with surgery as soon as safely possible. There is a risk that the baby will develop progressive abdominal distension and secondary respiratory compromise. It can also lead to aspiration of gastric contents, which is extremely serious. D An echo should be performed post operatively – False. An echo has to be performed preoperatively. VACTERL association includes cardiac anomalies. It is important, for the child’s safety under anaesthetic and the surgical approach (a right approach is normal, but if heart or aorta is in the way, it has to be done on the left), to be aware of these conditions preoperatively. E Both conditions should be treated under the same anaesthetic – True. Both conditions are dealt with under the same anaesthetic. The fistula is usually treated first, followed by colostomy formation.
Key Point Patients with both TOF/OA and imperforate anus are high risk patients. Surgery should be planned for as soon as safely possible (usually within the first 24 hours of life). Q3 What other investigations are indicated in this baby?
Q2 R egarding the management of this baby, mark the following statements true or false. This is a very complex patient, requiring multiple urgent surgical interventions, for potentially fatal conditions. Meconium in the urine implies a recto-urethral or a rectovestibular fistula. The priority in this case is stabilising the patient, identifying any operative risk factors (doing an echocardiogram), and operating on both pathologies simultaneously. The correct answers are B. A cross matched unit of blood should be ordered as soon as possible, and E. Both conditions should be treated under the same anaesthetic. A If the baby desaturates again, bag and mask ventilation is first line of management – False. Bag and mask ventilation will cause a severe deterioration in the baby’s condition and should not be undertaken. The baby has a tracheo-oesophageal fistula. Forcing air into the trachea will lead to a significant proportion of air escaping into the gut. This will lead to progressive
Correct answers are A. Renal ultrasound scan, C. Ultrasound scan and X-ray of the back, and D. MCUG. This patient has several features of VACTERL association. This encompasses vertebral anomalies, anorectal malformations, cardiac defects, tracheoesophageal fistula and/or esophageal atresia, renal/radial anomalies and limb defects. It is therefore important to look for other possible abnormalities in this set, even in the asymptomatic child. A Renal ultrasound scan – Correct. As the VACTERL anomaly spectrum includes a range of renal and bladder anomalies, it is important that a thorough renal ultrasound scan is performed after the baby is stabilised. B CT of the abdomen and pelvis – Incorrect. There is no role for a CT scan in this scenario. The other mentioned investigations are sufficient to provide the relevant clinical information and do not expose the patient to the high dose of radiation associated with a CT.
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ANSWERS
Key Point TOF/OA and anorectal anomalies are part of the VACTERL association. It is important to investigate the patient for non-apparent anomalies, such as cardiac defects and renal and spinal anomalies.
IMPORTANT LEARNING POINTS
• Oesophageal atresia should be suspected in “mucusy” babies who don’t tolerate feeds. • If in doubt, a nasogastric tube can gently be inserted. This should be followed by a chest X-ray. • There are many variants of anorectal malformation. Two-thirds of patient have a communicating fistula to the genito-urinary tract, and one-third do not. • Patients with anorectal anomalies should be placed on prophylactic antibiotics as the communication between gut and urinary tract can lead to severe urinary sepsis and renal scarring.
ANSWER TO CASE 8
A 4-year-old boy presenting with fever and a rash Q1 After being intubated and ventilated, Jacob’s arterial blood gas shows a pH of 7.20, a pO2 of 10kPa, a pCO2 of 8.2 kPa, and a BE of -7. Which of the following adjustments will theoretically improve ventilation? The correct answer is C. Increase the peak inspiratory pressure. This question assesses knowledge on increasing CO2 clearance by adjusting ventilator settings. Increasing the respiratory rate or increasing the PIP are the most commonly used changes.
A Increase the oxygen concentration – Incorrect. This will not affect CO2 clearance and is not needed, as the oxygenation is currently normal. B Reduce the respiratory rate – Incorrect. Reducing the respiratory rate will reduce CO2 clearance, thereby increasing the pCO2. C Increase the peak inspiratory pressure – Correct. Increasing the peak inspiratory pressure will increase the tidal volume and will therefore increase the minute volume (minute volume = respiratory rate x tidal volume). This will increase the CO2 clearance, thereby reducing the pCO2. D Change the endotracheal tube to a laryngeal mask airway – Incorrect. Changing the airway support is unlikely to have a significant effect on the CO2 clearance, unless it is felt that there is a problem with it, e.g. blockage with secretions. E Increase the peak end expiratory pressure – Incorrect. Increasing peak end expiratory pressure may improve oxygenation but it will reduce the tidal volume, as the pressure difference between inspiration and expiration is reduced.
Clinical Cases: Difficult
C Ultrasound scan and X-ray of the back – Correct. Anomalies of the vertebrae, sacrum and coccyx (and underlying problems of the spinal cord and rectal/bladder innervation) can be part of the VACTERL association. It is therefore important to perform these tests, particularly given the skin dimple noted on examination (which is associated with spina bifida). D MCUG – Correct. A micturition cysto-urethrogram is indicated in patients with anorectal malformations with a urinary tract fistula. The bladder is frequently abnormal and is associated with vesicoureteric reflux. E Genetic workup – Incorrect. If the baby has no features suggestive of a syndrome, this is not necessary. If, however, the neonate has features suggestive of Down syndrome (or other abnormal features that do not fit the VACTERL association), a full genetic workup is warranted.
Key Point To increase CO2 clearance on the ventilator, the respiratory rate can be increased or the peak inspiratory pressure can be increased. Q2 Jacob’s BP has remained stable, but his hands and feet are still cool to touch, with weak pulses. His capillary refill time remains 3 seconds. His HR is now 165 bpm. He has not produced urine despite being catheterised in ED. What should be done next? The correct answer is B. Give another 20 mL/kg bolus of 0.9% sodium chloride. Circulatory compromise in septic shock needs to be aggressively managed. The first-line management is fluid boluses, and up to three boluses may be given in the first 30 minutes of resuscitation. After this, a definitive airway should ideally be achieved before giving further boluses, as there is a risk of precipitating pulmonary oedema. A Prepare to commence haemofiltration – Incorrect. Haemofiltration is not likely to help regain optimal perfusion of the tissues, as it only filters the blood, rather than improving tissue perfusion. Furthermore, this is not available in ED. B Give another 20 mL/kg bolus of 0.9% sodium chloride – Correct. Jacob’s clinical picture shows that he remains shocked despite fluid therapy. Children with meningococcal sepsis, such as Jacob, may require significant volumes of fluid to maintain adequate perfusion.
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Clinical Cases: Difficult
C Give 250 mL of colloid fluid – Incorrect. Fluid resuscitation should be with 0.9% sodium chloride, not with colloid, which is no longer recommended for use in children. Colloid may precipitate allergic reactions and is thus avoided. D Start inotropic support with milrinone – Incorrect. Whilst inotropic support is likely to be needed on PICU, the emphasis in the immediate phase should be on aggressive fluid resuscitation. After a third bolus, dopamine may be considered as it can be given peripherally. E Recheck his renal function – Incorrect. Sending a sample to the lab may give useful information, but will not influence the acute management.
Key Point Recognising and promptly treating shock is of paramount importance. This starts with aggressive fluid resuscitation, and up to 60 mL/kg can be given in the first 30 minutes. Delays can result in children developing decompensated shock (with hypotension), which can be a pre-terminal event.
Q3 Jacob’s mother is understandably distressed by his condition. She says he has not eaten all day, and she is worried about his nutrition. She asks if he can have some milk. What is the safest advice you can give in ED? The correct answer is D. Jacob will not be fed at present, but will receive IV fluids. In this situation, nutritional needs are secondary to acute resuscitation. The child may require imminent intubation, where an empty stomach is preferable. There may also be some gut ischaemia, as the child is in shock. Therefore, enteral feeding should be avoided. A Jacob can have some milk via a NG tube – Incorrect. Jacob is clinically shocked, and is likely to have an element of gut ischaemia due to poor perfusion. Therefore, he should not be fed enterally for the acute phase, in order to avoid complications. B Jacob will have TPN started by NG tube – Incorrect. TPN is administered via a central line. C Jacob will have TPN started via a central line in ED – Incorrect. For TPN, Jacob would need to have a dedicated line with full aseptic precautions. It should therefore be started in a controlled fashion, with full aseptic precautions taken, rather than in the acute resuscitation phase. TPN is also potentially toxic, so a decision as to whether it is needed should be made once the likely disease course is noted. D Jacob will not be fed at present, but will receive IV fluids – Correct. Jacob should not be fed enterally during the acute phase, in order to avoid complications. He will receive intravenous fluids (usually 0.9% sodium chloride with
5% dextrose) in order to maintain hydration and adequate blood glucose concentration. E A dietician will need to be consulted in ED before making a decision about feeding enterally – Incorrect. This decision making skill should be within the doctor’s skill set.
Key Point Enteral feeding is preferred in the stable patient. In the shocked patient, however, it can result in adverse effects in the setting of gut tissue ischaemia due to poor perfusion. IMPORTANT LEARNING POINTS
• Resuscitation should follow an ABCDE approach. • Resuscitation requires careful management of oxygenation and ventilation. These are manipulated slightly differently on the ventilator: oxygenation predominantly by changing mean airway pressure/FiO2, and ventilation predominantly by changing peak inspiratory pressure and respiratory rate. • In severe sepsis, it is important to aggressively fluid resuscitate early, with up to three fluid boluses required in the first 30 minutes. Dopamine can be started peripherally if needed. • Acutely, nutritional needs come secondary to managing shock. Therefore patients should be made NBM in severe sepsis, with consideration of TPN if enteral feeding is delayed for a prolonged period.
ANSWER TO CASE 9
A 3-day-old baby presenting with weak femoral pulses Q1 Which of the following investigations are essential in the assessment of this baby? The correct answers are A. Baseline bloods including CRP, blood cultures, and blood gas, B. Blood glucose, C. Four-limb BP, D. Echocardiogram, and E. Electrocardiogram. When presented with an unwell infant, consider common and serious differential diagnoses. In this case, the most likely differential diagnoses are sepsis or cardiac issues, although metabolic disorders can also present this way. Coarctation of the aorta is the most likely diagnosis. This is a narrowing in the descending aorta, which restricts the volume of oxygenated blood circulated to the body. This narrowing may cause back pressure through the heart and pulmonary circulation resulting in heart failure. These symptoms occasionally occur after 48 hours of life due to closure of the ductus arteriosus. Prior to the closure, the patent duct provided an additional route through which blood could escape, thus preventing the symptoms of heart failure. Other investigations must be undertaken as soon as possible. If a
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ANSWERS
A Baseline bloods including CRP, blood cultures, and blood gas – Correct. The blood gas may show a metabolic acidosis due to hypoperfusion. Sepsis should be strongly suspected in any neonate that acutely deteriorates; therefore, broad spectrum antibiotics should be started. B Blood glucose – Correct. Hypoglycaemia can exacerbate any existing problems and can in itself be fatal, if left untreated. C Four-limb BP – Correct. Classically, when measuring fourlimb BP (exactly as it sounds – measuring the BP in both arms and both legs) if an infant has coarctation, their upper limbs measure BP at least 20 mmHg greater than the lower limbs. This is again due to the increased pressure proximal to the narrowing of the aorta. Although a very valuable investigation if positive, this investigation is not a gold standard for diagnosis for coarctation of the aorta because of a high rate of false negatives. D Echocardiogram – Correct. Though it is unlikely that an echocardiogram can be performed acutely in most peripheral hospitals, it will give a definitive diagnosis. E Electrocardiogram – Correct. Cardiac monitoring and ECG may be useful investigations. They may show right ventricular hypertrophy. As the afterload increases in the systemic circulation, there is shunting across the foramen ovale from the left atrium to right atrium. This increases volume in the pulmonary circulation, and hence leads to right ventricular hypertrophy.
Key Point Although a baby might be born with a coarctation, they often deteriorate around day three of life. This is due to a ductus arteriosus being patent before this, and allowing blood to shunt around the narrowing. Q2 Y our team makes a diagnosis of coarctation of the aorta. Given this presentation, which two of the following options would you add to your management in addition to the general approach to an acutely unwell patient? The correct answers are A. Prostaglandin infusion, and C. Antibiotics in line with trust guidance on neonatal sepsis. The acute deterioration implies a trigger that has worsened the underlying coarctation. This is most likely due to closure of the ductus arteriosus (given that it is on day three), but it may also be due to a possible infection. Therefore both these possibilities need to be covered. A Prostaglandin infusion – Correct. This infant has presented on day three of life with symptoms suggestive of heart
failure. This indicates that the duct closure has contributed to the symptoms. In order to alleviate these symptoms, it is reasonable to attempt to re-open the duct with a prostaglandin infusion. This treatment is not without risk, and a major common side effect is apnoea. Prostaglandin infusion should only be commenced at the request of a senior registrar or consultant and on discussion with a tertiary cardiology centre. B DC cardioversion – Incorrect. There is no indication for DC cardioversion in this patient. He is conscious and has a regular HR. C Antibiotics in line with trust guidance on neonatal sepsis – Correct. Neonatal sepsis can present in a myriad of different ways, and although we assume that this infant’s heart failure is a result of his coarctation, it could also be sepsis. It is therefore prudent to take blood cultures and commence the baby on broad spectrum antibiotics as per local guidelines. D Atropine – Incorrect. Atropine is a drug which speeds up conduction through the AV node within the heart thus increasing HR. It is not indicated in this scenario. E Indomethacin infusion – Incorrect. Indomethacin is a cyclooxygenase inhibitor which is used to close a patent ductus arteriosus in premature infants. It does this by inhibiting prostaglandin synthesis.
Clinical Cases: Difficult
provisional diagnosis of “duct dependent cyanotic heart disease” is suspected, the management should be intubation, treatment with prostaglandin infusion and urgent transfer to a cardiac unit.
Key Point Do not forget that in any acutely unwell neonate, it is generally prudent to assume there may be an infection. Take blood cultures and commence intravenous antibiotics. Q3 A medical student in the paediatric department asks for tips on feeling for pulses during the standard neonatal examination. Which of the following statements is correct? The correct answer is A. Femoral pulses can be difficult to feel and if they are unsure, they should ask a senior to review the baby. A Femoral pulses can be difficult to feel and if they are unsure, they should ask a senior to review the baby – Correct. Femoral pulse palpation in neonates is a simple screening test for coarctation of the aorta. It should be incorporated into every routine infant examination. It can, however, be very difficult to feel femoral pulses, especially in a crying baby. Sometimes, it can take a few moments to isolate where the femoral pulses are and palpate them adequately; even experienced clinicians often take a minute or so to ensure that this is done. Consider gently holding the baby’s leg straight and exerting gentle pressure over the inguinal region with the index finger. B If the perfusion, colour and tone of the lower limbs is normal, palpation of the femoral pulses is not needed – Incorrect.
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Clinical Cases: Difficult
This is false as often babies will be able to perfuse their lower limbs despite a coarctation. C Weak and low volume femoral pulses are pathognomonic for coarctation of the aorta – Incorrect. Although palpation of femoral pulses is a useful screening examination for coarctation, weak pulses do not always demonstrate a coarctation. An important differential is that of shock, as the definition of shock is poor perfusion, which would mean the pulse volume is low. D It is best to feel for femoral pulses at the same time as assessing hip stability to save time – Incorrect. Try to concentrate on one aspect of the examination at a time to ensure that nothing is missed. E When palpation of the pulses is impossible, often the pulse can be heard when the femoral area is auscultated with the bell of a stethoscope – Incorrect. The femoral pulses should not be audible. If the femoral artery can be auscultated, it would imply turbulent blood flow in that area, which could indicate other pathology.
Key Point
D Cyanosis – Incorrect. Coarctation of the aorta is an acyanotic heart condition, and any signs of cyanosis should be thoroughly investigated as this would indicate significant hypoxia. This is not a recognised complication of coarctation repair. E Rib notching on X-ray – Incorrect. Rib notching is an X-ray finding which becomes present in children 4 to 12-years-old with an untreated coarctation. It is caused by erosion of the posterior surface of the ribs by large collateral vessels which have developed to support the systemic circulation. In a neonate, these collaterals have not yet developed, and it takes time for these collaterals to alter the contours of the ribs. When a coarctation is treated early, large collateral vessels do not have the time to form.
Key Point Patients with coarctation and other congenital heart disease may have lifelong complications, despite corrective treatment. IMPORTANT LEARNING POINTS
Coarctation of the aorta is a potentially fatal condition if not adequately managed. Newborn examinations are a vital time in which the diagnosis can be suspected, so always carefully check for femoral pulses. Q4 F our years later, you are a registrar in clinic and the same child comes to see you, having had corrective surgery. He is asymptomatic and thriving. What residual features would you expect to be present?
• It is important to assess femoral pulses on newborn examination, as this is an opportunity to diagnose coarctation of the aorta before it becomes symptomatic. • Coarctation of the aorta typically presents on day three of life, but can occur anytime in the first two weeks of life, as the ductus arteriosus may close later. Prostaglandin infusions keep the duct open and can be lifesaving. • Even in treated cases of coarctation, there may be longterm complications such as hypertension.
The correct answer is C. Hypertension. A Absent femoral pulses – Incorrect. Following repair of coarctation, there will no longer be a narrowing of the descending aorta and therefore no reason to find absent femoral pulses. B Brachio-femoral delay – Incorrect. The brachio-femoral delay is caused by the position of the narrowing in the descending aorta (after the branches of the brachiocephalic, common carotid and subclavian arteries), resulting in higher pressure and volume of blood being delivered to the upper limbs. When the narrowing has gone, this issue will no longer exist. C Hypertension – Correct. Several studies have investigated the presence of hypertension in children and young adults following successful repair of coarctation in infancy. The results show that hypertension remains despite adequate repair. It is hypothesised that this is due to reduced arterial reactivity, leading to a higher pressure of blood flow in order to gain vessel response.
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3.01
HISTORY TAKING ZESHAN QURESHI, CHRISTOPHER HARRIS AND MICHAEL MALLEY
INTRODUCTION
W ӹӹ
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CONTENTS 507 Introduction 507 Preparation 508 Presenting Complaint 510 Past Medical History 510 Birth History 511 Feeding History 511 Developmental History 511 Drug History and Immunisations 511 Family History 511 Social and Travel History 512 Systems Review 512 Concluding 512 Presenting Your Findings 512 Clinical Cases
512 Case 1: A 2-day-old baby presenting with jaundice 515 Case 2: A 6-week-old baby with a temperature 517 Case 3: A 13-year-old boy presenting with a wheezy episode 520 Case 4: A 5-year-old boy presenting with a swollen leg 523 Case 5: A 14-year-old girl presenting with acute abdominal pain
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ӹӹ
ӹӹ
ӹӹ ӹӹ
hen approaching a potentially unwell child, it is important to remember that children are not just small adults. Bear in mind the following: The child’s parents, relatives or carers may not have the whole story, especially if the child has been in someone else’s care, so a collateral history may be necessary. The parents’ “sixth sense” about their child’s health should always be taken seriously. Equally, parents may underestimate the seriousness of their child’s condition. Younger children may not be able to give any history, or the history given may be misleading. All children, but particularly younger infants, may have underlying congenital abnormalities that have remained undetected until presentation in extremis. Children can compensate physiologically for severe illness. As such, signs of deterioration may not be evident until late in the illness course. Children are dependent on adult carers for many things, particularly in early life. This can lead to opportunities for abuse, which can be easily missed if not specifically assessed. Decisions made during childhood with regards to health can impact on all areas of development. Pay special attention to putting the child at ease. The doctor should make a point of introducing themselves to the child individually, and perhaps let them lead introductions to the rest of the family. Toys are widely available in paediatric healthcare settings and they can be used effectively to relax younger children.
PREPARATION Usually, there will be time to prepare before seeing a child. During this time, review any pre-existing information. This may include: ambulance triage notes, nursing triage notes, referral letters, previous clinic letters, hospital discharge letters and current hospital notes. Often, allergies or social concerns are automatically highlighted on hospital computer notes. There may also be a handover from a colleague: don’t be afraid to ask them questions to clear uncertainty. Blood tests and imaging may have been requested, or recent investigation results may be available. This will give an initial impression of the key issues and the parent/patient agenda before going into the consultation.
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History Taking
Start by greeting the parents and child. Let them know who you are and why you are seeing them. Throughout the consultation: 1 Be confident, using good eye contact and open body language. 2 Be empathetic, ensuring all those in the room are at ease. 3 Ensure that the parents and child understand what is happening in the consultation. Give them time to speak, and explore their feelings with regard to each pertinent issue. 4 Avoid medical jargon. Explain any unfamiliar words or concepts in simple terms.
PRESENTING COMPLAINT History-taking should begin with open questions. It is worthwhile allowing the child or family member to explain
the problem in their own words. Resist the temptation to intervene with questions too quickly. Once they have finished, both open and closed questions can be used to clarify information. What is the problem? How long has it been going on? Has the child been well between symptoms? How has this problem affected the child’s development, schooling and home life? Focus on the main symptoms. Mnemonics can be helpful to explore symptoms. Examples of those commonly used are shown in Box 1. Think about relevant associated symptoms to the presenting complaint that may help “clinch” the diagnosis. It may be easier to think in terms of “boxes”, or a group of questions which immediately elucidate particular presenting complaints. Examples are shown in Table 1.
Box 1: Helpful mnemonics for history taking SOCRATES
ODPARA
P3MAFTOSA
Site. Onset. Character. Radiation. Associated features. Timing. Exacerbating and alleviating factors. Severity.
Onset. Duration. Progression of the main presenting complaint. Aggravating factors. Relieving factors. Additional symptoms.
Presenting complaint. Past medical history. Personal history i.e. feeding/growth/ development/birth history. Medicines. Allergies. Family history. Travel history. Other factors. Social factors. Alleviating/relieving factors.
TABLE 1: Common symptoms to consider in history taking Symptom
Key Points
Differential Diagnoses
Fever.
• • • • • • • • •
Cough/coryza. Difficulty breathing. Vomiting/diarrhoea. Urinary symptoms. Rigors. Rashes. Joint pain/inflammation. Signs of meningism, including infectious contacts. Foreign travel.
• • • • • • • • •
Vomiting.
• • • • • • • • • • •
Timing (as related to feeds, or in early morning). Substance vomited (bilious or bloody). Diarrhoea. Abdominal pain. Obstructive symptoms. Fever. Urinary symptoms. Signs of meningism. Symptoms of diabetic ketoacidosis (DKA). Foreign travel or infectious contacts. Weight loss.
• Gastroenteritis. • Appendicitis. • Obstruction, e.g. volvulus, adhesions, malrotation, pyloric stenosis in neonates. • UTI. • Meningitis. • DKA. • Raised intracranial pressure (ICP).
Upper respiratory tract infection (URTI). Pneumonia/bronchiolitis. Gastroenteritis. Appendicitis. Urinary tract infection (UTI). Otitis media. Septic arthritis/osteomyelitis. Soft tissue infection, e.g. abscess, cellulitis. Meningitis/encephalitis.
Continued
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Presenting Complaint
TABLE 1: Continued Key Points
Differential Diagnoses
Wheeze.
• • • • • •
• • • • • • •
Faltering growth.
• Weight/height trajectory.
Onset and progress of wheeze. Treatment at home. Trigger. Personal or family history of atopy. Previous admissions and severity. Effect on life (e.g. missing school).
Food in: • Diet content. • Diet volume. • Liquid intake. Food out: • Diarrhoea (including steatorrhoea). • Vomiting. • Reflux symptoms. Increased metabolic requirements: • Chronic illnesses. • Malignancy.
Asthma. Bronchiolitis. Viral induced wheeze. Foreign body inhalation. Anaphylaxis. Gastro-oesophageal reflux. Cardiac failure.
Broad range of differentials including: • Malabsorption. e.g. coeliac disease, inflammatory bowel disease (IBD), cystic fibrosis (CF), chronic liver disease, chronic diarrhoea/vomiting. • Perinatal factors. e.g. intrauterine growth restriction (IUGR), prematurity. • Endocrine. e.g. growth hormone deficiency, diabetes, thyroid disorders. • Genetic. e.g. Turner syndrome, achondroplasia, Russell-Silver syndrome. • Increased metabolic requirements. e.g. recurrent infections, cardiac disease. • Non-organic. e.g. neglect/child abuse, inappropriate diet.
History Taking
Symptom
Other factors: • Low socio-economic status or markers of neglect. Rash.
• • • • •
Distribution. First noticed when and where. Itchy/non-itchy. Blanching/non-blanching. Type of lesion – macule/papule/vesicle/blister/ purpura. • Mucosal involvement. • Atopic history, allergic contacts. • Associated symptoms e.g. fever, joint pains, abdominal pain, bleeding symptoms.
Macular/papular rashes: • Viral rash (including measles/rubella). • Sandpaper rash = Streptococcal infection. • “Salmon pink” rash = Juvenile idiopathic arthritis (JIA). • Eczema. • Contact dermatitis. Vesicles: • Chickenpox/shingles. • HSV (think infection of underlying eczema: eczema herpeticum). Purpura: • Meningococcal sepsis. • Henoch-Schönlein purpura (HSP). • Idiopathic thrombocytopenic purpura (ITP). Wheal and flare: • Allergy. • Viral urticaria. Primary skin infection: • Cellulitis. • Hand-foot-and-mouth disease. • Impetigo. • Ringworm.
Seizure.
Think chronologically: • What happened before the seizure: aura, trauma, fever? • What happened during the seizure: absence, focal, generalised, tonic/clonic, tongue biting, incontinence? • Duration of seizure. • Medications given. • What happened after the seizure: post-ictal phase? • Previous seizures. • Red flag symptoms: weight loss, focal neurology, persistent reduced GCS post seizure, seizure >15 minutes duration, non-blanching rash or other signs of meningism, past history of trauma.
• • • • • • • •
Limp.
• • • • • •
Think in terms of age: All ages: • Septic arthritis. • Osteomyelitis. • Trauma or physical abuse. • Malignancies (e.g. leukaemia).
Pain. Recent viral symptoms. Fever. Trauma. Birth history (especially breech delivery). Elevated BMI (particularly in teenagers).
Epileptiform seizure. Febrile convulsion. Non-epileptiform or pseudo-seizure. Hypoglycaemia. Electrolyte imbalance. Infection. e.g. meningitis/encephalitis. Malignancy. Trauma/elevated intracranial pressure (ICP).
Continued
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History Taking
TABLE 1: Continued Symptom
Key Points
Differential Diagnoses
• Any systemic symptoms e.g. diarrhoea, rashes, extraarticular manifestations of JIA. • Signs of malignancy, particularly haematological. Think: i) general sign, e.g. weight loss/malaise/growth delay; ii) red cell lineage, e.g. signs of anaemia; iii) white cell lineage, e.g. recurrent infections and iv) platelet lineage, e.g. bleeding/bruising). • Positive family history.
Under 5 years: • Developmental dysplasia of the hip. • Transient synovitis. • Haemophilia. 5–12 years: • Perthe’s disease. • Transient synovitis. • IBD/rheumatological disease. >12 years: • Slipped upper femoral epiphysis. • Juvenile idiopathic arthritis. • Other rheumatological causes.
Blood in urine.
• • • • • • • • •
Painful or painless. Microscopic or macroscopic (frank). Isolated or with proteinuria. Infective symptoms e.g. fever, dysuria, frequency. Abdominal pain. Oedema/signs of nephrotic involvement. Trauma. Recent viral infections. Other health concerns: HIV infection, hepatitis B infection, diabetes, haemoglobinopathy. • Menstrual cycle. • Signs of bleeding elsewhere. • Family history of renal disease.
Non-haematuria causes: • PV bleeding/menstruation. • Oxalate crystals in babies. • Myoglobinuria. Non-glomerular causes: • UTI. • Renal vein thrombosis. • Malignancy. • Renal stones. Glomerulonephritis: • Post-infectious glomerulonephritis. • IgA nephropathy. • HSP • Systemic lupus erythematosus (SLE).
Possible NonAccidental Injury.
• • • • • •
• Accidental injury (including pathological fractures, e.g. with osteogenesis imperfecta). • Non-accidental injury.
Establish mechanism of injury and any complications. Timing of injury and delay in presentation. Witnesses of the injury. Previous hospital visits. Any other injuries. Detailed social history including household members, carers. • Social worker involvement.
Be quantitative. As children often cannot report subjective things themselves, it is important to get objective evidence. In acute histories, ask how many times they have passed urine in the last 24 hours and establish their fluid intake, whether in bottles, cups or breastfeeds. Quantify the episodes of vomiting and diarrhoea in a similar manner.
PAST MEDICAL HISTORY The past medical history not only gives important hints to the current diagnosis but also to the risk of future deterioration. For example, an infant who spent three months ventilated on the neonatal unit and is now presenting with wheeze is at high risk of rapid deterioration. Most children have no chronic illnesses, and those that do usually only have one condition to enquire about. Take a detailed history of each underlying disease. Key questions to ask in common chronic conditions are shown in Table 2. In general, for any chronic disease find out: ӹӹ Age at diagnosis. ӹӹ Current medication, and healthcare professionals involved in care. ӹӹ Hospital admissions associated with the disease, and treatment received. ӹӹ Specific treatment plans in place (e.g. children with
metabolic disorders usually have an algorithm in place for how they need to be managed in the event of deterioration). ӹӹ Progression of illness, i.e. stable/worsening/getting better. ӹӹ Impact of disease on the child’s life. Also ask about any operations. Other screening questions may include whether the child ever sees their primary care physician, and how often they have visited the emergency department (ED).
BIRTH HISTORY At a minimum, ask the gestation of the pregnancy, any major complications in pregnancy or birth and whether the child was admitted to a neonatal unit. Note that birth history is less important in a clinically well child more than 2-years-old. Parents of neonates who had a prolonged stay in the neonatal unit are likely to keep a copy of their discharge letter. Note: ӹӹ Gestation at birth and any antenatal concerns (including anomalies on scans). ӹӹ Mode of delivery and indications. ӹӹ Need for resuscitation and initial APGAR scores (if known). ӹӹ Birth weight/centile, and whether this remained stable, deteriorated or improved.
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Social and Travel History
TABLE 2: Examples of disease specific questions to ask Key details
Asthma.
• Age at diagnosis, current management, recent investigations (including peak flow) and whether the disease is stable/ worsening/improving. • Number and severity of hospital admissions (including use of intravenous therapy, and intensive care admissions). • Frequency of steroid treatment for acute exacerbations. • Frequency of salbutamol inhaler usage when well. • Triggers. e.g. household smoking. • Other impacts of the disease, e.g. poor exercise tolerance, weight loss.
Inflammatory bowel disease.
• Age at diagnosis and subtype of disease, including any endoscopy or imaging results. Recent investigations, current management and whether the disease is stable/worsening/improving. • Number and severity of acute exacerbations, including hospital admissions and treatment. • Other impacts of the disease, e.g. faltering growth.
Type 1 diabetes.
• • • •
Epilepsy.
• Age at diagnosis, current management and compliance. • Frequency/type of seizures, including hospital admissions and in-patient treatment. • Other impacts of the disease, e.g. side effects of medication.
Cerebral Palsy.
• Level of functionality, including any carers required and any assisted feeding. • Details of recent hospital admissions, and any long-term medications. • Ceiling of care, if in place.
Age at diagnosis and current management. Quality of glycaemic control, including most recent HBA1c result. Frequency of hypoglycaemic or hyperglycaemic episodes, including hospital admissions and diabetic ketoacidosis. Other impacts of the disease, e.g. faltering growth.
ӹӹ Any respiratory support required, including number of days with each treatment (e.g. intubated for five days). ӹӹ Significant diagnosis whilst an inpatient, e.g. group B streptococcus, necrotising enterocolitis. ӹӹ Fitness at discharge, including feeding or oxygen support. ӹӹ Medications given at discharge.
requires a detailed assessment of all four developmental domains (gross motor, fine motor, vision/hearing and social/self-care). If presenting with a simple unrelated condition like a viral infection, a much briefer history is acceptable.
FEEDING HISTORY
When taking a drug history, include doses and frequencies. Ask the parent if they have a list of their child’s medications with them. Specifically inquire about allergies, including non-drug allergies and the nature of the allergic reaction. Check that the child is up-to-date with immunisations. Make note of over-the-counter medications and vitamin supplements. Compliance is also important, particularly with inhalers for asthma and insulin for diabetes, so ask if there are any issues with taking prescribed medications.
Feeding history is most relevant to neonates. It is important to enquire whether they are breast fed or bottle fed, how often they feed and how much they consume. Other indicators of feeding quality are weight gain and number of wet nappies. In older children, going off food is relevant, but it is much more important to ensure they have adequate oral intake throughout illness (at least 50% of normal consumption).
DEVELOPMENTAL HISTORY Simple screening questions may include: Do you have any concerns about your child’s development? Has anyone else mentioned concerns? How does your child get on at school? How does he/ she compare to his siblings? A child presenting with faltering growth
DRUG HISTORY AND IMMUNISATIONS
FAMILY HISTORY Relevant questions include: Do any illnesses run in the family? Is there any history of childhood death or recurrent miscarriage in the family? It is also important to assess for consanguinity,
History Taking
Conditions
and a polite way to ask this is: Were the parents related before marriage? Genetic conditions may present with sudden infant death or miscarriage that may go undiagnosed even at post mortem. This history should prompt further screening. Chronic illness in the family requiring medication can increase the possibility of accidental ingestion of medications by inquisitive young children or purposeful overdose by older children.
SOCIAL AND TRAVEL HISTORY Social history provides insight into the child’s normal day-to-day life. Relevant questions include: Who stays with the child? Who was looking after the child when they became unwell? Are any of those who live at the same address unwell? Are there any problems with damp or pests? Are there smokers in the household? Travel history is particularly relevant if the child is presenting with infective symptoms. The key question is: Has the child travelled abroad recently? Do not feel awkward asking sensitively about a child’s home situation. At a minimum, find out who lives with
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History Taking
the child, and who else, if anyone, is involved in their care (e.g. au pairs, partners, grandparents). Ask about their progress in school. Ask about social services involvement. It can be useful to normalise this question and phrase this as: This is a question we have to ask to everyone. Have you or Jamie ever had any help from social services or had a named social worker? If the answer is yes, then explore the nature of these social concerns.
SYSTEMS REVIEW Ask general questions about health, covering neurology (headaches, drowsiness, fits), cardiology (palpations, chest pain, dizzy episodes), gastrointestinal (vomiting, bowel movements, abdominal pain, weight loss), respiratory (wheeze, breathlessness, chest pain), skin (rashes), orthopaedics/rheumatology (joint/ bone pain), genitourinary (pain on urination, increased urinary frequency, bed wetting), haematology (bruising, bleeding). This can be brief in a focused history.
CONCLUDING Make sure the main ideas, concerns and expectations of the parent/child have been elicited: Is there anything you think I’ve missed or anything you are particularly concerned about? Do you have any questions for me at the moment? To end, summarise the key points and give the parent/child an idea of what to expect now. This can be brief; for example: So, just to summarise, Jamie has had three previous admissions with exacerbations of Crohn’s disease and has presented today with diarrhoea and vomiting over the last 12 hours. You are worried he might have another exacerbation. I’ll just examine Jamie and then we can discuss what is going on in more detail.
PRESENTING YOUR FINDINGS The history may be presented to a senior clinician for review, to a colleague at
handover, or to an examiner during an assessment. It is helpful to open with a summary of the history and then detail the important positive and negative findings of the history. After presenting the history, suggest your next steps, i.e. examination, investigations and initial therapy. For example:
Snapshot “Alex is a 7-year-old boy, who presented with mum to ED this afternoon. He has a 24 hour history of difficulty in breathing, consistent with a mild-to-moderate exacerbation of asthma, requiring admission.”
Current Presentation “Alex has had a cough for two days and has developed progressive difficulty in breathing over the last 24 hours. He has a low grade temperature and a dry cough. He has been using two puffs of his salbutamol inhaler every four hours without improvement, but is still speaking in complete sentences.”
smokers in the house and the family have never had any involvement with social services.”
Summary and Plan “In summary, Alex is a 7-year-old boy presenting with a likely acute exacerbation of his asthma. I would like to proceed to examine Alex, review his observation chart, perform a peak flow measurement and consider oral steroid/salbutamol therapy.”
CLINICAL CASES
CASE 1
A 2-day-old baby presenting with jaundice Doctor Briefing You are asked to see Jamie, a 2-day-old baby on the postnatal ward who is jaundiced. Jamie is mum’s first baby and she has had trouble establishing feeding. Please take a history from his mother, with a view to making a diagnosis.
Background about Asthma
Mother (actress) Briefing
“Alex is a known asthmatic, diagnosed at 3-years-old, and is on regular beclomethasone and PRN salbutamol. He has had three hospital admissions this year and four courses of oral steroids for acute exacerbations. Two years ago, he was admitted to the paediatric intensive care unit (PICU) for three days, receiving IV salbutamol, and being intubated. His known triggers are pet dander and URTI. He also has a history of eczema. His normal peak flow reading is around 300 L/min. He misses about two days of school per month due to his asthma.”
Jamie is your first baby, and you delivered five days ago at Queen Hospital. The baby was born at 36 weeks, with a birth weight of 2300 g, by normal vaginal delivery, and cried at delivery. You had one temperature of 38.2°C during labour and one dose of antibiotics just before delivery. The pregnancy was fine from your perspective: all your scans and blood tests were normal. Your blood group is A+. You have had difficulty establishing feeding and don’t feel like Jamie has had a good feed yet, despite trying every two to three hours. In retrospect, you think the jaundice started yesterday on day 1 of life. You are in a stable relationship with your husband and are hoping to go home today. You are concerned because you don’t know what is causing the jaundice and because you are worried that you might have to bottle feed your baby if he doesn’t take to the breast. If specifically asked, Jamie’s dad has alpha-1-antitrypsin deficiency.
Additional Background Information “Alex was born at term without complication and there have been no concerns about his growth and development. He takes no other medications, has no allergies and his immunisations are up-to-date. Alex’s mother has asthma and his father has hay fever. Alex lives in a flat with his parents and his 5-year-old brother. There are no
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CASE 1: A 2-DAY-OLD BABY PRESENTING WITH JAUNDICE
Assessment
History of Presenting Complaint (Jaundice and Poor Feeding) Question
Justification
Answer
Evolving thought process
When did you first notice the jaundice?
Jaundice on day 1 of life is more worrying and may relate to infection/haemolysis.
“Day 1.”
A bit more worried, since early onset.
How are you feeding Jamie, how often, and how is that going? Is Jamie latching on to the breast? How long is each feed?
Dehydration worsens jaundice, and it is therefore important to optimise feeding.
“Breastfeeding 2-3 hourly, but not been latching on properly, and not lasting for more than a few minutes.”
Has the baby passed any wet and dirty nappies? If so, how many?
Wet nappies serve as marker of hydration status. Failure to pass meconium can indicate gastrointestinal obstruction.
“There has been one dirty nappy, and two mildly wet nappies.”
Has the baby been drowsy, had any funny movements or felt floppy?
May potentially be a result of dehydration, or if the jaundice levels are very high, may suggest the baby is at risk of kernicterus.
“No.”
How has Jamie’s breathing been? Has he had any temperatures?
Jaundice may relate to an infection. Although remember there may be an infection without a temperature.
“Breathing is fine, and there have been no temperatures.”
Has Jamie had any other problems between delivery and now, other than the feeding and jaundice?
Screening for other problems that may be relevant.
“No.”
Poor feeding may be significantly contributing to this jaundice. Alternatively, the feeding may be poor as the child has an infection which has precipitated the jaundice.
History Taking
For all scenarios: ӹӹ Introduction. ӹӹ Ask a mixture of open and closed questions.
ӹӹ Ensure understanding throughout. ӹӹ Summarise your findings to the parents, and give them an opportunity to raise any other issues.
Sounds like the baby is systemically well.
Obstetric and Birth History Question
Justification
Answer
Evolving thought process
How was the pregnancy? Were there any problems on the scans? What is your blood group?
Antenatal diagnosis may be helpful. Maternal blood group will determine the risk of haemolytic blood disorders (O- is high risk).
“All scans were normal. Blood group is A+.”
No additional concerns identified. There is a potential set up for ABO incompatibility, which may explain the jaundice. Will need to check the baby’s blood group and check a direct antigen test (DAT).
How many weeks pregnant were you when Jamie was delivered? Was it a vaginal delivery, forceps or caesarean? Did he cry straight away or need help?
Premature babies are more vulnerable to jaundice. Forceps may result in cephalohaematoma that can give rise to jaundice. A baby requiring resuscitation may have an infection.
“Jamie was born at 36 weeks, by normal vaginal delivery, and cried straight away.”
Prematurity may be contributing to the jaundice.
What was the birth weight?
Low birth weight infants are more likely to become jaundiced and to have difficultly feeding.
“2300g.”
Low birth weight may be contributing to the jaundice.
Were any issues identified on the newborn baby check?
General screen for problems. The presence of jaundice is usually checked for on the baby check.
“No.”
No additional concerns.
How long was it between the rupture of your membranes and delivery? Did you have any temperatures during labour? Were you given antibiotics during labour? Do you know if you’ve had a positive test for the bug Group B strep?
These are all risk factors for infection, which is particularly important in day 1 jaundice.
“Delivered within two hours of membrane rupture. One temperature of 38.2°C during labour, given one dose of antibiotics, just before delivery, and mum has never had a positive group B strep test.”
Getting more concerned about possible infection, as we have day 1 jaundice, prematurity, and a temperature during labour (with inadequate antibiotics). As a result, this baby will need antibiotics.
Continued
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History Taking
Question
Justification
Answer
Evolving thought process
Do you have any other children? Any health problems with them now or in the past?
Group B strep infection in a previous child increases risk in subsequent births.
“First child.”
Being a first time Mum may contribute to difficulty establishing feeding. Mum also likely to be particularly sensitive.
Do you (or dad) have any health problems that you are aware of?
Many causes of jaundice are hereditary, e.g. hereditary spherocytosis or alpha-1-antitrypsin deficiency.
“Dad has alpha-1-antitrypsin deficiency.”
Alpha-1-antitrypsin deficiency is a cause of jaundice. It may be helpful to screen the child for this, particularly if the jaundice is prolonged or if mum is a carrier as well.
Drug History Question
Justification
Answer
Evolving thought process
Is Jamie on any medication? Has he had Vitamin K? Does he have any allergies?
Unlikely to be on any medications, but useful to know as may have been started on antibiotics.
“No medications, no allergies, and has had one vitamin K injection.”
No new concerns identified.
Question
Justification
Answer
Evolving thought process
Who is currently at home? Does Jamie have a social worker? Is dad around?
Find out if there are any social concerns or if wider support is needed.
“I live with Jamie’s dad, and we are in a stable relationship. No social worker involved.”
No social concerns at present.
Social History
Developmental history and immunisation history are not relevant in a baby at 2-days-old, as they are yet to meet the first milestone for either. It is appropriate to check the newborn examination record to see if any problems were identified.
Concluding Question
Justification
Answer
Evolving thought process
Is there anything that I’ve missed, or anything that you are particularly concerned about? Do you have any questions for me at the moment?
May identify useful information that has been missed. Will also help framing communication with the mother.
“I’m really keen to breastfeed, but I am afraid it’s failing. I am also worried that we don’t know what has caused the jaundice.”
Worth asking a breastfeeding midwife to give support. Need to take time to explain possible causes of jaundice to mum.
PRESENTING YOUR FINDINGS
Jamie is a 36 week 2300 g baby on day 2 of life, who has presented with jaundice beginning on day 1. Jamie was born by normal vaginal delivery and required no resuscitation. Mum had one temperature during labour, with inadequate antibiotic cover. Antenatal scans are normal, and mum has blood type A+. This is mum’s first baby and she is still establishing breastfeeding. Jamie has passed two mildly wet nappies today. Dad has alpha-1-antitrypsin deficiency. There are no social concerns. My differential diagnosis is jaundice secondary to infection, or blood group incompatibility, exacerbated by prematurity and poor feeding. I am going to cannulate Jamie, and then check serum bilirubin (SBR), full blood count (FBC), urea and electrolytes (U&Es), C-reactive protein (CRP) and blood cultures. I will start benzylpenicillin and gentamicin, and check the baby’s blood group and DAT. I will make a decision about phototherapy when the SBR comes back, and if the CRP is high, I will consider a lumbar puncture. Blood sugars need to be monitored, and if breastfeeding cannot be established after further support, we will need start top-up feeds, either formula or expressed breast milk, at 90mL/kg/day.
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CASE 2: A 6-WEEK-OLD BABY WITH A TEMPERATURE
CASE 2
A 6-week-old baby with a temperature You are asked to see Fred, a 6-week-old baby in the ED, who had a temperature at home of 39°C. He is otherwise well, but mum wants him checked just to be safe. Please take a history from his mother, with a view to making a diagnosis.
Mother (actress) Briefing Jamie is your second baby, and you delivered six weeks ago at King’s Hospital. The baby was born at 38 weeks, with a birth weight of 4 kg, by an elective C-section (due to breech presentation) and cried at delivery. You had one raised temperature of 39°C during labour and were given two doses of antibiotics. The pregnancy was fine from your perspective: all your scans and blood tests were normal.
Assessment For all scenarios: ӹӹ Introduction. ӹӹ Ask a mixture of open and closed questions. ӹӹ Ensure understanding throughout. ӹӹ Summarise your findings to the parents, and give them an opportunity to raise any other issues.
History Taking
Doctor Briefing
Fred is feeding well, every three hours on the breast, for 15 minutes a time. He is putting on a good amount of weight, and is tracking the 50th centile on the growth chart. You are in a stable relationship with your husband and are expecting to be sent home. Your other child is completely healthy and has never had any medical issues. You are concerned because you don’t know what is causing the temperature, and want reassurance that it’s probably nothing. If specifically asked, you tested positive on a swab for group B streptococcus during the pregnancy.
History of Presenting Complaint (Temperature) Question
Justification
Answer
Evolving thought process
When did you first notice the temperature? How high has it been, and for how long? Have you given anything to bring the temperature down?
Establishing the history of the temperatures gives clues as to the likelihood of there being an infection, and how many days it may have been present.
“He has only had one temperature this morning of 39°C. We didn’t give anything for it.”
Since the temperature is particularly high, it is more likely to be related to pathology. As it has only just manifested, a clinical focus may not be obvious yet.
How has Fred been in himself? Is he more sleepy or tired than usual? Has he had any floppy episodes, or funny movements?
Looking for general signs of being unwell that would be consistent with a clinically apparent infection
“He is completely well in himself.”
Reassuring, although it is still concerning that he has an unexplained temperature.
How are you feeding Fred, how often, and how is that going? If breastfeeding, is Fred latching on to the breast? How long is each feed?
Septic babies are less likely to feed well.
“Breastfeeding is going well, Fred is latching on to the breast every 3 hours for 15 minutes.”
Has Fred passed any wet and dirty nappies? If so, how many?
Poor feeding can lead to dehydration, and a poor urine output is a marker of septic shock.
“There has been one dirty nappy and eight wet nappies in the last 24 hours, which is the same as normal.”
How has Fred’s breathing been? Any cough, and if so is anything being coughed up? Any rashes or swellings anywhere? Any vomiting or diarrhoea? Any abnormal movements that could be fits?
Trying to localise the site of any possible infection.
“He has been completely well.”
No specific focus of infection is identified.
Has Jamie had any other problems between delivery and now, other than this temperature?
Screening for other relevant problems.
“No.”
Being reassured that Fred has generally been a well baby.
Being reassured that this child is likely to be clinically well at present.
Obstetric and Birth History Question
Justification
Answer
Evolving thought process
How was the pregnancy? Were there any problems on the scans?
Antenatal diagnosis may be helpful; for example, antenatally diagnosed pelviureteric junction (PUJ) obstruction increases the risk of urinary tract infections (UTIs).
“All scans were normal”
No additional concerns identified.
Continued
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History Taking
Question
Justification
Answer
Evolving thought process
How many weeks pregnant were you when Jamie was delivered? Was it a vaginal delivery, forceps or caesarean? Did he cry straight away or need help?
Premature babies are more vulnerable to infection. A baby requiring resuscitation may have had an occult infection.
“Jamie was born at 38 weeks, by elective C Section for breech, and cried straight away.”
No additional concerns identified.
What was the birth weight?
Low birth weight infants are more likely to have infections.
“4kg.”
No additional concerns identified.
Were any issues identified on the newborn baby check?
General screen for problems. The presence of jaundice is usually checked for on the baby check.
“No.”
No additional concerns identified.
How long was it between the rupture of your membranes and delivery? Did you have any temperatures during labour? Were you given antibiotics during labour? Do you know if you’ve had a positive test for the bug Group B strep?
These are all risk factors for infection.
“Fred delivered within 4 hours of membranes rupturing. There was one temperature of 39°C during labour, but I was given two doses of antibiotics. I tested positive for Group B streptococcus during pregnancy.”
Evidence for possible infection increasing, with maternal temperature and group B streptococcus. Adequate antibiotic cover was given. Vertically transmitted infections can potentially present late.
Past Medical/Surgical History Question
Justification
Answer
Evolving thought process
Has Fred ever had any operations? Have you been to hospital with him since birth? Does he have any known medical conditions?
May all be linked to the temperature, e.g. recent surgery may mean a post-operative intraabdominal collection.
“Fred has had no previous operations, no hospital attendances and no medical diagnoses.”
No new concerns identified.
Drug History Question
Justification
Answer
Evolving thought process
Is Fred on any medication? Does he have any allergies?
Unlikely to be on any medications, but useful to know as may have been started on antibiotics, and drug interactions should be considered. Drugs may also be a clue to underlying diseases. Allergies are unlikely in a six week old breast fed baby.
“No medications, no allergies.”
No new concerns identified.
Developmental History Question
Justification
Answer
Evolving thought process
Is Fred smiling? Can he fix his eyes on people/things he is interested in and follow them as they move?
At six weeks, a baby would generally be expected to fix and follow, and to social smile.
“He can do both.”
No developmental concerns at present.
Family History Question
Justification
Answer
Evolving thought process
Anyone currently ill in the family? Any recent Infection can be highly contagious. travel? Any unusual foods eaten lately?
“Grandma and Granddad both currently have a cold.”
Possible source of infection from grandparents.
Do you have any other children? Any health problems with them now or in the past? Do you (or dad) have any health problems that you are aware of?
“No health problems with the other children or in the family.”
No additional concerns identified.
Group B strep infection in a previous child makes it more likely this child will have it. There may be a history of Sudden Infant Death Syndrome (SIDS).
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CASE 3: A 13-YEAR-OLD BOY PRESENTING WITH A WHEEZY EPISODE
Social History Justification
Answer
Evolving thought process
Who is currently at home? Does Fred have a social worker? Is dad around? Does anyone smoke at home?
Find out if there are any social concerns, or if wider support is needed. Smoking increases risk of infection in the child.
“My husband and I live together, and have been happily married for several years. We are both non-smokers, and there is no social worker.”
No social concerns at present.
Immunisation history is not relevant in a six-week-old baby, as the first immunisations are at eight-weeks-old.
Concluding Question
Justification
Answer
Evolving thought process
Is there anything that I’ve missed, or anything that you are particularly concerned about? Do you have any questions for me at the moment?
May identify useful information that has been missed. Will also help framing communication with the mother.
“I am really keen to go home.”
Needs a careful and sensitive discussion about a management plan.
PRESENTING YOUR FINDINGS
Fred is term baby who presents at six weeks of age very well in himself, but with a one-off temperature at home of 39°C. There is no difficulty in breathing, no seizures, no rashes, no vomiting and no diarrhoea. Fred is feeding well at the breast, with a normal number of wet nappies. Jamie was born by an elective C-section for breech, required no resuscitation and went home with mum after a normal newborn baby check. Mum had one raised temperature during labour, and a background of Group B streptococcus, with two doses of antibiotics given during labour. Antenatal scans are normal. Fred is growing well, tracking the 50th centile. Grandma and granddad both currently have an upper respiratory tract infection (URTI), but there is no family history of note. This is mum’s second child, and the other child is well. There are no social concerns. I am concerned about possible infection, particularly considering the maternal background, although there is no clear focus for infection identified. After doing a full examination, I will cannulate the baby, and check the FBC, U&Es, CRP and blood cultures. I will get a clean catch urine sample, and a chest X-ray if there are any signs of respiratory disease on examination. A nasopharyngeal aspirate will be helpful if the baby has signs of possible bronchiolitis. In view of the history of Group B streptococcus and fever, I would also perform a lumbar puncture. I will start broad spectrum antibiotics. Fred is currently feeding well, so I’d be happy for him to continue breastfeeding at present.
History Taking
Question
CASE 3
A 13-year-old boy presenting with a wheezy episode Doctor Briefing You are asked to see a boy, Steven, 13-years-old, in the ED who has started wheezing at home. Please take a history from his mother, with a view to making a diagnosis.
Mother (actress) Briefing Steven is your only child, and you are worried about him because he is wheezing and has difficulty breathing. He is breathing fast and having to pause in the middle of sentences to catch his breath. His shortness of breath has come on gradually. He does not have a fever but he has had a bit of a cold for the last few days. He is off his food, but drinking about two-thirds of normal. Steven occasionally has had wheezy episodes in physical education lessons, but he usually gets better quickly. Steven is otherwise well, is not on any medication and has never attended hospital before. He was born at 35 weeks by a normal vaginal delivery. He is up-to-date with his immunisations, developing normally, growing well and performing well academically. You are a single mother, but are coping well. Steven stays with Dad every other weekend. You don’t smoke. Dad and the paternal granddad both have eczema. You are concerned because this is the worst you’ve ever seen Steven, and he can’t talk to you properly. If specifically asked, you are also worried that his dad smokes and that this might contribute to the breathing problems.
Assessment For all scenarios: ӹӹ Introduction. ӹӹ Ask a mixture of open and closed questions. ӹӹ Ensure understanding throughout. ӹӹ Summarise your findings to the parents, and give them an opportunity to raise any other issues.
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History Taking
History of Presenting Complaint (Wheeze) Question
Justification
Answer
Evolving thought process
When did the wheeze start? What was he doing at the time? Was it sudden/gradual? How has it progressed since it began?
Sudden onset wheeze could imply foreign body aspiration or anaphylaxis.
“The wheeze came on gradually whilst walking home, and is gradually getting worse and worse.”
Unlikely to be foreign body aspiration, and less likely to be anaphylaxis, although both can present atypically.
How has his breathing been otherwise? Does he use more effort than normal to breathe? Has it affected his speech?
Children can be happy wheezers or in marked respiratory distress, and how their breathing affects them will influence treatment.
“Steven is breathing much faster than normal and struggling to keep up. He is unable to complete sentences.”
There is significant respiratory distress, which will require urgent treatment.
Has he had a cough, and if so, is he coughing anything up? Any fevers? Has he had a runny nose, a sore throat or any ear pain or discharge?
A respiratory tract infection is a common cause of an asthma exacerbation, or viral-induced wheeze.
“No fever. He has had a cold for the last few days, with a cough and a snotty nose.”
This appears like a viral infection has triggered a wheezy episode. It may or may not be asthma.
Any vomiting or diarrhoea? Any headache, neck stiffness, fits? Any pain, difficulty peeing, or change in the colour or smell of the urine? Any rashes or joint swelling?
Given the significant respiratory distress, it is worth enquiring about other possible sources of infection.
“None.”
Likely to be an isolated respiratory issue.
Is he eating and drinking normally or less than normal? Is he peeing and pooing the same amount as usual?
Gives a measure of how unwell the child is, and any dehydration.
“He is completely off his food, but has had five cups of water today.”
He is remaining sufficiently hydrated in this episode.
How was Steven before this episode? Has he ever wheezed before? Does he ever wheeze or cough while exercising, or at night? Has he ever had any tests for breathing issues before (X-ray, peak flow, lung function tests)?
Gives a measure of any underlying lung disease.
“He wheezes sometimes when playing sports. He has never had any tests looking at his lungs.”
Makes underlying lung disease, i.e. asthma, more likely.
Past Medical/Surgical and Birth History Question
Justification
Answer
Evolving thought process
Was Steven born on time or early? Were there any complications at birth?
Premature babies are more likely to develop asthma.
“He was born at 35 weeks, by a normal vaginal delivery, with no complications.”
Prematurity might predispose to asthma.
Does Steven suffer from asthma, eczema or hay fever? Any other medical problems? Has he ever been admitted overnight to hospital or had to miss school with illness?
Atopic diseases are linked “He is otherwise completely well.” together. Previous admissions/ medical disorders may explain current symptoms, e.g. heart disease or gastro-oesophageal reflux disease.
No other concerns.
Question
Justification
Answer
Evolving thought process
Is Steven on any medication? Have you given him anything for this episode? Does he have any allergies?
Medications may be a clue to an underlying diagnosis. Even if he doesn’t have any medications, parents may give a child another sibling’s or a parent’s medication, e.g. salbutamol inhaler.
“No medications, no allergies.”
No new concerns identified.
Drug History
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CASE 3: A 13-YEAR-OLD BOY PRESENTING WITH A WHEEZY EPISODE
Growth and Developmental History
Concluding
Justification
Answer
Evolving thought process
Question
Justification
Answer
Evolving thought process
Are there any worries or concerns about Steven’s development compared to his peers at school? Is he performing well in assessments?
There is no specific concern about his development in the initial history, but it is useful to get a broad idea of his current status.
“No concerns at all.”
No new concerns identified.
Needs a careful and sensitive discussion about smoking cessation for the father.
Chronic disease can be associated with faltering growth.
“Yes, he is growing well (75th centile when plotted).”
Less likely to have a severe, chronic underlying disease.
May identify useful information that has been missed. Will also help framing communication with the mother.
“Mum is concerned this may be related to Dad’s smoking.”
Is Steven growing as expected for him?
Is there anything that I’ve missed, or anything that you are particularly concerned about? Do you have any questions for me at the moment?
Immunisation History Question
Justification
Answer
Evolving thought process
Has Stephen had all of his immunisations?
Unimmunised children are at risk of childhood infections.
“Yes.”
No new concerns identified.
Family History Question
Justification
Answer
Evolving thought process
Is anyone currently ill in the family? Any recent travel?
Infection can be highly contagious, and may explain the presenting illness.
“None.”
No new concerns identified.
Do you (or dad, or grandparents) have any health problems that you are aware of?
Atopic diseases run in families.
“Dad and granddad on the father’s side have eczema.”
There is a family history of atopic disease, making asthma more likely.
Social History Question
Justification
Answer
Evolving thought process
Who is currently at home, and where does Steven stay? Does Steven have a social worker? Is dad around? Does anyone smoke at any place he stays?
Find out if there are any social concerns, or if wider support is needed. Smoking increases risk of infection in the child.
“I am separated from Steven’s dad. Steven spends every other weekend with dad, who smokes. There is no social worker.”
No social concerns at present, although dad’s smoking may be contributing to this.
History Taking
Question
PRESENTING YOUR FINDINGS
Steven is an unwell 13-year-old boy that has presented with wheeze and difficulty in breathing, on a likely background of asthma. The wheeze came on gradually whilst walking home and is associated with laboured breathing, leading to an inability to complete sentences. He has not taken any medications for his breathing. Over the last few days, he has had URTI symptoms. Oral fluid intake is around two-thirds of normal. Steven was a premature baby (born at 35 weeks gestation), and although he has had a few mild wheezy episodes whilst exercising, he has had no major medical problems up until today. Dad and granddad have eczema. Growth and development appears normal, and immunisations are up-to-date. Steven splits time between mum and dad, who are divorced. Dad is a smoker. The most likely diagnosis is a viral exacerbation of asthma, although other things to consider in a wheezy child are foreign body inhalation, anaphylaxis, heart failure and gastro-oesophageal reflux. As this child appears very unwell, I would want to do a rapid, focused respiratory examination, and after that, begin treatment with oral corticosteroids and back-to-back nebulisers. I will cannulate Steven, get a venous blood gas, and baseline bloods including FBC, CRP and U&Es, knowing also that I might need the cannula for intravenous bronchodilator therapy afterwards. I will also arrange a chest X-ray, and if Steven is compliant, try to get a peak flow reading.
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History Taking
CASE 4
A 5-year-old boy presenting with a swollen leg Doctor Briefing You are asked to see Eric, a 5-year-old boy in the ED, who has presented with a swollen right leg. Please take a history, with a view to making a diagnosis.
Mother (actress) Briefing You (Mary Smith, age 40), are the mother of Eric, aged five. You are no longer with his father, and have had two children with your current partner, who is your husband. Eric stays with his dad every other weekend. You noticed today when you picked up Eric from his dad’s place that his right thigh was swollen, and immediately got concerned that he has been seriously injured. He is looking a little frightened and not moving that leg as much as the other one. He was asleep when you picked him up from dad, but as soon as he woke up, he was crying, and you noticed the swollen leg. There are no other injuries. Eric is otherwise well. He was born on time, by normal vaginal delivery, in good condition. He is meeting all of his developmental milestones, is growing well (75th centile), and is up-to-date with his immunisations. He suffers from no medical conditions, has never attended ED before, is on no medications, has had no previous operations and has no allergies. Eric
has just started attending infant school at Springfield High School, Jefferson Street, London, SE19 5FG. He is doing well, performing similarly to his peers. Eric lives at home with you, his step-dad (Benjamin Smith, age 45), and his two half-siblings (Sarah Smith, aged 1, and Suzanna Smith, aged six weeks), at the address 27 Joseph Coldcastle Close, New Hampton, London, SE12 4AX. He spends every other weekend at his dad’s house: 12 Humberback Street, New Story, London, WE13 5BE. Dad’s name is Fredrick Divan, aged 50, and other residents in that house include two step-brothers of Fredrick’s: Franklin Divan (aged 14), and Jonathan Divan (aged 16), plus Fredrick’s current partner: Sophia Harrison, (aged 22). You split from his dad due to domestic violence concerns. You are concerned because Eric won’t stop crying and you don’t know what happened to his leg. You immediately blame it on Eric’s dad, whom you feel has a temper that can’t be controlled. You want the pain to be put under control, and you want the police involved now.
Assessment For all scenarios: ӹӹ Introduction. ӹӹ Ask a mixture of open and closed questions. ӹӹ Ensure understanding throughout. ӹӹ Summarise your findings to the parents, and give them an opportunity to raise any other issues.
History of Presenting Complaint (Injury) Question
Justification
Answer
Evolving thought process
Could you tell me, in chronological order, when you noticed a problem with Eric, and what has happened since?
Gives a time course of events.
“I dropped Eric off at his dad’s house two days ago, and picked him up today in the afternoon. His dad had said there had been no problems, and Eric seemed peacefully asleep when I picked him up. An hour later, when Eric woke up, he was screaming in pain, looking a little frightened. I immediately undressed him to try and identify any problem. I noticed that the middle of his right thigh was red and swollen, and that he wasn’t moving that leg. I then gave him some paracetamol and rushed him to hospital.”
Eric has a significant injury to his leg, for which we do not have a clear explanation. It may relate to a non-accidental injury or may be an underlying medical problem.
Did anyone witness any event? And when do you think the swelling might have started?
Gives more details about time course.
“He was injured at his dad’s house, but I’m sure he is covering it up!”
Concern about an injury under dad’s care, but also know that it could have happened after mum took over, or could be something medical instead, e.g. cellulitis.
What does the leg look like? What is Eric able to do with the leg compared to normal? Any fevers?
Injury could be a possible fracture, or possible infection.
“The leg looks slightly bent from the middle, and Eric is not moving it. No temperature.”
Fracture sounds much more likely than other possibilities, e.g. cellulitis.
Are there any other possible injuries elsewhere?
Multiple injuries could imply underlying bone pathology or a nastier non-accidental injury (NAI). There also may be bruises, bite marks, etc., from other NAIs.
“None.”
Reassured that this may be one isolated injury, but still will look very closely for other injuries.
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CASE 4: A 5-YEAR-OLD BOY PRESENTING WITH A SWOLLEN LEG
Past Medical/Surgical and Birth History Justification
Answer
Evolving thought process
Was Eric born on time or early? Were there any complications at birth? Does Eric suffer from any medical problems? Has he ever been admitted overnight to hospital, or had to miss school with illness?
Bone disorders, e.g. osteogenesis imperfecta, predispose to fractures. Recurrent fractures or injuries may give a pattern to non-accidental injury or alternatively imply a possible underling medical disorder.
“He was born on time with no complications. He is otherwise completely well, and has never been admitted to hospital.”
No other concerns identified.
Drug History Question
Justification
Answer
Evolving thought process
Is Eric on any medication? Have you given him anything for this episode? Does he have any allergies?
Medications may be a clue to underlying diagnosis. May also give clues as to possible overdoses in certain presentations.
“No medications, no allergies.”
No new concerns identified.
History Taking
Question
Growth and Developmental History Question
Justification
Answer
Evolving thought process
Are there any worries or concerns about Eric’s development compared to his peers at school? Can he jump? Does he draw, and if so what kind of things can he draw? How many words can he use? What cutlery does he use to eat with? Is he performing well in assessments?
If Eric is not using one leg, this may relate to a gross motor problem. Suspicion of NAI also raises the possibility of neglect: if this is the case, there may be associated developmental delay.
“Not doing much today. Normally can jump, draws in scribbles, uses about 60 words, and feeds with a fork and spoon (all age appropriate).”
No new concerns identified.
Is Eric growing as expected for him?
Chronic disease can be associated “Yes, he is tracking the 75th with faltering growth. centile”
Less likely to have a severe, chronic underlying disease.
Immunisation History Question
Justification
Answer
Evolving thought process
Has Eric had all of his immunisations?
Unimmunised children are at risk of childhood or invasive infections, and with a suspicion of neglect, immunisations might have been missed.
“Yes.”
No new concerns identified.
Family History Question
Justification
Answer
Evolving thought process
Do you (or dad or any grandparents) have any health problems that you are aware of?
Bone formation disorders, e.g. osteogenesis imperfecta, have a familial inheritance (although these are rare).
“None.”
No new concern identified.
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History Taking
Social History Question
Justification
Answer
Evolving thought process
Tell us about your family. Please could you tell us all the places where Eric stays, including the name and age of all residents at each site. What school does he attend? Is there anyone else that regularly sees him? Do you have a social worker, and if so why did they get involved?
It is very possible that this was a case of NAI, and so it needs to be investigated. To do this, all contacts need to be identified. Try to take as much detail as possible including name, date of birth, and addresses.
“Eric lives at home with me (Mary Smith, age 40), his step dad (Benjamin Smith, age 45), and his two half siblings (Sarah Smith, one-year old, and Suzanna Smith, six-weeks-old), at the address 27 Joseph Coldcastle Close, New Hampton, London, SE12 4AX). He spends every other weekend at his Dad’s house: 12 Humberback Street, New Story, London, WE13 5BE). Dad’s name is Fredrick Divan, aged 50, and other residents in that house include two step brothers of Eric’s: Franklin Divan (aged 14), and Jonathan Divan (aged 16), plus Fredrick’s current partner: (Sophia Harrison, aged 22). Eric attends Springfield High School, Jefferson Street, London, SE19 5FG. A social worker is involved with us (Denise Lewise), who first got involved when there were domestic violence concerns.”
There is an identified social worker who may have more information on this case. There are many possible contacts that may have been involved in a NAI.
Concluding Question
Justification
Answer
Evolving thought process
Is there anything that I’ve missed, or anything that you are particularly concerned about? Do you have any questions for me at the moment?
This may identify useful information that has been missed. It will also help framing communication with the mother.
“I am very concerned this was caused by his Dad, and I want the police involved immediately.”
Needs a careful and sensitive discussion about the next steps of management.
PRESENTING YOUR FINDINGS
Eric is a 5-year-old boy presenting with a likely fractured shaft of the right femur, which is a possible non-accidental injury. Mum and dad are no longer together, but Eric visits dad every other weekend. I have taken a detailed history of where Eric stays and who is around him. Mum picked up Eric from dad today, and although dad said everything was okay, one hour later, Eric woke up screaming in pain and was noted to have a red, swollen, deformed right upper leg. Eric was not using the right leg, and despite taking paracetamol, he remained in significant pain. No other injuries were reported, and Mum came straight to ED after identifying the problem. Eric is otherwise medically well, with no previous hospital attendances, all immunisations up-to-date, growing well and all developmental milestones being met. A social worker is already involved with this family; her involvement started because of domestic violence concerns. The most likely diagnosis is a fractured right shaft of the femur, but there is no adequate explanation at present as to how this happened. I would like to give adequate analgesia, order both lateral and anteroposterior (AP) radiographs of the right femur and contact the orthopaedic surgeons for review. I will complete a detailed examination, including a body map of any other injuries. Once the diagnosis is confirmed, social services and the police need to be contacted, and as no adequate explanation can be found for the injury, a work up for NAI should be completed, with consideration of a skeletal survey, ophthalmology review, CT head and blood tests, including clotting and bone profile.
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CASE 5: A 14-YEAR-OLD GIRL PRESENTING WITH ACUTE ABDOMINAL PAIN
CASE 5
Doctor Briefing Samantha is a 14-year-old girl who independently presents to the ED with abdominal pain. Please take a thorough history and discuss your findings with the examiner.
Patient (actress) Briefing You are Samantha, a 14-year-old girl. You have come to ED predominantly because of severe abdominal pain. It started two days ago, and was all across the abdomen, coming on gradually. The pain was sharp, and has now shifted to your lower abdomen on the right hand side. During this time, you have vomited three times, but you have not experienced any diarrhoea. You are off your food completely, and have hardly drunk anything. The pain is there constantly, and is worsened by any movement. It is the worst pain you have ever had, and today it is getting progressively worse. You have no other symptoms, although your period is a few days late. You opened your bowels
History Taking
A 14-year-old girl presenting with acute abdominal pain
normally today, and are peeing normally, except a bit less than before. You have asthma, for which you take a salbutamol inhaler when needed. You have no allergies, and all your immunisations are up-to-date. There are no concerns regarding growth (stably on 75th centile) and development. You live at home with mum and dad, but came straight from school to ED because the pain was so bad. Your mum has said she’ll meet you in the ED, and is on her way. You do not have a social worker. You are in a relationship with a 17-year-old boy, and are sexually active. You are concerned because you think you are pregnant. You don’t want your mum to know that you’ve been having sex with your older boyfriend.
Assessment For all scenarios: ӹӹ Introduction. ӹӹ Ask a mixture of open and closed questions. ӹӹ Ensure understanding throughout. ӹӹ Summarise your findings to the parents, and give them an opportunity to raise any other issues.
History of Presenting Complaint (abdominal pain) Question
Justification
Answer
Evolving thought process
When did you first notice you were ill, and what’s happened since?
Gives a time course of events.
“The pain started two days ago. It was really bad and wouldn’t go away. It has just been getting worse and worse. It’s now the worst pain I could possibly imagine.”
This does not sound like it is getting better on its own, and could be serious.
Where is the pain, and has it spread anywhere? Did it come on quickly? What type of pain is it? Does anything make it better or worse?
May hint at diagnosis.
“It started everywhere, but now it is mainly here (points to right iliac fossa). It’s better when I’m really really still.”
Sounds like classical history for appendicitis, but may also be other things like ectopic pregnancy, urinary tract infection, torsion of ovarian cyst. Worse on movement hints at peritonitis.
Is your tummy more swollen than usual? When did you last open your bowels?
Need to consider intestinal obstruction.
“I last opened my bowels today, and my tummy looks the same as always.”
Obstruction unlikely.
Any temperatures? Have you thrown up, or had diarrhoea? What about any problems peeing? Any sick relatives/ recent travel/recent changes in diet?
Key associated symptoms may help clinch diagnosis, e.g. vomiting, diarrhoea, abdominal pain in gastroenteritis.
“I’ve thrown up three times today. My temperature is ok, and no one is sick around me. I haven’t been on holiday in forever, and I’m eating what I always eat.”
Vomiting may be a sign of infection, but given lack of other factors, is also consistent with appendicitis.
How is your eating and drinking today, compared to normal? Are you peeing a normal amount or less than usual?
General marker of being unwell, “I’ve lost my appetite, and have only plus may hint at hydration had a few sips of water today. I’m status. peeing less than usual.”
Are you currently getting periods? Have you ever had sex? When was your last period?
Need to consider sexually transmitted diseases, and ectopic pregnancy. If the parents were present, it might be useful to speak to the child on their own aswell.
May be markedly unwell, and at risk of dehydration.
“I get periods, and the last one was Concerned about ectopic pregnancy three days late. I do have sex; I particularly, given the severity of have a boyfriend.” the pain. May be issues concerning child protection with the relationship. Continued
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History Taking
Question
Justification
Answer
Evolving thought process
Are you feeling unwell in any other way? For example, difficulty breathing, cough, headache, fits, drowsy episodes, pain elsewhere?
Abdominal pain may be part of a systemic pathology. Also may have referred pain in the abdomen e.g. from right lower lobe pneumonia.
“I feel fine otherwise.”
Likely to be something abdominal causing the problem.
Past Medical/Surgical and Birth History Question
Justification
Answer
Evolving thought process
Were you born on time, or early? Were there any complications around birth?
Could explain the abdominal pain. Previous abdominal surgery often results in the appendix being taken out, even if it is not for an appendicectomy.
“I haven’t been told about any problems with me as a baby. I have mild asthma, and take my asthma pump sometimes. I don’t remember ever staying in hospital.”
No other concerns identified.
Do you have any major medical problems? Any previous operations? Have you ever had to stay overnight in hospital, or had to miss school with illness?
Drug History Question
Justification
Answer
Evolving thought process
Are you on any drugs for your health? Have you taken anything for this pain? Do you have any allergies?
Medications may hint at underlying diagnosis. It may also give clues as to possible overdoses in certain presentations. Analgesia so far can also give insight into level of pain.
“I take a blue inhaler for asthma when I feel wheezy- I don’t know the name of it though. I don’t have any allergies. I have not taken any drugs today.”
Will need analgesia quickly, as has not had anything yet.
Growth and Developmental History Question
Justification
Answer
Evolving thought process
What school do you attend? How is school going? Any problems with academic performance?
Unlikely to affect the differential diagnosis, but developmental problems could be identified at this stage and fed into appropriate support systems if needed.
“I am at St Bartholew’s school, and things are going fine.”
No new concerns identified.
Are you growing well in terms of your height and weight? Any recent weight loss?
Chronic disease may result in faltering growth and abdominal pain, e.g. inflammatory bowel disease.
“Yes, I am about average height and weight for my class. I haven’t lost weight recently.”
Less likely to have a severe, chronic underlying disease.
Immunisation History Question
Justification
Answer
Evolving thought process
Have you had all of your immunisation jabs?
Unimmunised children are at risk of rarer infections.
“I don’t know, but probably.”
No new concerns identified.
Family History Question
Justification
Answer
Evolving thought process
Do you (or parents or grandparents) have any health problems that you are aware of?
Certain gastrointestinal disorders, e.g. inflammatory bowel disease, have a familial tendency.
“None.”
No new concern identified.
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CASE 5: A 14-YEAR-OLD GIRL PRESENTING WITH ACUTE ABDOMINAL PAIN
Social History Justification
Answer
Evolving thought process
Where do you stay, and who do you live with? Do you have a partner at the moment? Can you tell me about them? Are mum or dad coming in today? Do you have a social worker?
Important to identify any possible social concerns, and will ideally need to contact parents about any interventions.
“I live with mum and dad, and am an only child. I’m dating a 17-year-old boy, and we love each other very much. I don’t remember ever seeing a social worker. Mum is coming in later.”
Is in a relationship with a significantly older partner. Has presented without parents, although mum is on her way. Maybe some child protection concerns, but need to find out more information.
Concluding Question
Justification
Answer
Evolving thought process
Is there anything that I’ve missed, or anything that you are particularly concerned about? Do you have any questions for me at the moment?
May identify useful information that has been missed. Will also help framing communication with the mother.
“I’m very worried about being pregnant, and do not want mum or dad to know I am having sex or that I have a boyfriend. Please don’t tell them.”
Needs a careful and sensitive discussion about the next steps of management.
History Taking
Question
PRESENTING YOUR FINDINGS
Samantha is a 14-year-old girl who presents on her own to the ED with right iliac fossa pain and vomiting consistent with appendicitis. The pain started two days ago, as a diffuse abdominal pain, but is now a sharp, severe pain localised to the right iliac fossa. It is made much worse on movement. She has also experienced three episodes of vomiting during this time. There is no diarrhoea, and she has recently opened her bowels. She is off her food and is hardly drinking, with a reduced urine output. Additionally, Samantha is currently sexually active with a 17-year-old partner, and her period is three days late. She reports no other symptoms of note. Samantha has a background of mild asthma, treated with PRN salbutamol. She has no allergies, and all her immunisations are up-to-date. There are no concerns regarding her growth, nor regarding her development. She lives at home with mum and dad, and mum is coming in to see us. At this point, the most likely diagnosis is acute appendicitis, but I would like to rule out ectopic pregnancy. Initially, I would like to give adequate analgesia, and contact the general surgeons for review. I will do a detailed examination, particularly looking for signs of peritonism. I will insert a cannula, and take blood for FBC, clotting, U&Es, CRP and group and save. I would also like to dipstick the urine, and do a urine pregnancy test. Samantha may be Gillick Competent, but ideally I would like to speak to her parents and get the family on board with the management plan. There are also possible safeguarding issues that need to be addressed with regards to the older boyfriend, underage sex, and absent parents in ED (although we have been told mum is on her way).
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3.02
EXAMINATION AMY MORAN AND ZESHAN QURESHI
CONTENTS 527 Introduction 527 Medical Devices
527 Central Venous Access 528 Respiratory Support 530 Surgical Conduits 531 Neurosurgical 531 Mobility Aids and Orthoses
533 Cardiology
Examination 533 Starting the Examination 534 Inspection 537 Palpation 538 Auscultation 541 Completing the Examination
542 Developmental Assessment
543 General Advice 543 Starting the Examination 543 Gross Motor Skills 544 Fine Motor and Vision 545 Speech, Language and Hearing 545 Social and Self-care 545 Completing the Examination
552 ENT Examination 552 Starting the Examination 552 Inspection 553 Palpation 553 Special Tests 554 Completing the Examination
555 Examination of the Eye
555 Starting the Examination 556 Inspection 556 Special Tests 556 Strabismus (malalignment of the eyes) 558 Completing the Examination
558 Gastrointestinal Examination
559 Starting the Examination 559 Inspection 561 Palpation 562 Percussion 563 Auscultation 563 Completing the Examination
564 Musculoskeletal Examination
565 Starting the Examination 565 pGALS 568 Inspection 569 Palpation 569 Movement 572 Hypermobility 572 Completing the Examination
573 Examination of a Neck Mass
574 Starting the Examination 574 Inspection 574 Palpation 574 Auscultation 574 Completing the Examination
575 Neurological Examination
576 Starting the Examination 576 Examination of Upper Limbs 580 Examination of Legs 585 Examination of Gait 586 Cranial Nerve Examination 591 Cerebellar Examination 592 Examination of the Head
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MEDICAL DEVICES
CONTENTS
595 Respiratory
Examination 596 Starting the Examination 596 Inspection 597 Palpation 597 Percussion 598 Auscultation 598 Completing the Examination
599 Thyroid
Examination 599 Starting the Examination 599 Inspection and Palpation 601 Completing the Examination
602 References and
Further Reading
MEDICAL DEVICES INTRODUCTION
P
aediatric examinations can be fun and are a chance to make your patient smile and laugh. They are a balance between systematic and opportunistic examination. Although some children can be unwilling or too upset to be examined, using play techniques can help win over any tantrum (Figure 1-4). For some children, this will be their first encounter with a doctor so keeping the interaction friendly helps to minimise fear. With older children, chat to them, find out their likes and dislikes and explain to them what you are going to do before you do it, at each stage. Wherever possible, remain at the same height as the child to reduce intimidation. Practice is essential, particularly before a clinical exam. Use every opportunity
Children will often have medical devices in situ. A good knowledge of it may identify relevant past medical history, as well as facilitate ongoing management (e.g. knowing where blood samples might be taken from).
Central Venous Access ӹӹ Portacath®. This is an implanted port that sits under the skin connected to a central venous catheter (Figure 5). The port is accessed with a specific needle (Figure 6). Medication can be given via the port and blood can be taken from it. It is seen most commonly in patients undergoing long-term intravenous treatments, such as chemotherapy, regular blood transfusions (e.g. sickle cell disease, thalassaemia) or regular intravenous antibiotics (e.g. cystic fibrosis). The
FIGURE 1
EXAMINATION
593 The Child in the Wheelchair 594 Completing the Examination
to examine lots of patients, including siblings of inpatients and young relatives. Practice will make it easier to combine clinical knowledge with examination findings to form a diagnosis – or at least it will draw some broad differentials. The examination chapter is laid out in order of some of the key systems that feature in both acute, outpatient and exam settings. In nearly all circumstances all systems will need to at least briefly be assessed. Certain rules apply to all examinations: ӹӹ Remember to wash hands before seeing a patient. ӹӹ Begin with an introduction to both the parent and the child. ӹӹ Explain what is going to be done before doing it. ӹӹ With younger children, take consent from parents. Tell the child what is going to be done. If the child is asked whether it is ok to examine their chest and they then say “no,” it is then difficult to proceed. Instead say, “now I am going to listen to your chest.” ӹӹ When examining for pain, look at the child’s face to gauge their reaction rather than the part of the body being examined. ӹӹ When finished, thank the child.
A
B Asking a child to blow bubbles to help them relax (A) and for a younger child, blowing bubbles for them (B).
main complications are infection, port dislodging (the port can sometimes flip too), thrombosis or blockage of the catheter. ӹӹ Hickman line. This is a brand of tunnelled central venous catheters used for long-term treatment such as chemotherapy (Figure 7). Medication may be given via the line and blood can be taken from the line. If not used regularly, the lines should be flushed. As with any central venous device, blockage, dislodgement, thrombosis and infection can occur. The line can also split; it may sometimes be possible to repair small splits depending on their location. Note that a Broviac line is similar to a Hickman line, but the lumen is smaller, meaning it can be used in smaller children and infants. ӹӹ PICC (Peripherally Inserted Central Catheter) line. These may be inserted under local or general
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03
EXAMINATION
anaesthetic, depending on the child’s age (Figure 8). PICC lines tend to be in place for a few weeks, rather than months to years. They need to be flushed regularly to avoid blocking, and the complications are the same as for the other types of central venous devices. Note that Power PICC is a brand of PICC that allows contrast media to be injected for CT scans.
FIGURE 2 Using toys or books to distract a child.
Respiratory Support
FIGURE 3
A
B
Here a teddy bear is used to turn auscultation into a game.
FIGURE 4 Thanking a child for persevering with an examination might keep them in a good mood!
ӹӹ CPAP (Continuous Positive Airway Pressure). This gives positive airway pressure to keep alveoli open and aid recruitment (Figure 9). It can be delivered via nasal prongs or a nasal mask. It can offer pressures of usually 4-8 cm H2O in air or oxygen. ӹӹ Nasal cannula oxygen. This is a simple way to deliver oxygen to a baby (Figure 10). Increasingly, HFNC (High Flow Nasal Cannula Oxygen) is used as a means to generate pressure similar to CPAP. HFNC has the same indications as CPAP and is delivered via nasal cannula. A flow rate is set, usually in a range of 2 L/min to 8 L/ min, again with oxygen or in air. ӹӹ Endotracheal tube. This is used in the severely unwell child/baby (Figure 11). It allows mechanical ventilation via a variety of techniques, such as high frequency oscillation and patient triggered ventilation. ӹӹ Tracheostomy. This is a type of surgical airway (Figure 12). There are a number of indications for its creation, such as vocal cord paralysis and tracheomalacia. A Swedish nose is a small cap attached to the tracheostomy to allow humidification.
Inhalers ӹӹ Inhaler. Inhalers deliver inhaled medication to airways. Different drug companies use different shapes. ›› Turbohalers. Bullet shaped, e.g. Pulmicort® and Bricanyl®: twisted by the patient to activate (Figure 13). ›› Metered dose inhalers. Traditional L-shaped inhalers. These are pressed during inhalation to
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Syringe
Entry point into subclavian vein
EXAMINATION
Tunnelled portion of line under the skin Tip sitting in superior vena cava
Tubing
Portacath external access point
Needle
Skin
Port Catheter
FIGURE 5
FIGURE 6
Portacath®. The port is visible by a small raised area, usually under an arm, the lateral chest wall or on the anterior chest wall.
Accessing the port of the Portacath®.
Entry point into subclavian vein Tunnelled portion of line under the skin Entry point at skin surface Tip sitting in superior vena cava
Tip sitting in superior vena cava FIGURE 7 Hickman line. It usually exits the lateral or anterior chest wall. There is a cuff beneath the skin to keep the catheter in place. Different numbers of lumens are available depending on the intended use of the line.
Entry point at skin surface
FIGURE 8
PICC line. This is usually inserted via the antecubital fossa, with the tip sitting in the SVC just outside the right atrium.
FIGURE 9
FIGURE 10
CPAP.
Nasal cannulae.
CPAP attachment CPAP secured to hat
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EXAMINATION
FIGURE 11
ET tube
ET tube secured to hat
Endotracheal (ET) tube.
FIGURE 12 A
B
Ventilator circuit attachment
Tracheostomy. Not attached to ventilator (left), attached to ventilator (right).
deliver the drug, and should be used with a spacer (Figure 14). ›› Accuhalers. Round, circular inhalers e.g. Seretide™ or Flixotide™. They have a dose release lever on the side (Figure 15). Different colours help to indicate type of drug: ›› Blue. Bronchodilator, e.g. salbutamol and terbutaline. ›› Brown. Corticosteroid, e.g. beclomethasone. ›› Purple. Combination, e.g. Seretide™ (salmeterol and fluticasone). ›› Green. Atrovent® i.e. ipratropium. ›› Orange. Flixotide™. ›› Red. Combination bronchodilator and corticosteroid, e.g. Symbicort® (formoterol and budesonide). ӹӹ Spacer. This is a plastic chamber that enhances inhaled drug delivery (Figure 16). ӹӹ Acapella®. This is a type of airway clearance used in cystic fibrosis as part of physiotherapy (Figure 17). It looks like a small spacer device. It has a mouthpiece that the child breathes into, generating positive end-expiratory pressure (PEEP) to open airways. ӹӹ Flutter®. This is similar to the above, but looks more like a large whistle (Figure 18).
Surgical Conduits ӹӹ Antegrade Continence Enema (ACE). This uses the appendix to create a passage from the colon that opens out onto the anterior abdominal wall as a small stoma. Catheters can be inserted through this to empty faeces from the colon. It is performed for example in spina bifida patients. ӹӹ Mitrofanoff. This uses the appendix to create a conduit from the bladder into a small stoma on the external abdominal wall for neuropathic bladders, such as in spina bifida. A catheter can then be passed directly through this new opening into the bladder to empty it.
Canister
FIGURES 13 and 14
Rotate anticlockwise then clockwise to load dose
Cap
Turbohaler (left) and Metered dosed inhaler (right).
Cap
Mouth piece
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MEDICAL DEVICES
FIGURE 15 Number of doses remaining
Accuhaler.
FIGURE 16 Spacer and facemask. Facemask added to spacer
Mouth piece
EXAMINATION
Pull back to load dose
ӹӹ Gastrostomy. This is a surgical opening into the stomach from the external abdominal wall (Figure 19). It is used for feeding or medication. It may also be referred to as PEG which refers to the operative method used – Percutaneous Endoscopic Gastrostomy. ӹӹ Jejunostomy. This is a surgical opening into the jejunum. It is used in patients for whom a gastrostomy is unsuitable or still results in significant gastro-oesophageal reflux symptoms. PEGs can be extended to create a PEG-J. Note: Distinguishing a jejunostomy from a gastrostomy is often difficult, but they may be labelled. ӹӹ Vesicostomy. This is a surgical opening into the bladder from the lower abdominal wall that allows drainage of urine from the bladder. It is performed in cases of neuropathic bladder or bladder outlet obstruction. ӹӹ Ileostomy/Colostomy. These bags collect faecal matter (Figure 20) out of a surgical opening. The higher up this is coming from in the GI tract, the more liquid the material is. Ileostomies are usually in the right lower quadrant, colostomies in the left lower quadrant. They may be associated with a refeeding fistula at a distal site, through which faecal matter is ‘refed’ into the distal bowel.
Neurosurgical
Inhaler
Spacer
FIGURES 17 and 18 Acapella (top) and Flutter (bottom).
ӹӹ Ventricular reservoirs. This is used to tap CSF for ventriculomegaly/progressive hydrocephalus (Figure 21). ӹӹ Ventriculoperitoneal Shunt (VP Shunt). This drains excess CSF from ventricles (Figure 22). The shunt may become blocked, infected or dislodged. Also look for a scar in the upper quadrant of the abdomen. ӹӹ External Ventricular Drain (EVD). This is unlikely to be encountered in an examination as it is used temporarily to drain excess CSF from ventricles (Figure 23). Antibiotics can be given through it, directly into the CSF. These are usually seen in PICU or NICU at neurosurgical centres on patients with obstructive hydrocephalus.
Mobility Aids and Orthoses ӹӹ Wheelchairs. These come in many variants (Figure 24). Selfpropelled wheelchairs are propelled by the users, meaning the user turns the wheels themselves. Powered wheelchairs are battery operated, and may be driven via a variety of controls including joystick, head control and breath-assisted devices. Additionally, a patient may need back, neck or head supports on the wheelchair. ӹӹ Walkers. These may have no wheels and need to be lifted to move (which offers the greatest stability) or may have two or four wheels, depending on level of mobility (Figure 25). Four-wheeled walkers may also have seats, allowing use for longer distances. ӹӹ Other mobility aids/contracture prevention. These may be in the form of special shoes, insoles or joint splints (Figure 26).
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EXAMINATION
Feeding syringe
Closed feeding port
Clamp opened
Extension tubing FIGURE 19
Ileostomy
Opened feeding port
Gastrostomy.
Liquid faeces in stoma bag
FIGURE 20
Ventricular reservoir at site of anterior fontanelle
Ileostomy.
Manometer
Tubing to connect the drain
CSF in ventricles
FIGURE 21 Ventricular reservoir. Appears as a small mound over the anterior fontanelle.
90 degrees
Scar from shunt insertion
Palpable shunt site
30 degrees
CSF drained FIGURE 23 FIGURE 22
External ventricular drain. Fluid drains from the reservoir into a tube connected to a cylinder and bag that hangs from the bed. The patient is sat at 30 degrees, and the ear is levelled with a marker on the EVD.
VP shunt. This drains CSF from the ventricles, via a series of connecting catheters, into the peritoneal cavity for treatment of hydrocephalus. This shunt can be palpated in the scalp/neck.
Body support Increased padding for back and legs Joystick control
Large wheels to enable propelling using arms
FIGURE 24 Variation in wheelchair design.
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Foot supports
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CARDIOLOGY EXAMINATION
FIGURE 25 B
K-Walker.
EXAMINATION
A
FIGURE 26 Knee-ankle-foot orthosis (A) and wrist splint (B).
CARDIOLOGY EXAMINATION
Summary Checklist Global assessment of child on approach. Inspection. Hands, face, neck, chest, back, abdomen, legs. Palpation. Apex beat, thrills and heaves, hepatomegaly. Auscultation. Heart sounds, murmurs. Bedside tests. Observation chart, plot height/weight, BP, ECG, urine dipstick.
Box 1: Clinical features of heart failure
All unwell children presenting to hospital will require a cardiovascular examination. Particular incidences where a more detailed assessment may be required include: 1 Child with possible pathological murmur of unknown cause. Often children (particularly newborns on their routine checks) are noted to have an incidental murmur. This needs to be assessed to identify its exact nature, and whether it is associated with signs of heart failure or any other indication of cardiovascular compromise. 2 Child presenting with an irregular heart rhythm or episodes of syncope or chest pain. This may be associated with underlying cardiac disease. Do they have valvular heart disease? Do they have heart failure (Box 1)? 3 Child who is acutely unwell. This may be a result of a critical cardiac lesion (e.g. coarctation of the aorta) or myocardial dysfunction secondary to sepsis or pericarditis for instance. Additionally, administration of intravenous fluids and boluses may cause circulatory overload tipping children into heart failure, so this needs to be continually reassessed for during resuscitation. Common patients that are likely to appear in exams are shown in Table 1. Note that any child with a severe uncorrected cardiac defect is also likely to have faltering growth.
• Shortness of breath/tachypnoea.
Starting the Examination
• Poor feeding.
The examination begins by observing the child upon entering the examination room. On introduction to the child/parents, begin with a broad assessment: 1 Does the child look well or unwell? What colour are they? A corrected cyanotic heart lesion will not result in a completely pink child. Are they chatting with no shortness of breath? Are they sweating and uncomfortable? 2 Are there signs of chronic ill health? Do they look underweight? Are there any drains, central lines or long term feeding tubes in place? 3 Are there medications nearby? Is there a portable oxygen cylinder? Are they on a monitor? 4 Are there dysmorphic features? (Table 2)
• Sweating. • Low oxygen saturations. • Crackles/wheeze. • Tachycardia. • Third/fourth heart sounds. • Apex may be displaced. • Hepatomegaly. • Peripheral oedema.
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EXAMINATION
Inspection
Hands
In order to undertake a more focused inspection, the child needs to be exposed. In an older child, they may be wearing a vest and lifting this or moving it to one side when the necessary area is inspected is fine. It is not necessary for bras to be removed. Older children can be examined at a 45° angle, toddlers seated upright on a parent’s lap, and a baby flat on the bed.
ӹӹ Signs of general ill health. This will include wasting of the small muscles in the hands from chronic disease and scars from previous cannulation. Xanthoma is also important, due to the association of hypercholesterolaemia with both hypertension and Alagille syndrome. ӹӹ Capillary refill. (Figure 27). The examiner should push down for five seconds with their index finger on the pulp of the child’s finger and count the number of seconds it takes for normal colour to return: it should take less than two seconds. Check centrally (over the sternum) as well. ӹӹ Clubbing. Clubbing will appear as a result of long standing cyanotic heart disease; therefore it does not appear in children under 6-months-old (Figure 28). ӹӹ Peripheral stigmata of infective endocarditis. ›› Splinter haemorrhages (nails) – also associated with SLE and trauma.
TABLE 1: Common patients that may appear in clinical examinations Diagnosis
Possible clues
Flow/Innocent murmur.
• Well child. • A soft ejection systolic murmur that does not radiate, may alter with respiration and position.
Ventricular septal defect.
• Well child. • A pansystolic murmur heard loudest at lower left sternal edge. • They may have a thrill at lower left sternal edge.
Pulmonary stenosis.
• Well child. • An ejection systolic murmur at upper left sternal edge, radiating to back. • They may have a thrill in the pulmonary region.
Atrial septal defect.
• Fixed splitting of the second heart sound. • An ejection systolic murmur at upper left sternal edge.
Aortic stenosis.
• An ejection systolic murmur at upper right sternal edge, radiating to the carotids. • They may have a thrill in the carotid region.
Patent ductus arteriosus.
• A continuous murmur throughout systole and diastole, loudest at the upper left sternal edge. • Bounding pulses palpable. • Wide pulse pressures.
Surgically corrected Tetralogy of Fallot.
• Possible cyanosis. • An ejection systolic murmur (residual pulmonary stenosis). • They may also have a diastolic murmur (pulmonary regurgitation) or a pansystolic murmur (residual VSD). • Midline sternotomy scar (from corrective surgery). • They may also have a lateral thoracotomy scar if a temporary shunt was placed before definitive surgery. • They may also appear malnourished/small for gestational age.
Surgically corrected transposition of the great arteries.
• An ejection systolic murmur (may have right ventricular outflow obstruction). • A midline sternotomy scar (from corrective surgery) and drain scars.
Surgically/ endovascularly corrected valve lesion.
• A midline sternotomy scar and drain scars OR groin scar. • They may have murmur from restenosis, a leaky valve, or a flow murmur across a new valve. There may also be a loud click if a metallic valve was inserted.
TABLE 2: Common syndromes associated with cardiac disease Syndrome
Appearance
Associated cardiac lesions
Turner syndrome.
• • • •
• Coarctation of aorta. • Bicuspid aortic valve.
Williams syndrome.
• • • • • •
Small upturned nose. Long philtrum. Flat nasal bridge. Wide mouth. Small chin. Small/widely spaced teeth. • May have stellate pattern to the iris.
• Peripheral pulmonary stenosis. • Aortic stenosis (supravalvular).
Trisomy 21.
• • • •
• AVSD. • VSD.
Noonan syndrome.
• Low set/posteriorly rotated ears. • Widely spaced eyes (hypertelorism). • Down slanting palpebral fissures. • Webbed neck. • Ptosis.
• Pulmonary stenosis. • ASD.
Alagille syndrome.
• Broad, prominent forehead. • Deep sunken eyes. • Small pointy chin.
• Peripheral pulmonary stenosis. • Tetralogy of Fallot.
22q11.2 deletion.
• Long, narrow face. • Wideset/almond shaped eyes. • Cleft lip/palate. • Small/low set ears. • Small mouth.
• Tetralogy of Fallot. • VSD.
Webbed neck. Wide-spaced nipples. Shield-shaped chest. Wide carrying angle of the arms.
Single palmar crease. Low set ears. Flat nasal bridge. Upslanting palpebral fissures.
(AVSD = atrioventricular septal defect, VSD = ventricular septal defect, and ASD = atrial septal defect)
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CARDIOLOGY EXAMINATION
FIGURE 27
B
EXAMINATION
A
C
Capillary refill. Compressing the capillary bed (A), resultant pale finger (B), and colour returning (C).
A
A
B
C B
FIGURE 28 Testing for clubbing (in an unclubbed child) by two different methods. A. Inspecting the outstretched fingers. B/C. Asking the child to bend their index fingers, and bring the dorsal surfaces of the distal phalanx together. Demonstrate first. A diamond should be visible between the nail beds.
›› Janeway lesions (red, non-tender lesions on the palms). ›› Osler’s nodes (painful lesions on the distal fingers). ӹӹ Bony abnormalities. Absent thumbs or radius (Holt-Oram syndrome), absent radius (VACTERL syndrome).
FIGURE 29 Palpating the pulses: Radial (A), brachial (B), and carotid (C). Note that whilst measuring the pulse the respiratory rate can also be counted.
Pulses and Blood Pressure ӹӹ Radial pulse. Count the pulse rate for 30 seconds (Figure 29). Is it regular? Does it collapse on lifting the arm up (aortic regurgitation)? This pulse is difficult to feel in babies and therefore is usually not palpated in the examination. It is absent on the left side post a coarctation repair (if a subclavian flap is used). ӹӹ Brachial pulse. Assess pulse volume. The brachial pulse is absent on one side when a coarctation repair has been made using the subclavian flap technique. ӹӹ Blood pressure. The cuff width needs to be approximately two thirds the length of the upper arm, and when placed on it needs to encircle the upper arm completely (Figure 30). Normal ranges for heart rate and blood pressure are shown in Table 3.
C
TABLE 3: Normal values for HR and BP by age Age
Normal HR
Normal systolic BP
0 to 1-year-old
110-160 bpm
80-90 mmHg
1 to 2-years-old
100-150 bpm
70-95 mmHg
2 to 5-years-old
95-140 bpm
85-100 mmHg
5 to 12-years-old
80-120 bpm
90-110 mmHg
>12-years-old
60-100 bpm
90-120 mmHg
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EXAMINATION
FIGURE 30
FIGURE 33
Measuring blood pressure.
A
Face ӹӹ Dysmorphic features. Assess the face shape, the eyes, the ears, the mouth, the tongue, the lips and the chin (Table 2). ӹӹ Conjunctiva. Look for pallor (anaemia) (Figure 31). ӹӹ Xanthelasma. Yellow plaques around the eyelid. May be seen in familial hypercholesterolaemia. ӹӹ Polycythaemic appearance (very red). Associated with cyanotic congenital heart disease. ӹӹ Central cyanosis. Ask the child to stick out their tongue and look underneath it for blue discolouration. Also observe the colour of the gums and lips. ӹӹ Dental caries (decay). Indicates poor dental hygiene. This is a risk factor for infective endocarditis.
Neck ӹӹ Jugular venous pressure. This is only measured in older children (Figure 32).
FIGURE 31 Assessing for anaemia. Ask the child to pull one of their lower lids down themselves and look up to the ceiling. To put the child more at ease, this can be demonstrated on the examiner or on the parent first.
Measure the height of the JVP from above the sternal angle. ӹӹ Carotid pulse. This is only measured in older children. Measure one side only. It is good for getting an appreciation of pulse volume and character (Figure 29).
B Inspecting the chest from the behind (A) and the front (B). Lift up each arm to inspect the axillae.
FIGURE 32
Back
Measuring JVP. Position the child with their back at a 45° angle to the bed, with their head turned away from the side of the neck being inspected.
Chest ӹӹ Harrison’s Sulcus. This is seen in conditions with increased pulmonary blood flow (and also chronic asthma). It appears as a groove along the lower chest where the diaphragm inserts onto ribs. ӹӹ Scars. Inspect the anterior chest, the axilla and the back. Look for drain scars, as well as surgical incision scars (Figure 33-4). ӹӹ Respiratory rate. Tachypnoea is associated with cardiac disease. Measure respiratory rate whilst measuring the pulse.
ӹӹ Oedema. Inspect for sacral oedema (Figure 35). It may be easier to do this when the patient is already sitting forward (e.g. after auscultating the base of the lungs). ӹӹ Basal lung crepitations. Auscultation of the lung bases can be done here to look for crepitation associated with left heart failure (Figure 36).
Abdomen ӹӹ Hepatomegaly. This is associated with right heart failure. It will be smooth rather than hard.
Groin ӹӹ Femoral pulse. An absent/reduced femoral pulse is associated with coarctation of the aorta. Whilst palpating the femoral pulse, palpate the brachial or radial pulse, looking for delay (Figure 37). Radio-femoral delay indicates coarctation of the aorta but can be harder to appreciate in children than in adults. ӹӹ Cardiac catheterisation scars. These will be small scars in the groin overlying the femoral arteries.
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CARDIOLOGY EXAMINATION
1 FIGURE 34
4
Chest drain scar Any major cardiac surgery
3
3 1
Median sternotomy Open cardiac surgery e.g. TGA, Tetraology of Fallot, hypoplastic left heart, complex cardiac defects, valve repair
5
2
EXAMINATION
2
Right thoracotomy BT shunt PA banding Non cardiac surgery e.g. lobectomy
2
4 5
6
Pacemaker scar Left thoracotomy BT shunt Coarctation repair PDA ligation PA banding Non cardiac surgery e.g. lobectomy
6 Cardiac catheterisation scar Surgical scars. TGA (transposition great arteries), BT (Blalock-Taussing), PDA (persistent ductus arteriosus), PA (pulmonary artery). Cardiac catheterisation procedures include valve surgery and PDA closure.
Legs ӹӹ Oedema. This may be present due to heart failure (Figure 38).
FIGURE 35
FIGURE 37
Inspecting for sacral oedema. Push down on a bony prominence such as the posterior superior iliac spines for five seconds and then release.
A
FIGURE 36
B
Auscultation for basal crepitations.
Palpating the femoral pulse. In older children, the femoral pulse should be palpable through the clothes and therefore modesty can be preserved (A). Palpating for brachio-femoral delay in a baby (B).
Palpation ӹӹ Apex beat (Figure 39). The apex beat should be in the midclavicular line, at the 4th-5th intercostal space. It may be in an abnormal position (e.g. displaced in cardiomegaly or on the right side in dextrocardia), or become more forceful (e.g. left ventricular hypertrophy). ӹӹ Ventricular heaves (Figure 40). These indicate ventricular hypertrophy. ӹӹ Thrills. These occur as a result of turbulent blood flow that can be felt as a vibration on the skin surface. Feel in the zones shown in Figure 41. Thrills suggest valvular or cardiac disease. Pulmonary stenosis may be associated with a thrill at the left sternal edge. A VSD may be associated with a thrill at the left lower sternal edge. Note that a thrill in aortic stenosis can often be felt in the suprasternal notch (Figure 42).
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EXAMINATION
FIGURE 38
FIGURE 40
Assessing for pedal oedema. Palpate over the medial malleolus: push down for five seconds and then release.
A
B
Feeling for: Left ventricular heaves. Horizontally place the ulnar side of the palm on the left side of the chest, at the level of the apex, feeling for impulses (A), and right ventricular heaves. Place the ulnar side of the palm vertically against the left sternal border (B).
FIGURE 39 FIGURE 41
Suprasternal notch: aortic stenosis Subclavicular: patent ductus arteriosus
1
Right upper sternal edge (2nd intercostal space): aortic lesions
2 3 4
A
5 6 7
Left upper sternal edge (2nd intercostal space): pulmonary lesions Left lower sternal edge (5th intercostal space): tricuspid lesions/ VSDs Left midclavicular line (5th intercostal space): mitral lesions
Sites to palpate for thrills.
Auscultation B FIGURE 42
C Feeling the apex beat: Feel both sides of the chest to identify which side the heart is on (A), localise the apex beat with one finger (B), and use the other hand to count the number of rib spaces from the sternal notch to the apex (C).
Feeling for a thrill of aortic stenosis with the tips of the fingers in the suprasternal notch.
In a baby that is sleeping, it may be prudent to auscultate first and then go back and carry out the rest of the examination. Smaller children may be anxious about a stethoscope, and various strategies can be employed to put them at ease (Figure 43). Listen in all of the same zones as were palpated for thrills (except the suprasternal notch) (Figure 44). Listen with the bell and the diaphragm in the mitral zone, and just the diaphragm elsewhere. Palpate a central pulse concurrently. Note that children are likely to find palpation of the carotid pulse uncomfortable for longer periods, so it may be preferable to palpate the brachial pulse.
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CARDIOLOGY EXAMINATION FIGURE 43
B
C
D
E
F
EXAMINATION
A
Examine a doll (A), examine the mother (B), and/or examine yourself (C) at the start if the child seems anxious. Then, before putting the stethoscope on the child’s chest, put it in a more comfortable/comical place like their nose (D) or hand (E)! Even when doing the exam, let them be playful (F). There is a wide range of possibilities for entertaining/relaxing a child!
FIGURE 44
A
B
D Auscultating for murmurs at: Mid clavicular line, 5th intercostal space (A), lower left sternal edge, 5th intercostal space (B), upper left sternal edge, 2nd intercostal space (C), and upper right sternal edge, 2nd intercostal space (D).
During auscultation, it is important to assess: 1 What heart sounds are present and whether they are normal or diminished. Fixed splitting of the second heart sound (no variation with respiration) is a feature of ASD and is best heard over ULSE. A loud second heart sound is associated with pulmonary hypertension or increased pulmonary blood flow, e.g. large VSD or PDA.
C
2 Presence of any additional heart sounds: a A third heart sound (S3) heard after the second heart sound can be normal but is also a possible sign of heart failure. It is best heard with the bell over the apex and will sound like “Kentucky,” i.e. 1-2-3. b A fourth heart sound (S4) is rarely heard (it immediately precedes the first heart sound) but is pathological when present. It may indicate heart failure or pulmonary hypertension. This sounds like “Tennessee,” in the order 4-1-2. c A gallop rhythm is heard in tachycardic patients with third and fourth heart sounds (sometimes just a loud third heart sound is heard as the third and fourth heart sounds have “fused” together). d Ejection clicks are associated with pulmonary and aortic stenoses due to the forceful opening of these valves. e A midsystolic click is associated with mitral valve prolapse. It occurs in the middle of systole, as the valve prolapses. f Opening snap. Heard after the second heart sound in mitral stenosis.
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EXAMINATION
3 Whether any murmurs are present. Feel a central pulse simultaneously. If a murmur is present, assess: a Loudness. i How loud is the murmur generally? This should be graded (Table 4). ii In which area of the chest is the murmur loudest? iii Check if the murmur gets louder with: 1 Leaning forward (aortic lesions – Figure 45). Innocent murmurs may change with position (Still’s murmur may be louder and venous hum quieter when the patient is supine). 2 Inspiration (right sided lesions) /expiration (left sided lesions). 3 Turning onto the left side (mitral lesions). 4 Listening with the bell (mitral stenosis). b Character. Harsh sounding murmurs are usually pathological. c Timing. Systolic, diastolic, or continuous. Include length, i.e. ejection systolic vs. pansystolic. d Radiation (Figure 46). Check if it radiates to the: i Back (pulmonary stenosis). ii Axilla (mitral regurgitation). iii Neck (aortic stenosis). TABLE 4: Levine grading scale of cardiac murmurs Grade
Thrill
Auscultation
1.
No.
Only faintly audible with a stethoscope.
2.
No.
Faint, but easily audible on auscultation.
3.
No.
Moderately loud and easily heard on auscultation.
4.
Yes.
Loud but not audible without a stethoscope.
5.
Yes.
Very loud but not audible without a stethoscope.
6.
Yes.
Audible without a stethoscope.
FIGURE 46
A
B
C FIGURE 45
Auscultation for radiation to the: neck (A), axilla (B), and back (C).
Leaning forwards to exaggerate aortic murmurs/ elicit positional variation for an innocent murmur.
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CARDIOLOGY EXAMINATION
Tables 5a-d below show some key cardiac lesions and their distinguishing features. TABLE 5a: Pansystolic murmurs Site
Radiation
Additional features
VSD.
• LLSE.
• None.
• Squatting may make louder. • May have a thrill.
Mitral regurgitation.
• Apex.
• Axilla.
• Louder on expiration/rolling onto left.
Coarctation.
• ULSE/infrascapular.
• Back.
• Absent/reduced femoral pulses. • May also be a continuous murmur.
EXAMINATION
Lesion
(LLSE = left lower sternal edge, ULSE = upper left sternal edge, VSD = Ventricular Septal Defect)
TABLE 5b: Ejection systolic murmurs Lesion
Site
Radiation
Additional features
Pulmonary stenosis.
• ULSE.
• Back.
• Louder on (i) lying flat, and (ii) inspiration.
Aortic stenosis.
• URSE.
• Carotids.
• Louder on (i) sitting forward, and (ii) expiration.
ASD.
• ULSE.
• None.
• Fixed splitting of S2. • May be an associated diastolic tricuspid flow murmur if there is a large left-to-right shunt.
Pulmonary flow murmur.
• LLSE (usually).
• None.
• Soft, short, non-harsh, no thrill.
(LLSE = left lower sternal edge, ULSE = upper left sternal edge, URSE = upper right sternal edge, LLSE = left lower sternal edge, ASD = Atrial Septal Defect)
TABLE 5c: Diastolic murmurs Lesion
Site
Radiation
Additional features
Mitral stenosis.
• Apex.
• Axilla.
• Mid diastolic. • Louder on (i) rolling onto the left, and (ii) expiration.
Aortic regurgitation.
• URSE.
• Also heard at LLSE.
• Early diastolic. • Louder on (i) sitting forward, and (ii) expiration. • Collapsing pulse.
(LLSE = left lower sternal edge, URSE = upper right sternal edge)
TABLE 5d: Continuous murmurs Lesion
Site
Radiation
Additional features
PDA.
• Under left clavicle.
• Back.
• Bounding pulses.
BT shunt murmur (palliative procedure for TOF, pulmonary atresia, critical PS etc).
• URSE.
• Widespread.
• Will have right or left thoracotomy scar. • Child likely to be cyanosed.
Coarctation.
• ULSE/infrascapular.
• Back.
• Absent/reduced femoral pulses. • May also be pansystolic.
(URSE = upper right sternal edge, ULSE = upper left sternal edge, BT = Blalock–Taussig, TOF = Tetralogy of Fallot, PS = Pulmonary Stenosis)
Completing the Examination ӹӹ Do a general paediatric examination. Bedside tests include: ӹӹ Review the observation chart.
ӹӹ Plot the height and weight on an age and sex appropriate chart. ӹӹ ECG. ӹӹ Urine dipstick, looking for haematuria as evidence of endocarditis. ӹӹ Fundoscopy if endocarditis is suspected.
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EXAMINATION
CASE SCENARIO
PRESENTING YOUR FINDINGS
Thomas is a 6-year-old boy seen in clinic for review today.
SCENARIO 1 Thomas is a six-year-old boy who has attended clinic today for review of his murmur. You have been asked to perform a cardiovascular examination on him and present your findings. EXAMINATION CHECKLIST Domain
Findings
Evolving thought process
Global assessment • Well. of child. • Some dysmorphic features. • Looks small.
• Unlikely to have an acute pathology. • May have an underlying syndrome, which may be associated with a cardiac disorder. • Needs to have height/weight plotted.
Inspection.
• Upslanting palpebral fissures. • Flat nasal bridge. • Not cyanosed. • No scars.
• Cardiac lesion not severe enough to require surgery or to cause cyanosis. • May have Trisomy 21, which is associated with VSD, ASD, and AVSDs.
Palpation.
• Normally placed apex. • No thrills or heave. • Normal pulses.
• No additional pathology identified.
Auscultation.
• Normal heart sounds. • No click. • 3/6 pansystolic murmur LLSE. • Does not radiate.
• Consistent with VSD.
DEVELOPMENTAL ASSESSMENT This section will focus on performing a developmental assessment in a child under five years. A full assessment is usually only conducted by subspecialty paediatricians, so here broad assessments of each of the key domains will be touched upon. Within this, it should be possible to assess the developmental age of a child to within about six months. For each domain, given an approximate developmental age, establish what the child can do and importantly what they cannot do - although they may not display what they can do if they decide not to co-operate! The developmental examination should answer: 1 Is this child’s development appropriate for their age? 2 If there is delay, is it one domain or is it global?
TABLE 6: Patient conditions that may appear in clinical examinations Condition
Possible clues
Cerebral Palsy – diplegia or hemiplegia.
• Isolated motor delay – gross and fine possibly.
Duchenne Muscular Dystrophy.
• Delayed motor milestones. • May also be speech delay.
Trisomy 21.
• Delayed motor milestones due to hypotonia. • May be hearing concerns. • Typical facial features.
Autism Spectrum Disorder.
• Delays in speech and social interaction.
Global developmental delay.
• Delay in more than two domains.
Normal child.
• No delay evident.
Thomas looks well and appears comfortable and acyanotic at rest. He has dysmorphic facies, with upslanting palpebral fissures and a wide flat nasal bridge. Thomas has a normal pulse rhythm and volume with a rate of 92 beats per minute. He has no scars on his chest. His apex beat is non-displaced. On auscultation of his chest, he has a 3/6 pansystolic murmur heard loudest at the lower left sternal edge that does not radiate. There is no evidence of cardiac failure. These findings are consistent with a VSD in a child with Trisomy 21. To complete my examination, I would like to plot Thomas’ height and weight on an age and sex, trisomy 21 appropriate growth chart, measure his BP and perform an ECG. To confirm the diagnosis of a VSD, Thomas would need to be referred for an echocardiogram.
Summary Checklist Global assessment of the child on the approach. Gross motor skills. How the child moves overall. Fine motor skills and vision. Interacting with objects, drawing, building blocks. Language and hearing. Expressive and receptive language. Social and self-care. Interactions with others. Completing the examination. Testing other domains. If not done, a neurological examination may be appropriate. Bedside tests. Plot height/weight, review observation chart.
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3 If there is delay, is it significant or mild? Common exam scenarios are shown in Table 6.
When testing a child’s development, here are some top tips: ӹӹ Have a logical schema in mind for assessing each of the domains. Leave gross motor skills to the end in the older child as this will involve getting them to run and jump and after this they may not be so willing to sit down and draw! ӹӹ Check that the child can speak/understand English, can see and can hear. ӹӹ If testing requires use of equipment, e.g. a pencil or bricks, observe what the child does with them first before asking for a specific task. ӹӹ Test what the child can do and test what the child cannot do to obtain the maximum developmental age. ӹӹ Get down to the same level as the child. An adult standing over a child is very intimidating. ӹӹ Involve the parent in the activity if the child is wary. ӹӹ Remove the equipment from one activity before moving onto the next so that there are not too many distractions for the child. ӹӹ If a child is unable to perform a task, then they are “unable to demonstrate to me today” rather than they cannot. It may be that they just did not want to perform on the day, so always base your summary on what you have seen today rather than dismissing the child completely.
Starting the Examination Before examining the child, initial thoughts can be gathered. These will be specific to the child being examined, but may include: 1 Dysmorphic features. This may hint at a possible underlying cause for developmental delay. 2 Paraphernalia in the room. Mobility aids, e.g. wheelchairs imply motor delay. Medications around the bed may point to a possible diagnosis. 3 Interactions with parents, and general behaviour. Much of the developmental assessment may happen without formal assessment occurring. The child may be running and jumping around the room: instantly, the gross motor development is likely to be at least 3-years-old. They may be speaking in two word phrases to mum: speech development is at least 18-months-old. 4 Gross appearance of the child. From this initial observation, it should be possible to guess a rough age range for the child. It should be possible to tell roughly if a child is under 6-months-old, 6 to 12-months-old, 1 to 3-years-old or over 3-years-old, although of course with faltering growth, estimates may be misleading.
Gross Motor Skills The tests below are roughly listed based on what is expected in younger children first. Start at what appears to be the most
EXAMINATION
General Advice
appropriate stage. If the child looks under six-months-old, start at the beginning. If they demonstrated walking with a steady gait on entering the room, there is no need to test for developmental milestones achieved before this landmark. 1 Movements and position at rest. Are they moving all four limbs, and are they moving them equally? Can they roll over? (5 to 6-months-old). 2 Primitive reflexes (non-mobile child). a Moro. Sudden downward dropping of head (whilst supporting bottom) results in both arms being flexed/ abducted (< 3-months-old). b Grasp. Examiner places finger in baby’s hand, and the baby reacts by grasping it. (< 3-months-old). c Stepping. Holding the baby upright on a flat surface, the baby lifts one foot and then the other as if stepping/walking (< 4-months-old, and reappears at 12-months-old). d Asymmetrical tonic neck reflex. With the baby lying on their back: when their head moves to one side, the arm and leg on the side the head is turned towards extend, and the opposite arm and leg flex (< 6-months-old). 3 Place in ventral suspension. Is there any head lag? a Some head lag (6-weeks-old). b No head lag. Also able to lift head above midline (3-months-old). 4 Place them prone. Can they: a Lift head and/or raise chest resting on elbows? (3-months-old). b Raise chest with fully extended arms? (5 to 6-months-old). 5 Place the child in a sitting position. Can they: a Sit supported/unsupported with a curved back (5 to 6-months-old). b Sit unsupported with a straight back (8 to 9-months-old). c Crawl/bum shuffle (8 to 9-months-old). 6 Pull to a standing position. Can they: a Stay in a standing position? (10-months-old). 7 Moving on two legs. Can they: a Cruise. Walk while holding on to things (10-months-old). b Walk. i With an unsteady gait (12-months-old). ii With a steady gait (15-months-old). c Run (18-months-old). d Stoop down to pick things up (18-months-old). e Jump and kick a ball (2-years-old). f Ride a tricycle (3-years-old). g Hop (4-years-old). h Stand one foot for ten seconds (5-years-old). 8 Walking up and down stairs. a 2 feet per step (2-years-old). b 1 foot per step going up, 2 feet per step going down (3-years-old). c 1 foot per step going up, 1 foot per step going down (4-years-old).
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EXAMINATION
9 Other. a Bounces and catches a ball (5-years-old). b May be able to ride a bike without stabilisers (5-years-old).
FIGURE 48
Fine Motor and Vision Start by generally observing the child playing, and crudely assess what they can do. Assess them whilst on the same level as they are, and make sure that there are no distractions around (e.g. clear up building blocks when another test is being done). Give them a crayon and see what they draw. Then draw the different images (from a line to a triangle, depending on the child’s age). Cover the image when drawing it so that the child cannot see how you have drawn it (Figure 47). This tests copying. If the child can see how you have drawn the image, this instead tests imitation, which happens earlier than copying. For each construction with the bricks, produce it for the child first, and then break the construction, and ask them to make it again (Figure 48). If they cannot do this, try asking them to imitate the construction, where they see how you have made it. Fine motor landmarks are shown in Table 7 and 8.
A
B
C
D
Demonstrating building a L-shaped train (A), child replicating a L-shaped train (B), child replicating 6 brick stairs (C), and child replicating ten brick stairs (D).
Additional skills tested include: ӹӹ Scissors. ›› Using both hands (2-years-old). ›› One handed (3-years-old). ›› Cuts a page in half. Cuts along a line progressing to cutting out shapes (4-years-old). ӹӹ Page turning. ›› Skimming pages (15 to 18-months-old). ›› Turning pages singly (2-years-old). ӹӹ Threading beads. ›› Can thread large beads (3-years-old). ›› Can thread small beads (4-years-old).
FIGURE 47
TABLE 7: Fine motor landmarks (Under 1)
A
B Drawing images in a manner that is hidden from the child (A), and exposing each image individually for the child to copy (B).
Age
Action
6 weeks.
• Holds hand in fist. • Grasps anything put in palm.
6 months.
• • • •
9 months.
• Crude pincer grip.
10 months.
• Fine pincer grip.
12 months.
• Casts objects away. • Bangs objects together. • Object permanence (can be tested simply by dropping a toy and seeing if the child looks for a fallen object). • Points at things.
Palmar grasp. Reaches out for objects. Brings object to midline. Hand to hand transfer (taking an object from one hand and putting it in the other). • Mouthing objects (putting them in their mouth).
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DEVELOPMENTAL ASSESSMENT
Vision
TABLE 8: Fine motor landmarks (Over 1)
Speech, Language and Hearing Ask/assess whether there are any concerns about hearing at the beginning and if English is the first language. Then speak to the child, getting them to speak back. Note landmarks described in Table 9. Useful props include picture books and small objects (e.g. bear, pencil and cup).
Age
Drawing
Building blocks
15 months.
Marks paper (cylindrical grasp).
Tower of 2.
18 months.
To and fro scribble.
Tower of 3.
2 years.
Circular scribble.
Tower of 6.
2.5 years.
Copies line.
–
3 years.
Copies circle (tripod grasp).
Tower of 9.
EXAMINATION
Test ability to fix and follow. Hold up an interesting (silent) object and see if the child follows it with their eyes. Children can follow to 90 degrees by six-weeks-old, and 180 degrees by three-months-old.
–
Social and Self-care This comes from observing the child, playing games with them, and getting a thorough history from the parents (Table 10).
Completing the Examination ӹ Do a general paediatric examination. ӹ Perform a neurological examination. ӹ Examine the eyes and hearing. Bedside tests include: ӹ Review the observation chart. ӹ Plot the height and weight on an age and sex appropriate chart.
Train with chimney (L shaped). 3.5 years.
Copies cross.
3 brick bridge.
4 years.
Copies square.
6 cube stairs.
5 years.
Copies triangle (and can draw person, house with chimney/windows/door).
10 cube stairs.
TABLE 9: Speech and language milestones Age
Understanding (receptive language)
Newborn.
Vocalising (Expressive language)
Age
Understanding (receptive language)
Vocalising (Expressive language)
• Crying.
2 years.
• Verbs and two part commands.
• 50 words and 2 word phrases.
2.5 years.
• Prepositions (e.g. put doll under table).
• 3-4 words together.
3 years.
• Comparatives (e.g. which circle is the biggest?). • Adjectives (e.g. which one is red?). • Negatives (e.g. which is not a fork?).
• What/where questions. Can give full name and sex.
4 years.
• Follows three part/complex instructions (e.g. first give me the pencil, then grab the key and put it in the cup).
• Why/when/how questions. • Talks in narratives/ sequences. • 4-5 word sentences.
5 years.
• Enjoys jokes.
• Talks about past/ present/future.
6 weeks.
• Startles to noise.
3-4 months.
• Turns to sound.
• Cooing.
5-6 months.
• Turns to own name.
• Babbling.
8-9 months.
• “Mama” “dada” said indiscriminately.
10 months.
• Understands “no”.
12 months.
• A few simple nouns (e.g. “mum”) and first name.
15 months.
• Follows simple commands. Points to body parts.
18 months.
• Lots of simple nouns (e.g. leg). • 10-20 words. Knows 2-3 body parts.
• A few individual words.
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EXAMINATION
TABLE 10: Social/self-care milestones Age
Social
6 weeks.
• Social smile i.e. child smiling back at you or when pleased.
8-9 months.
• Stranger anxiety (up until two years). • Plays peekaboo. • Holds/bites food.
12 months.
• • • •
18 months.
PRESENTING YOUR FINDINGS
Eva is a six-month-old corrected premature infant. She appears well, with no dysmorphic features. On assessment of her gross motor skills, she demonstrated the ability to sit supported with a curved back. When held in ventral suspension, she demonstrated the ability to lift her head above the midline.
Waves bye-bye. Claps Points. Drinks from beaker.
On assessment of her fine motor skills, Eva demonstrated the ability to bring both hands to the midline.
• Imitative play / domestic mimicry (copies parent’s actions at home). • Symbolic play, e.g. brushes doll’s hair, may feed/put doll to bed. • Removes socks / shoes.
2 years.
• • • • •
3 years.
• Plays with other children. • Shares. • Eats with fork and spoon.
4 years.
• • • •
Dresses and undresses. Concern/sympathy for others. Understands turn-taking. Eats with Knife and fork.
5 years.
• • • •
Very definite likes and dislikes. Can amuse themselves for long periods of time. Can care for pets. Chooses best friends.
Socially, she demonstrated the ability to smile. From my assessment of Eva, I would place her around the six month age based on her fine motor skills and between four and six months based on her gross motor skills.
Temper tantrums. Plays alongside other children (parallel play). Eats with spoon. Starts toilet training. Loses stranger anxiety.
I would like to assess the rest of Eva’s development, plot her length, weight and head circumference on an appropriate centile chart and perform a neurological examination. If these were all normal, I would keep her development under review until the age of two years.
CASE SCENARIOS SCENARIO 2 Eva is an ex-premature infant, born at 28 weeks gestation, now nine-months-old (six-months-old corrected). Observations during the developmental assessment are shown in Figure 49.
FIGURE 49 A. Placed in sitting position. B. Placed in ventral suspension. C. Observing hand movements. D. Observing social interactions.
A
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DEVELOPMENTAL ASSESSMENT
SCENARIO 3
PRESENTING YOUR FINDINGS
Observations during gross motor assessment are shown in Figure 50, and fine motor/vision assessment in Figure 51. FIGURE 50 A. Moving along floor. B. Interacting with an object placed in front of him. C. Unsupported on both feet.
James is an 18-month-old boy. He appears well, with no dysmorphic features. On assessment of his gross motor skills, James demonstrated crawling and standing with a steady stance. He was able to stoop down to the floor to pick a toy up with good balance.
EXAMINATION
James is an 18-month-old that has attended clinic today as a result of concerns about his development.
On assessment of his fine motor skills, James demonstrated to and fro scribbling but did not demonstrate circular scribbling. Although able to turn a thick-paged book, James skimmed multiple pages of a paper book at once. From my assessment of James, I would place him around the 18 month age, based on his fine motor and gross motor skills.
A
B
C
I would like to assess the rest of James’ development, plot his length, weight and head circumference on an appropriate centile chart and perform a neurological examination. If these were all normal, I would explore further what the specific development concerns were, and send off any appropriate investigations.
FIGURE 51 A. Using pen and paper. B. Using thick paged book. C. Using thin paged book.
A
B
C
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EXAMINATION
SCENARIO 4
FIGURE 56
Tristan has been brought to clinic today for a developmental review.
Fine motor assessment. A. Turning pages of a book. B. Threading beads onto string. C. Cutting paper using scissors.
Gross motor assessment is shown in Figure 52. Fine motor/vision assessment is shown in Figure 53-6. Additionally, it is noted that he can copy a circle but not a cross. Speech and language assessment is shown in Figure 57-9. Social behaviour/self-care is shown in Figure 60. There was no stranger anxiety throughout the interaction. FIGURE 52
FIGURE 54
Gross motor assessment. A. Standing on one leg. B. Jumping. C. Kicking a ball.
Fine motor assessment. A. Demonstrating a bridge, and driving a pen through it. B. Copying building a bridge a driving a pen through it.
B
A
A
A
B
C
B
FIGURE 55 Fine motor assessment. A. Demonstrating six brick stairs. B. Attempting to copy six brick stairs.
C
A
B
FIGURE 53 Fine motor assessment. A. Demonstrating a brick tower. B. Copying a tower of three. C. Copying a tower of six. D. Attempting to copy a tower of eight, but it becomes unstable.
A
B
C
D
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DEVELOPMENTAL ASSESSMENT
FIGURE 57
FIGURE 59
Tristan is asked questions surrounding a picture book.
Tristan is given instructions with a pen, cup and teddy bear in front of him.
A “Put the cup on the teddy!”
B “Who is not on the beach?” B “Which one do you write with?”
EXAMINATION
“Who is the biggest?”
Tristan is asked to point to his head.
I was asked to examine Tristan’s development today. Tristan appeared well grown and there were no dysmorphic features. Assessing Tristan’s gross motor skills, he demonstrated standing on one leg, jumping and kicking a ball. This would put his gross motor skills at those of a 3-year-old.
A
FIGURE 58
PRESENTING YOUR FINDINGS
Tristan demonstrated copying circles but not crosses. He demonstrated building a tower of six bricks and a three brick bridge. He did not demonstrate building a set of steps using six bricks, and on attempting to build an eight brick tower, the tower became unstable. Tristan turned book pages one at a time. He was able to thread large beads onto string and used scissors two-handed to cut. This would put his fine motor skills at those of a 3-year-old. Tristan demonstrated knowledge and understanding of size comparatives, body parts, prepositions, object function and negatives. This would put his speech and language skills at those of a 3-year-old.
C “Touch your head!”
“Which one is not brown?”
FIGURE 60 A. Playing. B. Drinking.
A
B
Finally, Tristan demonstrated symbolic play and was able to use a cup with one hand. There was no stranger anxiety throughout the interaction. I would like more information on social development, but it is likely to be in line with other parameters. Overall, I would estimate Tristan’s age to be 3-years-old. To complete my examination, I would like to plot his height, weight and head circumference on an appropriate centile chart and perform a neurological examination.
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EXAMINATION
SCENARIO 5
FIGURE 63
Ritchie has been brought to clinic today due to parental concerns that his development is not as advanced as his peers.
Receptive language. A brick, spoon and teddy bear are placed in front of the child.
A
Gross motor assessment is shown in Figure 61. Fine motor/vision assessment is shown in Figure 62. Additionally, it is noted that he can copy a triangle and build a ten brick staircase. Speech and language assessment is shown in Figure 63-4. Social and self-care are shown in Figure 65.
FIGURE 61
FIGURE 62
Gross motor assessment. A. Hopping. B. Ball catching.
Fine motor assessment. Cutting technique.
A
“Put the brick under the teddy!”
B
B
“Pick up the brick with the spoon and feed it to the teddy!”
FIGURE 64 Using expressive language
B
A
"Are you a boy or a girl?"
"What is your name?"
"Ritchie Edwards"
C
Boy
D "How do you make a jam sandwich"
"Do you like football?"
Take the bread. Put on butter. Add jam, put another bit of bread on it, then eat it!
"Yes. Last week I saw England play."
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DEVELOPMENTAL ASSESSMENT
FIGURE 65 Social/self care assessment. A. Eating with a knife and fork. B. Doing up buttons.
B
EXAMINATION
A
PRESENTING YOUR FINDINGS
I assessed all four domains of Ritchie’s development today. Ritchie appeared well grown and there were no obvious dysmorphic features. He has what appears to be a cafe-au-lait birthmark in centre of his forehead. Assessing Ritchie’s gross motor skills, he demonstrated hopping and catching a ball underarm. Mum tells me that he walks up and down stairs in an adult fashion. This would put his gross motor skills at those of a 5-year-old. Assessing Ritchie’s fine motor skills, he demonstrated building steps of 10 bricks. Ritchie demonstrated copying shapes including a triangle. He was able to cut in a straight-line using the scissors one- handed. This would put his fine motor skills at those of a 5-year-old. Assessing Ritchie’s speech, he was able to comprehend prepositions, and complex instructions, putting his receptive language at least at the level of a 4- year-old. His expressive language included knowing his name, and gender, as well as speaking in short sentences, with an appreciation for sequences of events. He was also able to talk about the past. His expressive language was at the level of a 5-year-old. Ritchie demonstrated eating with a knife and fork and doing up buttons, which puts him at least at the level of a 4-year-old. Based on my assessments today, Ritchie’s development is in keeping with a child of 4.5 to 5 years of age. To complete my examination, I would like to plot his head circumference, height and weight on a centile chart and perform a relevant neurological examination.
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EXAMINATION
ENT EXAMINATION ENT examinations should always be performed as part of a routine examination. Some specific times when examining the ENT system is particularly pertinent include the child presenting with fever, sore throat, ear pain, or symptoms and signs suggestive of upper airway obstruction. Neck lumps are covered separately on p573.
TABLE 11: Examples of causes of stridor in children Cause
Clinical features
Laryngomalacia.
• Infants. • Inspiratory stridor, worse when lying down, crying, and during URTIs. Improves when placed prone. • Usually child is growing well.
Croup.
• • • • • • •
Bacterial tracheitis.
• Stridor. • High fever. • Barking cough: not responsive to croup treatment and/or rapid deterioration. • May have been a preceding viral URTI.
Epiglottitis.
• • • • •
Stridor. Difficulty swallowing. Sat forward, drooling. Febrile. Irritable and toxic.
Swallowed foreign body.
• • • • •
Stridor. Difficulty swallowing. Drooling. Afebrile. May be a history of coughing/choking.
Anaphylaxis.
• • • • •
Stridor. Swelling of tongue. Wheeze. Allergic reaction: rash, swelling of face/eyes. Allergen exposure.
Subglottic and tracheal stenosis.
• History of prolonged/multiple intubations. • Subglottic stenosis: inspiratory or biphasic stridor. • Tracheal stenosis: often expiratory, may be due to external compression of trachea by vascular rings, masses, or abnormal aortic arch.
Starting the Examination This begins upon entering the room. Observe for the following: 1 Does the child look acutely unwell? Are they coughing/ making any additional respiratory noises? ›› Stridor. Inspiratory or expiratory, soft or harsh, indicates partial obstruction of the airway at level of supraglottis, glottis or subglottis, e.g. in laryngomalacia or croup (Table 11). ›› Stertor. Snoring sound due to partial obstruction of airway in oronasopharynx, e.g. large tonsils and adenoids. 2 Has any initial treatment or monitoring been started? Are they attached to oxygen or any monitoring?
Inspection This is more focused. Ideally expose the complete chest. Whilst the external ear can be inspected, a thorough inspection of the ear and throat with an auroscope is needed at the end of the examination.
Hands ӹӹ Rash. Scarlet fever is associated with an erythematous coarse rash that feels like sandpaper. ӹӹ Desquamation. Peeling of the skin, particularly of the finger tips, occurs in scarlet fever (and also Kawasaki disease).
Face ӹӹ Upper respiratory tract infection. Look for puffiness of the eyes, nasal discharge and redness of the nose/eyes. Listen for any cough. ӹӹ Conjunctivitis. Note that non-purulent bilateral conjunctivitis is a criterion for Kawasaki disease. ӹӹ Mastoid swelling. This suggests mastoiditis which can be a complication of otitis media. Look for swelling and redness behind the ear. It may result in the ear appearing to be pushed forward. ӹӹ Drooling. A child with an open mouth who is drooling indicates difficulty swallowing saliva. This may be due to tonsillitis or, rarely, bacterial tracheitis or epiglottitis. ӹӹ Lymphadenopathy. Red, swollen lymph nodes may be secondary to ENT infections. Lymph node abscesses may also develop. ӹӹ Discharge. From ear, skin lesions, or nose. ӹӹ Obvious facial nerve palsy. This may be secondary to mastoiditis or severe otitis media.
Mostly 6 months - 3 years. Inspiratory +/- expiratory stridor. Barking cough. Voice may be hoarse. Fever. Coryza. Worse at night.
Summary Checklist Global assessment of child. Inspection. Hands, face, chest, ears, throat. Palpation. Pulse, BP, lymphadenopathy. Special tests. Ear and throat examination. Completing the examination. Auscultate chest and heart, examine for hepatosplenomegaly, look for rashes. Bedside tests. Plot height and weight, swab throat, urine dipstick.
Ears ӹӹ Discharge. Look for any obvious discharge or blood coming from either ear.
Chest ӹӹ Respiratory rate and work of breathing. Respiratory distress may be secondary to fever or pain.
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ENT EXAMINATION
FIGURE 66
Palpation
A
Hands/arms
Neck ӹӹ Cervical lymphadenopathy. Reactive nodes will usually be small, mobile and tender (Figure 66). If any lymph nodes are palpated, palpate for lymph nodes in the axilla and inguinal region, as well as assessing for hepatosplenomegaly.
EXAMINATION
ӹӹ Pulse rate and rhythm. The child may be tachycardic if febrile, septic or in pain. ӹӹ Blood pressure. This is important, as any infection can lead to sepsis.
Special Tests Ears
B
Preauricular: ear, scalp Parotid: parotid, ear Retropharyngeal: tonsils Submandibular: tongue, floor of mouth Submental: lip, mouth, teeth Anterior cervical: thyroid, larynx, pharynx, tonsils Supraclavicular: thoracic, and abdominal cavity Occipital: scalp Post auricular: ear, scalp Posterior cervical: scalp, upper respiratory tract
Sternocleidomastoid muscle
Trapezius muscle
A. Palpate for cervical lymph glands from behind. B. The groups of nodes are listed with areas of possible sources of infection if they are reactive lymph nodes.
Positioning here is key (Figure 67). Hold the otoscope between your thumb and forefinger. Insert the otoscope, and look at: ӹӹ Ear canal. Look for any discharge. Examine for redness. Is there a foreign body present? A foreign body left within the ear can cause pain, discharge and infection. ӹӹ Ear wax. If the canal is full of wax, the ear drum cannot be visualised. ӹӹ Eardrum. A normal eardrum should be pink/grey and translucent in appearance. A red or dull drum suggests infection. There may be a perforation in the eardrum as a result of trauma or infection. ӹӹ Grommets. These are small circular devices or rings inserted into the ear drum to allow drainage of fluid in chronic otitis media with effusion (glue ear). Table 12 describes the signs of otitis media and otitis externa.
Throat/Tonsils Again, as for examination of the ear, positioning the child correctly helps to ensure success of visualisation (Figure 68). Use a tongue depressor and light source (e.g. pen torch or otoscope). Look for: ӹӹ Enlarged tonsils. Red and enlarged tonsils suggest tonsillitis. If they meet in the midline, they are referred to as “kissing tonsils”. Children with kissing tonsils, particularly with exudate, usually need to be admitted for intravenous antibiotics and given oral corticosteroids. They are at risk of airway obstruction. ӹӹ Exudate. White spots of pus covering the tonsils. This is more suggestive of bacterial tonsillitis. ӹӹ Quinsy. This is a complication of tonsillitis. It is a peritonsillar abscess that will require drainage. Often the child will not be able to fully open the mouth and the palate will be swollen outwards (convexly) rather than its usual concave appearance. ӹӹ Deviated uvula. This may indicate a quinsy if pushed away from the affected side. ӹӹ Palatal petechia. Look at the roof of the mouth. Occurs in infections caused by Epstein-Barr Virus (EBV). ӹӹ Strawberry tongue. Seen in scarlet fever.
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EXAMINATION
FIGURE 67 A
B
A. Positioning a young child for otoscopy. Younger children should be seated in their parent’s lap, facing to one side. The parent should then place one arm around the child’s body, and the other around the child’s forehead. Ensure one finger from the hand holding the otoscope rests against the face to protect against sudden movements. The holding of the child both comforts them and prevents them from sudden movements. Pull the ear down and back to better visualise the eardrum. B. Positioning an older child for otoscopy. In older children, pull the ear up and back.
TABLE 12: Summary of otitis externa and otitis media Otitis Externa
Otitis Media
• Ear pain.
• Ear pain.
• Hearing may be muffled.
• Fever.
• Ear may be itchy.
• May tug at ear.
• Red ear canal.
• Bulging tympanic membrane.
• Ear discharge.
• Effusion (purulent) behind tympanic membrane.
• Usually unilateral.
• Can be bilateral.
ӹӹ Teeth and gums. A koplick spot is white spot found on buccal mucosa opposite the lower molars. It indicates measles. Oral thrush will result in a white coating/spots to the tongue and/or gums.
Completing the Examination ӹӹ Perform a general paediatric examination. ӹӹ Hepatosplenomegaly. In the context of an acute pharyngitis/ tonsillitis is likely to indicate EBV infection. ӹӹ Rash. Examine the rest of the body for rash, including feet.
FIGURE 68 A
B
C
D
Throat examination. A/B. Younger child being positioned by parent, sat on their lap facing forward. The parent places one hand on the forehead and one arm around the child, enclosing both arms. C/D. Older child, with or without tongue depressor, depending on adequacy of view.
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EXAMINATION OF THE EYE
EXAMINATION CHECKLIST Domain
Findings
Evolving thought process
Global assessment of child on approach.
• Alert. • Looks miserable. • Coryzal.
• May have an upper respiratory tract infection (URTI).
Inspection.
• Pulling at ear. • No discharge from ear. • Mouth closed. • No chest recessions.
• Ear pain, possible ear infection.
Palpation.
• Tachycardia. • Normal BP. • Small cervical lymph nodes. • Feels hot.
• Tachycardia secondary to pain/fever/possible sepsis.
Throat examination.
• Tonsils not enlarged or red. • No exudate.
• Tonsils not inflamed.
Ear examination.
• Right ear drum dull and bulging. • Ear canal red.
• Likely right otitis media.
CASE SCENARIO SCENARIO 6 Paul is a 2-year-old boy who has presented to ED with fever, coryza and reduced appetite. Please perform an ENT examination.
EXAMINATION
Bedside tests include: ӹӹ Review the observation chart. ӹӹ Plot the height and weight on an age and sex appropriate chart. ӹӹ Swab any discharge or tonsillar pus. ӹӹ Urine dip for proteinuria/haematuria as throat infections can cause a glomerulonephritis.
PRESENTING YOUR FINDINGS
Paul is a 2-year-old boy whom I have seen in ED. He presented with a history of fever, coryza and a reduced appetite. Paul appears to have an acute febrile illness and appears miserable with this. He is febrile and tachycardic, with a normal blood pressure. On examination, Paul is coryzal and is pulling at his right ear. He has some cervical lymphadenopathy. Examining his right ear, the ear canal is erythematous and his tympanic membrane is red and bulging. His tonsils are normal. These findings are consistent with an upper respiratory tract infection, specifically a right-sided otitis media. To complete my examination, I will examine for lymph nodes elsewhere, palpate for hepatosplenomegaly and examine for other possible sources of infection. If his heart rate comes down after administration of analgesia/antipyretics, I would advise discharge, analgesia and antipyretics as needed and regular fluid intake. If it does not, he requires reassessment, as he may be septic or have an infection from another source.
EXAMINATION OF THE EYE Eye examination is carried out in the following situations: 1 If there is a change in the quality or size of the visual field. In younger children, clues to this may be the child bumping into things or being more clumsy than usual. 2 If malalignment of the eyes is noted or there is double vision. This may be noticed by parents, or teachers. 3 If there are neurological signs/symptoms. This includes any other neurological features, such as headaches. Common patients that are likely to appear in exams are shown in Table 13.
Starting the Examination The examination begins by observing the child on entering the room. Begin with a broad assessment: 1 Does the child look well or unwell? Do they have any dysmorphic features (e.g. Trisomy 21 is associated with squints)? Any features of cerebellar disease (e.g. tremor)? 2 Does the child wear glasses? The use of glasses implies a partially correctable visual defect. If a refractive error is being corrected, the thickness of lenses is a good proxy marker for the severity of the defect.
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EXAMINATION
TABLE 13: Common patients that may appear in clinical examinations Diagnosis
Possible clues
Squint (Strabismus).
• Most likely to be intermittent and related to refractive errors (child will have glasses).
Nystagmus.
• Can be congenital. • May be part of oculocutaneous albinism. • May be associated with cerebellar signs.
Cranial nerve palsy.
• III. Eye position is down and out, pupil may be dilated, and associated with ptosis. Can be congenital or secondary to tumour. • IV. Impaired depression of the eye from the abducted position. It can be congenital or acquired, e.g. due to head trauma. It often causes double vision and the head to be tilted. • VI. Impaired abduction (looking outwards) of affected eye. It can be congenital or acquired, e.g. trauma, raised intracranial pressure, or tumours.
Inspection ӹӹ Eye position. Look at where the pupils are in relation the rest of the eyes (Figure 69). In a squint, the pupils may be pointing in a different direction. ›› Nystagmus will be demonstrated by rhythmic to and fro movements of the eye. It can be horizontal, vertical or circular in direction and is involuntary. Any nystagmus identified should prompt assessment of hearing and the cerebellum. ›› Opsoclonus is brief, rapid movements of the eyes in all directions. It is described as “dancing eyes”. Opsoclonus myoclonus syndrome is a condition of opsoclonus, myoclonus and ataxia that, in children, may be due to infection but importantly can be a paraneoplastic phenomenon of neuroblastoma. ӹӹ Pupil size/symmetry. The pupils should be equal in size, and neither dilated or constricted, although this will vary depending on the lighting. ӹӹ Ptosis. A droopy eyelid could be congenital or a sign of a facial nerve palsy. ӹӹ Conjunctiva. Conjunctivitis implies inflammation of the eye, which may relate to allergy or an infection. Conjunctivitis may be purulent, which implies an infective cause, or non-purulent. Non-purulent conjunctivitis is a feature of Kawasaki disease. It can be differentiated from uveitis, Symmetrical corneal reflections FIGURE 69
Summary Checklist Global assessment of child on approach. Inspection. Glasses, eye position, pupil size/symmetry, eyelids, conjunctiva. Visual acuity. Both eyes together and then each individually. Visual fields. Test all four quadrants. Eye movements. In eight directions. Accommodation reflex. Look for convergence and constriction of pupils. Pupillary reflexes. Direct and indirect. Fundoscopy/slit lamp. Slit lamp via specialist. Squint testing. Latent vs. manifest. Paralytic vs. nonparalytic. Bedside tests. Review observations; plot height and weight.
which also causes a red eye, as the redness in conjunctivitis is usually greatest in the periphery of the eye. In uveitis, the redness increases approaching the limbus (the border between the sclera and the cornea) and is associated with pain and visual disturbances.
Special Tests ӹӹ Visual acuity. This should be measured for both eyes together, and then each eye individually. It should also be measured both with and without glasses if they are needed. Start at six metres, and if clear vision is not possible at this distance, progressively move in by one metre at a time. A Snellen chart is ideal, but for younger children picture charts might be used. In those with significant visual defects, detecting light and dark might be all that is possible. ӹӹ Visual fields. Initially test vision with both eyes open, and then test each eye individually. Test all four quadrants of vision. Confrontational testing should be used, whereby the examiner compares their visual fields to that of the patient as performed during cranial nerve examination (p587). Colour vision can be tested using a red pin, and similarly this can be used to map out the blind spot. ӹӹ Eye movements. (Figure 132-5). ӹӹ Rapid eye movements. Not routinely done, but may pick up nystagmus. (Figure 70) ӹӹ Accommodation. (Figure 130). ӹӹ Pupillary reflexes. (Figure 131). ӹӹ Fundoscopy/slit lamp examination. Fundoscopy is useful for assessing the red reflex (particularly in identifying congenital cataracts) (Figure 71). Look at the optic disc (particularly to identify papilloedema) (Figure 72). Slit lamp allows more detailed assessment by specialists.
Strabismus (malalignment of the eyes) Normal eyes. Note that the white light reflections (corneal reflections) are in identical positions in both eyes.
If strabismus (a squint) is noted, it is important to describe: ӹӹ Timing. Latent (intermittent, i.e. only appears when fixation is interrupted, e.g. covering the eye) or manifest (always
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EXAMINATION OF THE EYE
FIGURE 72
FIGURE 73A
A
A
Rapid eye movements. Make a fist with one hand, and keep an open palm with the other hand. Position both hands about 1m apart, and alternatively state “fist” and “palm”, asking the patient to look at each in response. This may elicit nystagmus. Do this with the hands separated in both the horizontal and vertical plane.
B
Cover/uncover test. A. Divergent squint at rest. B. Squint does not disappear on covering the left eye. The squint is manifest and paralytic.
EXAMINATION
B FIGURE 70
FIGURE 73B A
A A. Normal optic disc. B. Papilloedema with blurred disc margins.
B
C
FIGURE 71 Fundoscopy. A. Use a hand to check the opthalmoscope is in focus. B. Start at a distance checking the red reflex. C. Move in closer (use the right eye to look at the patient’s right eye). Stabilise the patient’s head with the other hand.
visible when eyes both open). Phoria denotes a latent squint, trophia denotes a manifest squint. ӹӹ Direction. Upward vs downward vs convergent (going inward - eso) vs divergent (going outward -exo). This
can be combined with trophia or phoria, e.g. an esotrophic squint is where the eye turns inwards. ӹӹ Eyes affected. Monocular (one eye) vs binocular (both eyes). ӹӹ Paralysis. Paralytic (nerve palsy/ muscle weakness) vs non-paralytic. Most squints are non-paralytic. An acquired paralytic squint is usually caused by a tumour or brain trauma. The above can be identified by: 1 Inspection. A manifest squint will be obvious without any special tests. 2 Corneal reflections (Figure 69). These should be in identical positions on both eyes on top of the pupil. If not, they are malaligned. 3 Eye movements. Perform the H test to identify paralysis (p587). 4 Cover/uncover test. Ask the child to fix on an object approximately one metre away. Cover each eye in turn and observe for movement of the uncovered eye. Repeat twice to identify more subtle movement. a When there is a squint present already, if the normal eye is covered, the eye with a squint may (Figure 73):
B
Cover/uncover test. A. Divergent squint at rest. B. Squint disappears on covering the left eye. The squint is manifest and non-paralytic.
i Correct and fix on the object being focused on (non-paralytic squint). This is because the dominant eye is no longer receiving an image, and so the eye with the squint becomes dominant. On uncovering the normal eye, the manifest squint will reappear, and the dominant eye will take up fixation again. ii Stay in the same position (paralytic). In this situation, e.g. cranial nerve II palsy, the eye cannot move because of a neuropathy. Note that covering the eye with the squint should not make a difference to the normal eye, as the normal eye is already fixed on an object. b When there is no squint present at rest.
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EXAMINATION
5 Alternate cover test. This tests for a latent squint (Figure 74). Again, have the child fix on an object ahead. Cover one eye and then move the cover to the other eye, observing for movement just as the eye is uncovered. Outward movement of the eye as it is uncovered means that when that eye was covered, the eye was turned inwards and vice versa.
FIGURE 74 Cover/uncover test. No squint at rest. Normal left eye, but latent squint in the right eye.
Completing the Examination ӹӹ Do a general paediatric examination.
i Covering a seemingly normal eye may reveal a latent squint. This is because fixation of that eye is interrupted. It can be identified by either looking through a semitransparent ”cover” or by quickly removing the cover, and looking for correction of the eye.
CASE SCENARIOS
Bedside tests include: ӹӹ Review the observation chart. ӹӹ Plot the height and weight on an age and sex appropriate chart.
PRESENTING YOUR FINDINGS
Joy is a nine-year-old girl seen in clinic for review today.
SCENARIO 7 Joy is a nine-year-old girl who has attended clinic today because of intermittent double vision. You have been asked to examine her eyes and present your findings. EXAMINATION CHECKLIST Domain
Findings
Evolving thought process
Global assessment of child.
• Appears well. • No dysmorphic features. • Not using glasses.
• Unlikely to have an acute pathology. No obvious causes of double vision.
Inspection.
• Looks normal.
• Need to find out what positions result in double vision.
Visual acuity.
• Normal.
• Unlikely to relate to a refractive error.
Visual fields.
• Normal.
• Optic nerve likely normal.
Eye movements.
• Develops double vision on lateral gaze of right eye. • Unable to abduct right eye.
• Lateral rectus palsy on right.
Accommodation reflex.
• Normal.
• No optic/oculomotor nerve pathology identified.
Pupillary reflexes.
• Normal.
• No optic/oculomotor nerve pathology identified.
Fundoscopy/slit lamp.
• Normal.
• No additional pathology identified.
Squint testing.
• Squint only present when looking right, but always present. • Unaffected by cover/uncover test.
• Paralytic squint.
Joy looks well and appears comfortable at rest. There are no obvious abnormalities with the eye at rest. On examination of eye movements, Joy reports double vision, worst on lateral gaze and there is a paralytic squint of the left eye on looking laterally. Visual acuity, visual fields, pupillary reflexes, accommodation and fundoscopy are all normal. This is consistent with a right sixth cranial nerve palsy. I would be concerned about a possible brain tumour and would like to complete a full neurological examination and request brain imaging (CT or MRI if available).
GASTROINTESTINAL EXAMINATION This should be performed in all general paediatric examinations. Specifically consider: 1 A child reporting abdominal pain. Where is this child’s pain most severe, how severe is it, and how might this help with diagnosis? Pain may be acute or chronic and palpation of the abdomen may localise it to an area, which will help narrow the differential, e.g. right iliac fossa pain in appendicitis. Consider whether the pain may be referred, e.g. lower lobe pneumonia causing abdominal pain. 2 Does this child have an acute abdomen? Assessment hinges on identifying signs of peritonism in the abdomen (Box 2). This is important to recognise as identification necessitates immediate treatment and surgical input. Such patients will not come up in medical school/post graduate assessments! Common patients that are likely to appear in exams are shown in Table 14.
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GASTROINTESTINAL EXAMINATION
Box 2: Signs of peritonism • Severe tenderness. • Guarding. • Percussion tenderness. • Abdominal rigidity. • Fever. • Abdominal distension. • Tachycardia/hypotension.
Wrists TABLE 14: Common patients that may appear in clinical examinations Disease
Possible clues
Sickle cell disease.
• • • •
Anaemia. Jaundice. Splenomegaly. Afro-Caribbean ethnicity.
Thalassemia.
• • • • • •
Frontal bossing. Anaemia. Jaundice. Hepatomegaly. Splenomegaly. Middle Eastern, Asian or Mediterranean origin.
Hereditary spherocytosis.
• • • •
Anaemia. Jaundice. Splenomegaly. Caucasian origin.
Liver transplant.
• Abdominal scar in the upper zone. • Palpable liver, with regular, hard edge. • May have original liver retained (auxiliary transplant), with the new liver in an ectopic position.
Surgical patient.
• Abdominal scar, with location and type correlating with likely pathology.
Starting the Examination The examination begins by observing the child upon entering the examination room. Whilst introducing yourself to the child/parents, begin with a broad assessment: 1 Does the child look well or unwell? Clues for this come from the child’s colour (pallor), respiratory rate and activity. If they are able to jump/run around, they do not have a peritonitic abdomen, whereas if they are staying absolutely still for fear of pain or if they look lethargic, this is more worrying. 2 Are there any signs of chronic ill health? See if the child looks cachectic, whether they have any medication around the bed, and if they have any medical devices, long term lines or feeding tubes in place. 3 Has any initial treatment or monitoring been started? Is the child on oxygen or intravenous fluids? Are they on a monitor?
Inspection The next phase is a more focused inspection. Ensure the child is exposed from the nipple to the knee. However, in older children, use a blanket to preserve their modesty. Babies should be fully undressed, taking care not to allow them to get cold.
Hands ӹӹ Signs of general ill health. This will include wasting of the small muscles
associated with excess oestrogen from chronic liver disease (rare in children). ӹӹ Leukonychia. White discolouration of the nails related to hypoalbuminaemia in chronic liver disease. ӹӹ Koilonychia. Spoon shaped nails, due to being thinned. Most commonly related to iron, B12 or folate deficiency.
ӹӹ Liver flap. (Figure 75) In hepatic encephalopathy, the hands will start to tremor. ӹӹ Pulse. Tachycardia, in any condition causing hypovolaemia, e.g. sepsis/GI bleeds, or pain e.g. appendicitis. ӹӹ Arteriovenous fistulae. This is used for renal dialysis.
EXAMINATION
Summary Checklist Global assessment of child on approach. Inspection. Hands, wrists, face, neck/upper torso, abdomen, legs. Palpation. Gross palpation, liver, spleen, kidney. Percuss. Liver, spleen, kidneys, bladder, ascites. Auscultate. Bowel sounds, bruits. Completing the examination. Inspect anus, external genitalia, hernial orifices. Bedside tests. Observation chart, inspect stool/urine, blood glucose, urine dipstick, plot height/weight.
Face ӹӹ Jaundice and pallor. Look for both in the conjunctiva (Figure 76). In an older child, ask them to pull their eyelid down themselves. ӹӹ Kayser-Fleischer rings. Rings seen around the iris as a result of copper deposition (Wilson’s disease). Requires formal slit lamp assessment. ӹӹ Oral ulceration. This is associated with inflammatory bowel disease or poor nutrition (Figure 77). ӹӹ Breath. Hepatic fetor associated with liver disease, or “pear drop” breath associated with ketoacidosis.
FIGURE 75 Liver flap. Ask the child to cock their wrists back and hold them in that position for 30 seconds.
in the hands from chronic disease and scars from previous cannulation. ӹӹ Clubbing. This is associated with chronic liver disease and malabsorption, e.g. inflammatory bowel disease. ӹӹ Pallor of palms. This indicates severe anaemia, e.g. in splenic sequestration in sickle cell disease. ӹӹ Palmar erythema. Redness of the palms due to increased vascularity is
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EXAMINATION
A. Exposing the sclera to look for jaundice. B. 10-year-old boy with jaundice.
ӹӹ Dentition. Poor dentition is associated with malnutrition. ӹӹ Glossitis. Inflammation of the tongue. This is associated with iron, B12 or folate deficiency. ӹӹ Angular stomatitis. Inflammation of the corner of the mouth. May relate to iron, thiamine, or B12 deficiencies. ӹӹ Freckling around the mouth. This is associated with Peutz-Jeghers syndrome, which can present with GI bleeding from polyps. ӹӹ Dysmorphic features. Facial features can give a clue as to possible pathology in a syndrome. For example, Alagille syndrome is associated with a broad forehead, sunken eyes, small chin and chronic liver disease.
FIGURE 77
Neck and Upper Trunk
FIGURE 76
A
B
Inspect the mouth, looking mainly at dentition and for ulceration.
FIGURE 78
2 9
3 4 5
2 6 3 7
4
6
5 10
Abdomen Ensure that the child is lying flat with their arms by their sides. Ask them to point to the site of any pain. Inform the child that this will be examined last and
ӹӹ Lymph nodes. Palpate lymph nodes in the neck and supraclavicular fossa. ӹӹ Gynaecomastia. This may be from excess oestrogens in chronic liver disease. It is also a normal variant, particularly in pubertal boys. ӹӹ Spider naevi. This is a central red spot with extensions radiating out like a spider’s web. It is due to dilated blood vessels, and occluding the central 1
1
spot will result in seeing the emptied peripheral veins filling from the centre. Classically seen in the superior vena cava distribution. ӹӹ Central venous access devices. There may be a temporary central line, e.g. post liver transplant, or a long term tunneled line, e.g. those on long term TPN. If they have since been removed, scars might be seen.
7
8
8
9 10
FIGURE 79
Transverse scar across the upper abdomen from a liver transplant.
Kocher’s incision Biliary/liver surgery e.g. Kasai procedure for biliary atresia. Extended transversely for liver transplant. Midline laparotomy Upper midline — upper GI surgery e.g. Nissen’s fundoplication for GORD. Longer midline incisions are used for major abdominal surgery more generally. Transverse upper abdominal incision Congenital diaphragmatic hernia repair. Splenic surgery. Pyloromyotomy scar Pyloric stenosis surgery. Appendicectomy scar Appendicitis surgery. Umbilical/sub-umbilical scar Hernia repair, exomphalos repair. Gastroschisis repair has scar within/in place of the belly button. Point incision scar Drains, ports, or biopsy sites. Inguinal scar Hernia repair. Lateral thoracolumbar scar Renal surgery e.g. nephrectomy. Hockey-Stick scar Renal transplant.
Common abdominal scars in paediatrics.
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GASTROINTESTINAL EXAMINATION
TABLE 15: Causes of abdominal masses Upper abdomen
Lower abdomen
• • • • • •
• • • •
Hepatomegaly. Splenomegaly. Liver transplant. Renal mass/enlarged kidney. Neuroblastoma. Wilms’ tumour.
Impacted faeces. Appendicular abscess. Palpable bladder. Renal transplant.
TABLE 16: Examples of how the site of abdominal pain may point to a diagnosis Disease
Site of pain
Additional possible examination findings
Constipation.
• Diffuse.
• Palpable faecal mass.
Acute appendicitis.
• Right iliac fossa.
• May have local or general peritonitis.
Gastroenteritis.
• Diffuse.
• Hyperactive bowel sounds. • May have fever.
Irritable bowel syndrome.
• Diffuse.
• No signs of peritonism. • Generally well.
Urinary tract infection.
• Suprapubic.
• Nitrites and leukocytes on urine dipstick. • Febrile.
Right lower lobe pneumonia.
• Right upper quadrant.
• • • •
EXAMINATION
if at any time it is too sore to continue, they should signal to stop. ӹӹ Distension. Distension may be secondary to flatus, fluid, fat, faeces, and do not forget foetuses in teenage girls. ӹӹ Dilated superficial veins. This is a sign of portal hypertension. They emanate from the umbilicus. ӹӹ Scars. Look anteriorly, posteriorly, in the flanks, and in the groin (Figure 78-9). These may be both operational incisions and from drains inserted at the same time. ӹӹ Drains. Surgical drains may still be in place post procedure, e.g. biliary drain post Kasai procedure for biliary atresia. ӹӹ Stoma bags. p531. ӹӹ Peritoneal dialysis catheter. This may be present in chronic renal disease. The catheter is inserted into the peritoneum, and left in situ. ӹӹ Antegrade colonic enema. p530.
Tachypnoea. Decreased breath sounds. Dull percussion note. Crepitations in right lower zone of the lungs.
Palpation Abdominal palpation in children is tricky, since it is important to keep the child as relaxed as possible. It is very important to distract them with another conversation so they do not concentrate on the pain and guarding/rigidity can be palpated accurately. It can be helpful to demonstrate the examination on a parent/teddy bear first. Another option is to turn the examination into a game, e.g. trying to feel/guess what they had for breakfast. Palpate as follows: ӹӹ Gross palpation. This is to assess for pain and for any masses (Figure 80). For neonates and small children, palpate four quadrants. In older children, palpate three upper, three middle, and three lower zones. Whilst palpating, look at the face for pain. For any masses, describe the size, site, tenderness and mobility. Consider also seeing if it transilluminates (e.g. hydrocele), or if there are overlying bruits (e.g. vascular legions). Possible causes of masses are shown in Table 15, and of pain in Table 16. ӹӹ Liver. (Figure 81). A normal liver can be felt up to two finger breadths below
FIGURE 80
FIGURE 81
A
A
B
B
Abdominal palpation. A. Optimal position, at the same level as the child. Start away from the site of pain, and always do light palpation before deep palpation. Look at the child’s face to observe for pain. B. Examination pragmatically done with the help of the mother.
Assessing for hepatomegaly. A. As the patient inhales, slowly move the hand up the abdomen from the right iliac fossa towards the costal margin. If a liver edge is felt, describe whether it is smooth/rough and soft/hard. B. Percuss to confirm the position of liver, starting at the upper chest.
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EXAMINATION
Box 3: Causes of hepatomegaly
FIGURE 82
FIGURE 83
• Inflammation. Infectious hepatitis (virus, bacteria, fungi, parasites). Autoimmune hepatitis. Drug induced hepatitis.
A
• Malignancy.
A
Primary, e.g. hepatoma, hepatoblastoma. Infiltrative, e.g. leukaemia/ lymphoma. • Vascular congestion. Right heart failure. Constrictive pericarditis. Budd Chiari syndrome. • Biliary obstruction. Biliary atresia. Choledochal cysts. Tumours.
B Assessing for splenomegaly. A. Start at the right iliac fossa, using the same technique as for the liver, but this time moving left and upward towards the left upper quadrant. B. If the spleen cannot be felt, ask the patient to rotate onto their right and repeat, as this may make the spleen palpable. Percuss to confirm.
Cystic fibrosis. • Storage disorders. Gaucher disease (lysosomal storage disease). Glycogen storage disease. Wilson’s disease. Hemochromatosis. Alpha 1 antitrypsin deficiency.
Box 4: Causes of splenomegaly • Portal hypertension, e.g. secondary to chronic liver disease, or congestive heart failure. • Malignancy, e.g. leukaemia, lymphoma. • Infection, e.g. malaria, tuberculosis, EBV.
the right costal margin. Causes of hepatomegaly are shown in Box 3. ӹӹ Spleen. Assess for splenomegaly. (Figure 82). Normal can be 1-2cm below costal margin. Causes of splenomegaly are shown in Box 4. If splenomegaly is found, look for lymphadenopathy elsewhere. ӹӹ Kidneys. Ballot the kidneys between your hands in each of the flanks (Figure 83). Causes of an enlarged kidney are shown in Box 5. Differentiating the kidney and spleen can be difficult, and key differences are shown in Table 17. ӹӹ Bladder. Palpate for an enlarged bladder in the suprapubic region. Note also that in a complete abdominal examination, the heart should be
• Connective tissue disease, e.g. SLE. • Haematological e.g. sickle cell, thalassaemia, haemolytic anaemia. • Storage disease, e.g. Gaucher disease.
palpated. If dextrocardia is present, the child may have situs inversus.
B Balloting the kidneys. Push up with the fingers of the hand that are on the back to move the kidney upwards so that it can be felt by the hand on the anterior abdomen.
Box 5: Causes of an enlarged kidney • Hydronephrosis. • Wilms’ tumour. • Polycystic kidney disease. • Renal cyst. • Renal vein thrombosis. • Tuberous sclerosis.
TABLE 17: Differentiating the kidney and spleen Spleen
Kidney
• Notch present. • Moves diagonally towards the right iliac fossa on inspiration. • Cannot get above it. • Dull to percuss. • Not ballotable.
• No notch present. • Moves down on inspiration. • Can get above it. • Resonant to percuss. • Can be bimanually ballotable.
Percussion ӹӹ Liver and spleen. Percuss the liver and the spleen. This will help assess the upper margin and confirm the lower margin. ӹӹ Ascites. If there is suspected ascites, assess for either shifting dullness (Figure 84) or a fluid thrill (Figure 85).
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GASTROINTESTINAL EXAMINATION
FIGURE 84 A
B
Auscultation Listen around 2 cm above the umbilicus: ӹӹ Bowel sounds. These may be normal, tinkling e.g. bowel obstruction, or absent e.g. peritonitis. ӹӹ Renal bruit. Present in renal artery stenosis. C
Assess for shifting dullness as follows: A. Percuss from the centre of the abdomen outwards, until a dull percussion note is found (representing fluid). B. Pinpoint the location where the note becomes dull. C. At this point, the examiner should keep their hand in the percussed position, whilst rolling the patient towards them. Wait 30 seconds, and then re-percuss. If the note is resonant, this implies that the fluid has shifted under gravity. Confirm this by rolling the patient back into their original position and re-percussing. The percussion note should become dull again.
FIGURE 85
EXAMINATION
ӹӹ Bladder. The bladder is dull to percuss, and if enlarged, can be measured by percussion up from the suprapubic region.
Completing the Examination Depending on the presenting symptoms, as well as performing a general paediatric examination, it is important to also inspect: ӹӹ The anus. Looking for fistula/skin tags (associated with Crohn’s disease). ӹӹ The external genitalia and hernia orifices. Abdominal pain could be referred pain, e.g. from testicular torsion. ӹӹ Rashes. e.g. purpura (HSP), pyoderma gangrenosum and erythema nodosum (IBD). ӹӹ Pitting oedema. This may be from hypoalbuminaemia, e.g. chronic liver disease. Bedside tests include: ӹӹ Review the observation chart. ӹӹ Plot the height and weight on an age and sex appropriate chart. ӹӹ Stool sample. ӹӹ Urine dipstick. ӹӹ Blood glucose.
Assessing for a fluid thrill. Ask a parent to place a hand over the centre of the abdomen (to prevent conduction through the skin). Firmly flick the abdomen on one flank and feel for a ‘thrill’ on the other side.
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EXAMINATION
PRESENTING YOUR FINDINGS
CASE SCENARIO
Claude is a 4-year-old who was seen in the renal clinic for review.
SCENARIO 8 Claude is a 4-year-old boy, who attends the renal outpatient clinic for routine review. You are asked to perform an abdominal/renal examination on him, and present your findings to the consultant. EXAMINATION CHECKLIST Domain
Findings
Global assessment • Alert, active, and of child on looks well. approach.
Evolving thought process • Unlikely to have an acute pathology.
• Previous central venous access scar. • Gastrostomy in situ. • Small scar to the right of his umbilicus. • Midline laparotomy scar.
• Has had major abdominal surgery in the past, which is likely to be the time when central venous access was acquired. Gastrostomy implies that there are current/past feeding difficulties. • Scar to the right of the umbilicus may relate to a previous surgical drain.
Palpation.
• Egg shaped mass over midline. • Laparotomy scar.
• Likely transplanted kidney. Given this information, the scar to the right of the umbilicus may have been a site of peritoneal dialysis.
Percussion.
• No organomegaly. • No ascites.
• No additional pathology identified.
Auscultation.
• Normal.
• No additional pathology identified.
Completing the examination.
• Normal.
• No additional pathology identified.
Bedside tests.
• Awaiting.
• Although looks well, possible faltering growth suggested by previous chronic disease (necessitating renal transplant), and gastrostomy being in situ. • May have blood/protein in urine with any renal pathology.
Inspection.
MUSCULOSKELETAL EXAMINATION A partial musculoskeletal or locomotor examination is undertaken in every paediatric assessment: gait is more often than not observed, as is an assessment of muscle bulk and gross movements. Consider the following more specific circumstances: 1 In a child presenting with joint pain, stiffness, or loss of function (e.g. difficulty with handwriting). Is this an isolated joint pathology/injury or part of a wider problem? 2 Screening for associated musculoskeletal involvement in a child with a predisposing condition. For example, psoriasis, IBD. 3 Assessment of hypermobility.
Signs and symptoms of joint disease are shown in Box 6. Common patients that are likely to appear in exams are shown in Table 18.
Box 6: Clinical features of joint disease • Pain. • Swelling. • Stiffness. • Erythema. • Locking. • Instability. • Loss of ability/function.
He looks well. He has evidence of previous central venous access. He has a gastrostomy in situ, a small scar to the right of his umbilicus and a midline laparotomy scar. The abdomen is soft and non-tender throughout, with no clinically detectable organomegaly. There is a firm, non-tender, egg-sized mass underlying the laparotomy scar. There was no associated bruit. He has no other stigmata of disease. These findings are consistent with a renal transplant. The scar to the right of his umbilicus is probably from previous peritoneal dialysis. It is not clear why he has a gastrostomy, but it could be for gastrointestinal or neurological pathology. To complete my examination, I would like to review the observation chart, dipstick the urine and formally plot Claude on a growth chart.
Summary Checklist Global assessment of the child on approach. pGALS screening. Gait, arms, legs, spine. Hypermobility screening. Beighton score. Inspection. Hands, skin, eyes, spine, individual joints. Palpation. Each individual joint. Movement. Passive then active movement of joint in question. Function. For important activities e.g. doing up buttons. Completing the examination. Auscultate heart (if hyperflexibility), palpate abdomen (if malignancy/ inflammatory arthritis suspected). Bedside tests. Review observations; plot height and weight.
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MUSCULOSKELETAL EXAMINATION
TABLE 18: Common patients that may appear in clinical examinations Possible clues
Juvenile idiopathic arthritis (JIA). (Table 19).
• • • •
IBD related arthritis.
• Often knees, ankles, wrists. • Associated signs of IBD e.g. oral ulcers, erythema nodosum, abdominal scars, colostomy at present or previously. • May appear malnourished.
Often symmetrical. Joints swollen, tender, movement restricted. May be eye signs e.g. uveitis. May be hepatosplenomegaly if systemic onset JIA.
Enthesitis related arthritis.
• Boys. • Large joint involvement including hip and sacroiliac joint. • Spine involved. • Dactylitis and uveitis may be seen.
Psoriatic arthritis.
• • • •
May be asymmetrical, often involves hands. Psoriasis rash. Psoriatic nail changes. Dactylitis.
Marfan/ • Hypermobility. Ehlers-Danlos • Scars, bruising, elastic skin. syndrome. • May have a heart murmur of aortic regurgitation or mitral valve prolapse. Osteogenesis imperfecta.
• • • •
Blue sclera. Bony limb deformities. May have an affected parent. Short stature and/or teeth affected (discolouration/damage).
pGALS Screening Questions
EXAMINATION
Diagnosis
Begin with the pGALS screening examination (paediatric Gait, Arms, Legs, Spine) to identify any difficulties in the schoolaged child. This should be performed in: ӹӹ An unwell child with pyrexia. ӹӹ A child with a limp. ӹӹ A child with regression of motor milestones/clumsiness. ӹӹ A child with chronic disease with musculoskeletal presentations (e.g. inflammatory bowel disease, but also inflammatory arthropathies).
1 Do you have any pain or difficulty moving your arms, legs, back or neck? This is associated broadly with a musculoskeletal problem. 2 Can you get dressed without any help? This is associated with difficulty in fine and gross motor skills. 3 Can you walk up and down stairs without any problems? This is associated with weakness in abductor muscles. The child will need to be exposed, ideally in shorts and girls in shorts and a vest top. Inspect the child standing upright from the front, back and sides (Figure 86). ӹӹ Skin rashes. e.g. psoriasis. ӹӹ Asymmetry. Difference in muscle bulk and leg length. ӹӹ Posture.
FIGURE 86
Starting the Examination The examination begins by observing the child upon entering the examination room. During introductions to the child/ parents, make a broad assessment: 1 Does the child look well or unwell? Are they in obvious pain? Do they have a major injury? 2 Are there signs of chronic ill health, do they look underweight? There may be an underlying inflammatory disorder contributing to the problem. 3 Are there limb supports around? e.g. walker, splints, wheelchair. The illness may already be diagnosed and partially managed. 4 Are there any dysmorphic features? e.g. Marfan’s syndrome.
A
B
C
Inspection from: A. Front. B. Back. C. Side.
TABLE 19: Differentiating features of JIA Juvenile Idiopathic Arthritis Systemic Onset (Still’s disease)
Oligoarticular
Polyarticular RF positive
Polyarticular RF negative
• Often symptoms from 10°
D
E
> 10°
The Beighton Score. A. Touching thumb to forearm. Score 1 for each side. B. Extending the little finger more than 90° at the MCP joint. Again score 1 for each side. C. Straightening the arm and extending the elbow more than 10° beyond neutral. Score 1 for each side. D. Straightening the leg and extending the knee more than 10° beyond neutral. Score 1 for each side. E. Touching the palms to the floor. Ensure that the feet are flat and that the legs are straight. Score 1 for this.
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EXAMINATION OF A NECK MASS
CASE SCENARIO
PRESENTING YOUR FINDINGS
Leann is an 11-year-old girl I saw in clinic this morning.
Leann is an 11-year-old girl who has presented to clinic today with a longstanding history of knee and hand pain. Please examine her and present your likely diagnosis. EXAMINATION CHECKLIST Domain
Findings
Evolving thought process
Global assessment of the child.
• Appears well grown. • Comfortable at rest.
• No evidence of an acute pathology.
pGALS screening.
• Normal gait, legs and spine. • Some swelling of the fingers and reduced grip.
• Appears to have hand pathology, need to examine this in more detail.
Inspection.
• Nail pits. • Dactylitis of right 4th finger, swollen DIP joints of left hand. • Psoriatic plaques over arms. • No uveitis.
• Psoriatic hand changes evident.
Palpation.
• Swollen, tender DIP joints, 2nd and 3rd fingers on left hand. • Swollen right 4th finger.
• Inflammation of DIP joints on 2nd and 3rd fingers on left and of whole 4th finger on right.
Movement.
• Reduced flexion of fingers.
Function.
• Difficulty with buttons and pencil grip.
Leann looks well grown for her age and appears comfortable at rest. On pGALS screening, I identified pathology in Leann’s hands, and therefore proceeded to examine the hands in more detail. On examination of her hand, Leann has evidence of psoriatic changes with nail pits bilaterally and dactylitis of the fourth finger on her right hand. She also has psoriasis plaques on both of her elbows. On palpation, the distal interphalangeal joints of the 2nd and 3rd fingers on the left hand are tender and swollen. Leann has reduced flexion of her fingers and a weak grip, which causes difficulties with undoing buttons and holding a pencil. My findings are consistent with psoriatic arthritis. To complete my examination, I would like to examine Leann’s other joints for evidence of further joint involvement and plot her height and weight.
TABLE 27: Common patients that may appear in clinical examinations Disease
Possible clues
• Inflammation is causing limitations of movement.
Thyroglossal cyst.
• In midline. • Moves upward on sticking out tongue.
• May be affecting school and getting dressed/ undressed.
Sternocleidomastoid tumour.
• Occurs in infants. • Just above clavicle. • Torticollis towards opposite side of the lesion.
Lymphadenopathy. • Reactive. • Infective. • Lymphadenitis.
• Often small. • May be painful with other signs of infection elsewhere. • Painful, swollen, overlying skin red.
Lymphoma.
• Painless enlargement of lymph node. • Associated fever, night sweats, weight loss, skin rashes. • Look for other enlarged nodes and hepatosplenomegaly.
Branchial cyst.
• Side of neck. • Can develop abscess, may be discharging. • Can swell during times of URTI and then recede.
Haemangioma.
• Red or bluish in colour. • Can ulcerate.
Autoimmune thyroiditis.
• • • •
Graves’ disease.
• Goitre. • Signs of hyperthyroidism, although may be euthyroid. • Proptosis. • Lid lag.
EXAMINATION OF A NECK MASS Neck masses have a wide range of possible causes and may be discovered incidentally. Common patients that may appear in exams are shown in Table 27. Summary Checklist Global assessment of child. Inspection. Neck and mouth. Palpation. Lymph nodes, and any neck mass. Special tests. Sticking tongue out and swallowing. Auscultation. Mass for bruit. Completing the examination. General lymphadenopathy, hepatosplenomegaly. Bedside tests. Review observations, plot height and weight.
EXAMINATION
SCENARIO 9
Goitre. Short. May be clinically euthyroid or hypothyroid. Often a girl.
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EXAMINATION
Starting the Examination
Palpation
The examination begins by observing the child upon entering the examination room. On introduction to the child/ parents, begin with a broad assessment: 1 Does the child look well or unwell? Many children with a neck lump will be otherwise asymptomatic, but bear in mind the child may have a severe infection. 2 Are there any signs of systemic disease? Look particularly for any suggestion of thyroid disease.
ӹӹ Lymphadenopathy. Palpate from behind the anterior cervical, posterior cervical and submandibular chains (Figure 66/90). If lymph nodes are present, are they small and reactive? Define their size. Lympahdenopathy indicates that there may be an infective process, particularly if they are painful. Painless lymphadenopathy may indicate lymphoma.
Inspection As for all examinations, begin inspection on entering the room by making a broad assessment if the child appears unwell and if there is any monitoring attached or treatment already in progress. Then focus on the neck and mouth:
For any mass identified in the neck, comment on: ӹӹ Size. ӹӹ Shape. FIGURE 90
Auscultation ӹӹ Thyroid bruit. This may be heard in Graves’ disease.
Completing the Examination ӹӹ Examine for lymphadenopathy in axillae and groin and hepatosplenomegaly. ӹӹ If a thyroid lump is suspected, assess the patient’s thyroid status. ӹӹ Perform a general paediatric examination.
Neck ӹӹ Scars. This may be from previous thyroid surgery/lymph node excision. ӹӹ Neck masses. Identify the location and size.
Mouth ӹӹ Tooth decay/gum disease. This can be a cause of infection resulting in a neck lump.
Surface. Skin. Consistency. e.g. firm, soft. Tenderness. Temperature. Mobility. Transillumination. Cystic masses transilluminate. ӹӹ Pulsatility. Indicates a vascular lesion. ӹӹ Relation to surrounding structures. e.g. trachea. ӹӹ Movement. This includes with sticking out the tongue (thyroglossal cyst) and with swallowing (thyroid mass). ӹӹ ӹӹ ӹӹ ӹӹ ӹӹ ӹӹ ӹӹ
Palpating lymph nodes.
Bedside tests include: ӹӹ Review the observation chart. ӹӹ Plot the height and weight on an age and sex appropriate chart.
CASE SCENARIO SCENARIO 10 Andrew is a two-year-old boy who has attended clinic today with a neck swelling. You are asked to examine the mass and present your findings. EXAMINATION CHECKLIST Domain
Findings
Evolving thought process
Global assessment.
• Appears well grown. • No dysmorphic features.
• No pathology identified.
Inspection.
• Mass right side of neck, along border of sternocleidomastoid muscle, 2cm x 1cm, central punctum that appears to be discharging yellowish fluid.
• Not in midline, therefore unlikely to be a thyroglossal cyst or thyroid mass. Appears to be discharging/ possibly infected.
Palpation.
• Smooth, fluctuant mass, slightly tender, no associated lymphadenopathy.
• Could be a branchial cyst.
Special tests.
• Position not altered by swallowing. • Child would not co-operate with sticking tongue out.
• No additional pathology identified.
Auscultation.
• No bruit heard.
• No additional pathology identified.
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NEUROLOGICAL EXAMINATION
PRESENTING YOUR FINDINGS
Andrew appears well and is comfortable at rest. He has a 2 cm x 1 cm swelling on the right side of his neck along the lower anteromedial border of the sternocleidomastoid muscle. The mass has a central punctum, which is discharging yellow fluid. The mass is smooth, fluctuant and slightly tender to palpate. The position does not change upon swallowing and there is no audible bruit. The mass is consistent with a right sided branchial cyst that may be associated with a sinus tract, in view of the discharge. To complete my examination, I would like to plot Andrew’s height and weight on a growth chart. To investigate the mass, I would like to refer Andrew for an ultrasound and surgical review.
NEUROLOGICAL EXAMINATION A neurological assessment of one form or another is merited in most paediatric presentations, both acute and in outpatients. Situations when you might be expected to perform a complete neurological examination include: 1 The child presenting with seizures or syncopal episodes. Do they have an underlying neurological disorder? Is there a space occupying lesion? 2 The child presenting with bladder/bowel disturbance, e.g. chronic constipation, enuresis. 3 Headaches. This may be a presentation of a space occupying lesion. 4 An obvious neurological deficit, e.g. foot drop, balance disturbance. 5 Developmental delay. A key distinction in examining the limbs is whether an upper or lower motor neurone lesion is present (Table 28). This Summary Checklist Global assessment of child on approach. Inspection. Upper limbs, face, torso, back, legs. Upper limbs. Power, tone, reflexes, sensation, coordination, function. Lower limbs. Power, tone, reflexes, sensation, coordination, function. Gait. Walking, tip toeing, running, walking on heels. Cranial nerves. I-XII. Cerebellar examination. Bedside tests. Review observations, plot height, weight and head circumference.
TABLE 28: Distinguishing between upper and lower motor neurone lesions Domain
Upper Motor Neurone Lesion
Lower Motor Neurone Lesion
Inspection.
• Pronator drift.
• Fasciculation (small muscle twitches, involuntary) and muscle wasting.
Tone.
• Increased (may be reduced initially).
• Decreased.
Power.
• Reduced.
• Reduced.
Reflexes.
• Brisk/normal.
• Reduced/absent.
Clonus.
• Yes.
• No.
Plantar reflex.
• Up going.
• Down going.
EXAMINATION
Andrew is a two-year-old boy who has presented to the clinic today with a neck mass.
section will also outline a focused assessment of a child in a wheelchair, and a schematic for examination of a child’s head. Common patients that are likely to appear in exams are shown in Table 29.
TABLE 29: Common patients that may appear in clinical examinations Disease
Possible clues
Duchenne Muscular Dystrophy.
• • • • • • •
Hemiplegic cerebral palsy.
• Arm held flexed on walking, more pronounced on running. • One side of body affected. • Increased tone. • Brisk reflexes.
Diplegic cerebral palsy.
• Scissoring gait. • Increased tone, more in legs than arms. • Brisk reflexes in legs.
Spina bifida.
• • • • • • •
May be in a wheelchair or need a frame to walk. Scar over the lower spine. May have scoliosis. Muscle wasting in legs. Reduced tone and power in lower limbs. Absent reflexes. Presence of a shunt.
Post-viral cerebellitis.
• • • • •
Well child. Wide gait. Romberg positive. Incoordination. Normal reflexes.
Hereditary Sensory and Motor Neuropathy.
• High stepping gait and foot deformities. • Distal muscle wasting ‘inverted champagne bottle’ legs. • Weakness more in legs than arms. • Absent reflexes. • Reduced proprioception.
Male. Waddling gait or walking on tiptoes. Lumbar lordosis. Hypertrophied calves. Gower’s sign. Absent knee jerk but ankle jerk remains present. May be speech delay.
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EXAMINATION
Starting the Examination For all the neurological examinations that follow, the examination starts on entering the room by inspecting the child in their surroundings and making a broad assessment. 1 Are they in a wheelchair? If so, how is it controlled and what supports are needed? A child requiring trunk, neck and head support infers a more severe phenotype. If not, have they walked into the room, and what is their gait like? 2 Is the child comfortable or in pain? Contractures can be very painful, and loss of function can result in the development of pressure sores. 3 Is the child cachectic or well grown? Chronic disease is associated with faltering growth. 4 Are there any devices, such as feeding tubes? The child may have poor swallowing. 5 Is there any obvious scoliosis? An “S” shaped curvature of the spine. In lordosis, the lower spine curves inward and the abdomen may be pushed forward. Kyphosis is the abnormal curvature of the upper spine giving a “hunched over” impression to the posture. 6 Are there any neurocutaneous markers? This includes café au lait spots, ash leaf macules, shagreen patches, neurofibromatosis, and port-wine stains. 7 Are there any obvious scars? e.g. spina bifida surgery. 8 Are there any dysmorphic features?
ӹӹ Neurocutaneous lesions. Neurofibromatosis is associated with café au lait macules. Cutaneous neurofibromas and axillary freckling develop from adolescence onwards. Look at the nails for subungual fibromas seen in tuberous sclerosis (p233). ӹӹ Muscle wasting. This implies lack of use or a peripheral neuropathy. ӹӹ Contractures. This is particularly associated with cerebral palsy. ӹӹ Casts, splints or orthoses. These may be used in cerebral palsy to improve function. ӹӹ Movements. Lack of movement of one arm may indicate hemiplegia. ӹӹ Relative proportion of arms, trunk and legs. In achondroplasia, the limbs are relatively short compared to the trunk. Achondroplasia is also associated with decreased upper arm length (compared to lower arm), and decreased thigh length (compared to lower leg). In children under one: ӹӹ Is there a hand preference? Hand preference under 18 months of age should prompt assessment for hemiplegia. ӹӹ How is coordination? Look how they are playing and reaching for objects (Figure 91).
Tone Check that it will not hurt the child to move their arm. Let them know they should ask you to stop if it hurts. Hold the patient’s hand in a handshake position, and use the other hand to support the elbow taking the weight of the arm. Test tone (Figure 92): ӹӹ Flexing the elbow. ӹӹ Supinating the wrist. ӹӹ With wrist circumduction. Tone may be increased (e.g. spastic cerebral palsy or acquired brain injury), normal, or decreased (e.g. ataxic cerebral palsy or myopathy).
Power Compare left to right, looking for reduced power (Box 7). Aim to test all of the spinal nerve routes and the peripheral nerves (radial, ulnar and median nerves). If the child is non-compliant, get them to do any task involving movement of the arms, and observe. Examination in older children is illustrated in Figure 93-7.
FIGURE 92
FIGURE 91
Ask about the child’s normal function: can they walk unaided? If not, what do they require for support?
Examination of Upper Limbs Inspection Next is a more focused inspection concentrating on the arm but also looking at the patient as a whole. Both arms need to be exposed to compare left to right. Look at the natural movements as well as the resting posture. Look for: ӹӹ Scars. These may be due to contracture release surgery.
Good coordination, good truncal tone, antigravity movement (power at least 3/5), and using both arms equally so no obvious weakness/arm preference.
Testing for tone.
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NEUROLOGICAL EXAMINATION
FIGURE 95
Box 7: Grading of Power 0. Paralysis, no contraction. 2. Can move with gravity excluded. 3. Can move against gravity but not against resistance. 4 . Can move against some resistance but weaker. 5. Normal power.
A
B
Wrist flexion and extension (C6-7), and radial nerve (wrist extension). Get the child to make a fist. The examiner should make a fist, then push down on the child’s fist (extension- A) and then up against it (flexion- B). During these movements, the child should try to resist movement.
EXAMINATION
1. Flicker of contraction.
FIGURE 93
FIGURE 96
A
B
Shoulder abduction (C5) and adduction (C6-8). Ask the child to hold their arms out at 90° to body, elbows bent, like ‘chicken wings’. Ask them to push their arms up (abductionA) and then pull their elbows into their body (adduction- B) against resistance.
Finger abduction (T1/ulnar nerve). Ask the child to spread their fingers. The examiner should try to push them back together, whilst asking the child to resist.
FIGURE 94 FIGURE 97
A
B
Elbow flexion (C5-6) and extension (C7-8). Ask the child to bend their arms up in front of their body. Hold their wrists and ask the child to pull the examiner towards them (flexionA) and then push them away (extension- B).
Thumb abduction (T1/median nerve). Ask the child to place the hand on a surface with the palm facing the ceiling. Ask them to then point their thumb directly to the ceiling, and then whilst pushing it down, ask the child to resist.
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EXAMINATION
Additional features may be noted if there is a suspected peripheral nerve injury (Figure 98-100).
FIGURE 100
FIGURE 101
FIGURE 98
A
Radial nerve palsy. Wrist drop.
FIGURE 99
Median nerve palsy “Hand of Benedict”. This only occurs on trying to make a fist. There is full flexion of the 4th and 5th MCP joints. However, the index and middle finger cannot flex at the PIP, DIP or MCP joints due to loss of function of the lateral two lumbricals.
In children under one: ӹӹ Pull infant from lying to sitting using their arms. One hand should support the head, the other hold the arms. ӹӹ Grasp. Assess this by asking the child to grab something, e.g. a finger. ӹӹ Place the child on their front; can they lift themselves up? Do they support their head when doing this? Do they lift themselves up onto their forearms or onto their hands?
B Biceps reflex (C5-6). With the arm bent and relaxed (e.g. across the lap), place a finger or thumb on the biceps tendon and strike it with the tendon hammer. Observe for contraction of the biceps muscle and/or elbow flexion. This is shown with (B) and without (A) reinforcement by clenching the teeth. FIGURE 102
A
Reflexes A
B Ulnar nerve palsy “claw hand.” At rest (A) and when trying to make fist (B). Note that both pictures show full extension of the 4th and 5th MCPs. These joints are not able to flex due to loss of function of the medial two lumbricals.
The child must be relaxed to elicit reflexes. Arm reflexes are shown in Figure 101-4. First explain the test, and demonstrate it, showing that the tendon hammer is not painful. If it is difficult to elicit the reflex, ask the child to clench their teeth, screw up their eyes or count backwards from 100. In patients under one-year-old, the above can all be attempted, as well as assessing for the presence of primitive reflexes (Table 30): ӹӹ In an age appropriate infant, asymmetry may indicate a problem with that limb, e.g. an asymmetrical Moro reflex could be due to a brachial plexus injury sustained at birth (Erb’s palsy). The affected arm will not move.
B
C Triceps reflex (C6-7). Several techniques to elict this are shown, as it is often difficult. Observe for contraction of the triceps muscle, and/or elbow extension. A. With the child’s arm bent, supporting the distal arm. B. Letting the arm dangle at the elbow. C. Supporting the forearm and the upper arm.
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NEUROLOGICAL EXAMINATION
FIGURE 103
Reflex
What is it?
Abnormal after..
Moro.
• Sudden movement of baby’s head downwards results in limb extension then flexion.
• 3 months.
Asymmetrical tonic neck reflex.
• With the baby lying on their back, and the head to one side, two things happen. • The arm and leg, on the side the head is turned towards, extend. The opposite arm and leg flex.
• 6 months.
Grasp.
• Place a finger in the palm of the baby’s hand. • They will grasp it, increasing their grip if you try to pull away.
• 3 months.
Stepping.
• Hold the baby upright, with their feet touching a firm surface. They will imitate walking.
• 4 months. • This reappears at about one year of age.
EXAMINATION
Supinator reflex (C5-6) Rest the child’s arm on their lap. Strike the arm directly above the wrist on the radial side, proximal to the styloid process. Observe for contraction of the brachioradialis, flexion of the elbow, supination of the forearm, and/or flexion of the fingers.
TABLE 30: Examples of primitive reflexes
FIGURE 107
FIGURE 105
FIGURE 104
A
B
Pronator drift. A. Ask the child to put both their arms out, palm up (supinated), as if they were holding a pizza. Ask them to close their eyes and count to 10, keeping their arms in place. B. In a positive test, the affected arm will pronate and fall. Getting help from a mother, and working with a younger child to elicit the biceps reflex.
FIGURE 106
ӹӹ Presence of primitive reflexes beyond a certain age is abnormal and could indicate developmental delay.
Pronator Drift Pronator drift tests for upper motor neurone lesions (Figure 105). This is because upper motor neurone lesions cause a relatively greater weakness in supination compared to pronation. Cerebellar lesions can also cause this sign.
Co-ordination See cerebellar exam for more detail (p591): ӹӹ Past pointing (dysmetria) and intention tremor (Figure 106). ӹӹ Dysdiadochokinesis (Figure 107).
A
B
A
B Finger-nose testing. Ask the child to touch the tip of their nose with their finger (A), and then to touch the examiner’s finger (B). The examiner’s finger should be held at full arm’s length from them and moved around as the exercise is repeated.
C Ask the child to place one hand, palm up, with the other hand on top of it (A). Then ask them to rapidly lift it up (B) and down (C) from the palm, alternating between pronation and supination.
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EXAMINATION
In children under one: ӹӹ Observe play and reaching for objects.
FIGURE 109 C4
Motor Function
T1 T2
Test function, e.g. grip strength (Figure 108), doing up a button, writing, or taking off clothes.
T3 T4 T5 T6 T7 T8 T9 T10 T11 T12
C5
FIGURE 108
C6 C7
Grip strength.
Sensation Light Touch, Pain, and Temperature (Spinothalamic Tract) Lesions in the spinothalamic tract manifest as symptoms on the contralateral side of the body. ӹӹ Light touch. Use a piece of cotton wool to test the dermatomes of the arm, comparing left to right (Figure 109). Use the upper sternum as a baseline. Do not test in order, and vary the time between each test, so that the child cannot anticipate the next test. Ask the child if it feels the same on both sides. Test the anatomical snuff box (base of dorsum of thumb) – this ensures the radial nerve sensory route is tested. Ensure that when testing C7, it is over lateral palm – median nerve, and when testing C8, it is over the medial palm or little finger – ulnar nerve (Figure 110). ӹӹ Pain. This is assessed using a neurotip. It is better than light touch at isolating the spinothalamic tract, as light touch fibres are also carried by the dorsal column. ӹӹ Temperature. This involves using hot/cold markers (e.g. test tubes filled with water) to assess hot and cold sensation in the same dermatomes. Not routinely done.
C8
C5
C6 C8
C7
Dermatomes of the upper limbs and torso.
FIGURE 110 Radial Nerve Ulnar Nerve Median Nerve
Sensory components of radial, ulnar and median nerves.
to close their eyes, and say when the vibration stops. If they cannot identify this, repeat it at the elbow. These tests are summarised in Figure 111.
Examination of Legs
Proprioception and Vibration Testing (Dorsal Column)
Inspection
Lesions in the dorsal column manifest as symptoms on the ipsilateral side of the body as the lesion. ӹӹ Proprioception. Hold the index finger on either side of the distal interphalangeal joint. First, with the child’s eye open, demonstrate to them upwards and downwards movement of the distal part of the finger. Remember to hold the finger on either side to move it, rather than using the finger pad. Then ask the child to close their eyes and identify whether you are moving their finger up or down. If proprioception is not intact at the finger, test it at the distal phalanx, and then the wrist, and then the elbow. ӹӹ Vibration sensing. Vibrate a tuning fork, and place it on the bony prominence at the wrist (ulnar styloid). Ask the child
The child’s legs will need to be fully exposed. Look from front and back, standing and lying: ӹӹ Scars. These may be from contracture release, or a muscle biopsy. ӹӹ Neurocutaneous lesions. ӹӹ Muscle wasting. This implies lack of use. ӹӹ Contractures. These are particularly associated with cerebral palsy. ӹӹ Casts, splints or orthoses. Such devices may be used in cerebral palsy to improve function, e.g. ankle-foot orthoses. Shoes may have special soles/supports. ӹӹ Movements. Lack of movement of one arm may indicate a hemiplegia.
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NEUROLOGICAL EXAMINATION
FIGURE 111
B
C
D
E
F
EXAMINATION
A
Sensation testing, whilst patient’s eyes are shut. A. Light touch with cotton wool. B. Pain with a neurotip. C. Vibration sense with a tuning fork, vibrating. D. Vibration sense with a tuning fork, stopping the vibration. E. Proprioception in the ‘up’ position. F. Proprioception in the ‘down’ position.
ӹӹ Hypertrophied calves. This is classically seen in Duchenne Muscular Dystrophy ӹӹ Relative proportion of arms, trunk and legs. In achondroplasia, the limbs are relatively short compared to the trunk and the thighs are shorter in comparison to the lower leg.
Tone Ensure the child is pain free and can move their legs without causing pain. Ask them to report any pain. Fix the hip joints
when assessing tone if possible. Ask the child to lie on the examination couch on their back. Perform manoeuvres such as (Figure 112): ӹӹ Roll the leg from side to side. ӹӹ Abruptly lift the knee. The ankle will come off the couch in hypertonia. ӹӹ Passively flex and extend the knee. Tone may be increased (e.g. spastic cerebral palsy or acquired brain injury), normal, or decreased (e.g. myopathy).
FIGURE 112
A
B
Assessing tone A. Rolling the leg. B. Abruptly lifting the knee.
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EXAMINATION
Power Compare left to right, looking for reduced power (Box 7). Aim to test all the spinal nerve routes, as well as the peripheral nerves (Table 31), although they are more difficult to isolate than with the upper limbs. If the child is non-compliant, get them to do any task involving movement of the legs, and observe. Tests are shown in Figure 113-6. The examiner should oppose each movement accordingly.
FIGURE 113
A
B
A. With the child lying flat on the bed, they should be asked to lift their leg straight up, whilst the examiner resists it (hip flexion L1-3). B. Then, from this position, they should be asked to push their leg back onto the bed, with the examiner’s hand now underneath the thigh, trying to resist movement (hip extension L4-S1).
FIGURE 114
A
B
Ask the child to bend their leg. A. Ask them to pull their heel into the bottom (knee flexion L5-S1). B. Ask them to then kick out as if kicking a football (knee extension L3-4).
FIGURE 115
A
FIGURE 116
B
A. Ask the child to point their foot towards their head (left ankle dorsiflexion L4-5) against resistance. B. Then ask them to point it towards the floor (plantar flexion S1-2), against resistance.
Ask the child to extend the big toe against resistance (extension of big toe (L5)).
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NEUROLOGICAL EXAMINATION
In children under one: ӹӹ Pull to stand; can they support their weight?
FIGURE 119
These are described in Figure 117-21. As for arms, the child must be relaxed. If difficult, reinforce the reflex by asking the child to grip their fingers together and try to pull them apart (Figure 122). FIGURE 117 A
B Getting help from a mother, and working with a younger child to elicit knee reflex.
EXAMINATION
Reflexes
FIGURE 120
C
Knee jerk (L3-4). The leg will kick out if the reflex is present, or alternatively contractions may be just visible in the quadriceps muscle. In a toddler, where the tendon is more difficult to isolate, place a finger in the area and strike this instead. Several techniques shown: A. Roll one arm over the one leg and under the other. B. With the legs crossed over sat in a chair. C. Lift up one leg at the knee, taking its entire weight.
Plantar reflex. Warn the child that this may tickle. Run a finger nail from the heel, up the outside of the sole of the foot to the little toe, and then across from the base of the little toe to the base of the big toe. Observe for flexion of the toes. Extension of the big toe is consistent with an upper motor neurone lesion. It is normal in children less than 1-year-old.
FIGURE 118 A
C FIGURE 121
B
Ankle jerk (S1). Hold the foot with one hand, slightly dorsiflexed. Observe for downward movement of the foot and contraction of the gastocnemuis muscle. Several positions are shown: A. The knee is bent and the hip externally rotated. B. As above, but the sole of the foot is struck rather than the tendon directly. C. Kneeling on chair.
Ankle clonus. Rapidly dorsiflex the foot (upwards). On stopping, there may be involuntary upwards beats of the foot, which is called clonus. Although a few beats of clonus may be normal, particularly in children under oneyear-old, sustained clonus suggests an upper motor neurone lesion.
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EXAMINATION
FIGURE 122
ӹӹ Vibration sensing. Vibrate a tuning fork and place it on the big toe, and perform this test as shown for the arm. If they cannot identify this, repeat on the malleolus, and then the tibial shaft, the tibial tuberosity, and finally the anterior iliac crest. Sensation tests are summarised in Figure 124. Peripheral nerves are less commonly damaged in the lower limbs than the upper limbs. However things to look out for are shown in Table 31.
Reinforcement.
In under 1: ӹӹ Primitive reflexes as described in Table 30.
Sensation Light Touch, Pain, and Temperature (Spinothalamic Tract) ӹӹ Light touch, pain and temperature are all tested as described in the arms section. Dermatomes for the legs are shown in Figure 123.
Proprioception and Vibration Testing (Dorsal Column) ӹӹ Proprioception. Hold the big toe either side of the interphalangeal joint, and perform this test as shown with the arms. If proprioception is not intact at the interphalangeal joint, test it at the carpometarsal joint, then the ankle, and then the knee. A
FIGURE 123
L1 L2 L3 L4
L3 L4 S2 S3 S3 S1 S2 L5
L2 S4 S5 L1 L2 L3
L5 S1
L5
L4
Dermatomes of the leg.
Co-ordination ӹӹ Heel-shin test. Figure 125.
TABLE 31: Peripheral nerves of the lower limb Nerve
Motor loss
Sensory loss
Obturator nerve (L2-4).
• Hip adduction.
• Medial thigh.
Femoral nerve (L2-4).
• Hip flexion. • Knee extension.
• Anteromedial thigh. • Medial lower leg, to medial malleolus.
Common peroneal nerve (L4-S3).
• Foot dorsiflexion loss gives characteristic foot drop. • Foot eversion loss gives inverted posture.
• Anterolateral lower leg. • Dorsum of foot/toes.
Sciatic nerve (L4-S3).
• Paralysis of all muscles below knee- gives foot drop from weight of foot (via tibial and common fibular branches).
• Posterolateral/anterolateral lower leg. Plantar surface of foot (tibial branch). • Lateral lower leg, and dorsal foot (common fibular branch).
B
FIGURE 124 Sensation testing, whilst the patient’s eyes are shut. A. Light touch with cotton wool. B. Pain with a neurotip. C. Vibration sense with a tuning fork, vibrating. D. Vibration sense with a tuning fork, stopping the vibration. E. Proprioception in the “down” position. F. Proprioception in the “up” position.
C
D
E
F
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NEUROLOGICAL EXAMINATION
FIGURE 125
FIGURE 126
A
EXAMINATION
B
Gower’s sign.
FIGURE 127 C
Demonstrate this first. With the child lying down, lift their heel up and onto their other leg, just below the knee (A), run it down the shin (B) and then bring it back to the start (C) and repeat. A
Gower’s Sign Ask the child to lie on the floor on their back. Then ask them to stand up without using their hands, if possible. If the child turns first onto their front and then rises from the floor by crawling up their legs, this is Gower’s sign. It indicates proximal muscle weakness such as in Duchenne Muscular Dystrophy (Figure 126). In children under one: ӹӹ Observe age appropriate activities e.g. bringing feet to mouth and crawling. ӹӹ Ensure the movements are smooth and equal on both sides. Finally assess function of the legs. This is usually done by observing walking/running.
C
D
E
Going from left to right: A. Walk unaided. Assess movement of legs, arms, and trunk. Then ask the child to walk quickly and then run. This will make a subtle hemiplegia more pronounced, with flexion of the arm on the affected side more obvious. B. Walk on tiptoes. Again this will make a hemiplegia more pronounced, as well as testing plantar flexion and muscle strength. C. Walk on heels. This will exaggerate any difficulty in dorsiflexion. D. Heel-toe walk. Ask the child to walk as if on a tight rope, with the one foot in front of the other. Show them first and stay with them as they perform it, in case they fall. A child with ataxia will find this exercise much more difficult. E. Walk on the outside of their feet (Fogg test). This will make a hemiplegic gait more pronounced.
TABLE 32: Different types of gait Types of Gait Signs
Cause
Foot drop “high stepping” neuropathic gait.
• The leg on the side affected has to be lifted high to avoid the foot dragging along the floor.
• Common peroneal nerve palsy. • L5 radiculopathy. • Peripheral mono/poly neuropathy.
Waddling gait.
• The pelvis drops on the contralateral side to the leg being lifted (Trendelenburg sign). • The abdomen is pushed forward due to lumbar lordosis.
• Congenital hip dislocation. • Proximal myopathy, e.g. Duchenne muscular dystrophy.
Dyskinetic gait.
• Irregular, jerky, involuntary movements.
• Cerebral palsy. • Basal ganglia lesion.
Stomping gait.
• The feet are slammed onto the floor, and sometimes lifted high up to achieve this. • This is made worse in the dark (since visual compensation cannot be applied).
• Loss of proprioception, e.g. B12/folate deficiency.
Ataxic gait.
• Unsteady. • Broad based. • Difficulty heel-toe walking.
• Cerebellar cause, e.g. post viral cerebellitis.
Examination of Gait It is crucial that this is done knowing what the child is capable of. Ensure the child is supported so that if they fall, they may be caught. The child should be asked to move in the manners demonstrated in Figure 127. Different types of gait are shown in Table 32. If there is an ataxic gait, use Romberg’s test to help differentiate between cerebellar and sensory ataxia (Figure 128).
B
Continued
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EXAMINATION
TABLE 32: Continued Types of Gait
Signs
Cause
Spastic hemiplegia.
• The arm is held flexed, adducted, and is internally rotated, with the hand held in a fist. • The leg is in extension, stiff, and in plantar flexion. • The leg is dragged in circumduction (in a semi-circle).
Spastic diplegia.
• Tightness of adductors causes the knees to come together/legs to cross at midline, giving a “scissoring gait”. • Dragging of both legs. • Narrow base. • Circumduction on both sides.
• Cerebral palsy. • Acquired brain lesions, e.g. stroke.
FIGURE 128 A
B
Romberg’s test. A. The patient stands, feet together, eyes open, and hands by their side. B. The patient closes their eyes, and is observed for one minute whilst the examiner looks for loss of balance and swaying.
ӹӹ Dysmorphic facies. Rare syndromes such as Goldernhar syndrome are associated with both dysmorphic features (incomplete development of the ear, nose, soft palate, lip and mandible), and cranial nerve palsies (e.g. VII and VIII). ӹӹ Eyes. Look for presence of glasses, and any obvious squint. ӹӹ Look for possible signs of cranial nerve abnormalities. ›› Ptosis (III). ›› Eye/pupil position (III, IV, VI). ›› Pupil symmetry (II, III). ›› Facial symmetry (VII). ›› Speech (V, VII, IX, X, XII). ›› Sternocleidomastoid/trapezius bulk (XI). The cranial nerves can either be tested in order, or by flow of movement down the face, e.g. after CN VII, test CN XII sticking their tongue out, then move down to test CN XI by turning their head.
CN I (Olfactory Nerve) In cerebellar ataxia, vision cannot compensate for the ataxia, but in sensory ataxia it can. Therefore, with cerebellar ataxia, there will be difficulty with standing even with eyes open, but in a sensory ataxia, the loss of balance will not become evident until the eyes are closed. The test is positive if the child can stand normally with their eyes open, but loses balance on closing their eyes.
Grading Motor Ability The General Motor Function Classification System (GMFCS) is a grading system from 1-5 used to classify motor ability in children with four limb motor disorders (the majority of which are cerebral palsy) (Table 33). It distinguishes the need for different assist devices and is therefore a comment on quality of movement. It can also have some prognostic use as older children are unlikely to change their GMFCS.
Cranial Nerve Examination Inspection A lot can be achieved in the cranial nerve assessment from general observation:
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ӹӹ Generally assessed from history rather than formal examination. ӹӹ Ask about smell, and test if necessary, e.g. with coffee, although ideally standardised smelling salts should be used. TABLE 33: General Motor Function Classification System GMFCS grade
Description of function
I.
No restriction in walking but may have difficulties with coordination or balance.
II.
No devices needed when walking, may need a rail for stairs and find outdoor terrain more tricky, running difficult.
III.
Needs a mobility device such as crutches, may be able to use a manual wheelchair.
IV.
Uses wheelchair most of the time, wheelchair is controlled by the user.
V.
Severely limited mobility, including of head and neck, even when supporting aids are used, e.g. wheelchair bound with head support.
(Adapted from Palisano et al. (1997) Dev Med Child Neurol 39:214-23 CanChild: www.canchild.ca).
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NEUROLOGICAL EXAMINATION
CN II (Optic Nerve) Acuity
Visual Fields The technique for this is shown in Figure 129. FIGURE 129
Fundoscopy ӹӹ Red reflex. This is lost in the presence of cataracts, or white if there is a retinoblastoma (Figure 71). ӹӹ Optic disc. A pink disc with clear margins should be seen. Blurred margins are seen in papilloedema, which is associated with raised intracranial pressure (Figure 72).
Reflexes ӹӹ Test for accommodation (Figure 130) and pupillary reflexes (Figure 131). ӹӹ These both rely on the optic nerve for sensory input, and the oculomotor nerve for the motor limb.
EXAMINATION
ӹӹ Check that the child can actually see with both eyes first. ӹӹ If the child is wearing glasses, test vision with the glasses on. ӹӹ Use a Snellen chart if available; otherwise ask them to read something in the room, e.g. a book or a sign on the wall.
medial to lateral visual fields (i.e. use the right hand to wiggle for the left lateral visual field, and the left hand to wiggle for the left medial visual field) so that the arm does not cross the field of vision.
FIGURE 130
A
B
Accommodation tested using a pencil. A. Hold a finger or an object (e.g. pencil) in front of the child’s face. Ask them to look off into the distance, and then suddenly focus on the pencil. B. The pupils should constrict and converge.
Visual fields testing.
FIGURE 131 A
ӹӹ The examiner should sit opposite the child 50 cm-1 m away, and ask the child to cover one of their eyes using the palm of their hand. At the same time, the examiner covers their opposite eye (i.e. the examiner covers their right eye if the child is covering their left eye). ӹӹ Whilst the child looks at the examiner’s nose, the examiner moves a wiggling finger inwards from outside of their visual field from the upper right corner, lower right corner, upper left corner and lower left corner: this tests all four quadrants. It is crucial that the examiner’s finger starts outside of their visual field: this is confrontational testing, so if the finger is outside of the examiner’s visual field (which is assumed to be normal) it should be outside of the child’s visual field as well. ӹӹ Ask the child to “yes” when they can see the finger. The examiner should swap hands when moving from
B
Pupillary reflexes. Shine a pen torch in each eye (A) and observe the effect (B). Look for constriction of the pupil (direct reflex) and then constriction of the opposite pupil (indirect reflex). Shine the pen torch in each eye twice, once for the direct reflex, once for the indirect reflex.
CN III (Oculomotor Nerve), CN IV (Trochlear Nerve) and CN VI (Abducens Nerve) ӹӹ This can be remembered as the “H test”. ›› Sit in front of the child, resting one hand on their head or chin. The examiner should place their finger 50cm from the child’s face, asking them to keep their head still and follow the finger with their eyes only (Figure 132-3). ›› The examiner should move their finger slowly in the shape of an “H”, forming a horizontal line across the visual field and two vertical lines at the periphery (Figure 134). This will demonstrate the full spectrum of extraocular eye movements (Figure 135). For completeness, it is useful to ask the child to look directly up and directly down as well. ӹӹ Look for the position of the pupil and any nystagmus. Ask the patient if they have any pain or any double vision at any point. Note: ›› The position of the pupil. ›› Is there a delay in or incomplete movement indicating a palsy? ›› Is there any double vision? At extremes of vision this is normal. ›› Are the eye movements painful? ›› Is there any nystagmus?
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EXAMINATION
FIGURE 132
FIGURE 135
Eye movement assessment. Assessing eye movements looking in eight directions. FIGURE 133 A FIGURE 136
B
FIGURE 138
Example of a right sided 6th nerve palsy (patient asked to look right).
FIGURE 137
Adapting to a younger child. Using toys and mum to help elicit clinical signs.
FIGURE 134 Inferior oblique
Superior rectus Up
Medial rectus
G a z e
Lateral rectus
Example of a right 3rd nerve palsy: lid lag, pupil down and out. Older children will complain of diplopia. In a complete 3rd nerve palsy, the pupil will be dilated and not react to light. It can be congenital or acquired due to infection, trauma, space occupying lesion or hypertension.
Down Superior oblique
Inferior rectus
Eye movements.
Examples of third and sixth nerve palsy are shown in Figure 136 and 137.
CN V (Trigeminal Nerve) ӹӹ Sensory division. Test each of the trigeminal nerve divisions: ophthalmic, maxillary and mandibular, i.e. forehead, cheeks, jaw.
Test left and right but do not go in an obvious order. Then ask if it felt the same on both sides (Figure 138). ӹӹ Motor division. Ask the child to clench their jaw. Palpate the side of the face (masseter muscle) and the temples (temporalis muscle), looking for loss of muscle bulk/strength (Figure 139). ӹӹ Jaw jerk reflex. This is not routinely tested but should be offered (Figure 140).
Sensation to the V1 division of trigeminal nerve. 1. With the child’s eyes closed, gently touch their face with cotton wool in each division of the trigeminal nerve. 2. Ask them to say “yes” when they can feel it.
ӹӹ Corneal reflex. Involuntary blinking of the eyes when the cornea is stimulated. The efferent limb (i.e. the blinking) occurs as a result of facial nerve activity. This is not routinely tested (Figure 141).
CN VII (Facial Nerve) ӹӹ Test each individual branch, as shown in Figure 142. A lower motor neurone lesion, e.g. Bell’s palsy, will affect all of the face. An upper motor neurone lesion, e.g. cerebral palsy, will affect the lower face only; i.e. the forehead is spared.
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NEUROLOGICAL EXAMINATION
FIGURE 139 A
FIGURE 143 B
EXAMINATION
Clenching teeth and testing: A. Masseter (left). B. Temporalis (right).
FIGURE 140
ӹӹ Test the taste supply to the anterior 2/3 of the tongue. It can be tested using salt/sugar on the lateral side of the tongue, with the mouth rinsed in between. This is not routinely tested. ӹӹ Examine the ears for vesicles if there is a facial palsy. This is associated with Ramsey-Hunt syndrome, which is Herpes Zoster reactivation in the facial nerve.
CN VIII (Vestibulocochlear Nerve) ӹӹ Test hearing. Basic props may be used for crude testing of hearing in younger children (Figure 143-4). Jaw jerk. With the mouth slightly open, tap the space between the chin and the lower lip with a tendon hammer. This should result in masseter contraction.
Testing hearing. Cover the patient’s eyes with one hand (so they cannot lip read), and occlude the ear not being tested (by pushing on the tragus, and rubbing the fingers together over that ear to create white noise). Whisper a number in the ear being tested. Get increasingly louder until the child can hear the number.
ӹӹ Rinne’s and Weber’s tests. These can be performed in older children. This differentiates between conductive and sensorineural deafness and requires a 512 Hz tuning fork. Activate the tuning fork first by tapping it on a hard surface/knee. Subsequent steps are shown in Figure 145. ӹӹ Assess balance. This is not routinely tested.
FIGURE 142 A
B
C
D
E
F
FIGURE 141 A
B
Corneal reflex. Touch the cornea with cotton wool (A) and look for blinking (B).
Facial nerve testing. From left to right, ask the child to: A. Raise their eyebrows (temporal branch). B. Close their eyes tightly, and then as the examiner, try and open them (zygomatic branch). C. Blow their cheeks out (buccal branch). D. Whistle (mandibular branch). E. Bare their teeth (mandibular branch). F. Stick forward their chin as if shaving (cervical branch).
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EXAMINATION
FIGURE 144
FIGURE 146 B
A
A C
B Crude hearing test. A. Use keys to make a sound, making sure the child cannot see them initially. B. Positive test if the child turns to the sound.
CN IX (Glossopharyngeal Nerve) and CN X (Vagus Nerve) ӹӹ Gag reflex. Gently check for sensation in the posterior pharyngeal wall on both sides. Then stick the tongue depressor into the back of the throat to elicit a gag reflex. Not usually tested.
Asking the patient to: A. Cough. B. Swallow water. C. Say “ahh” to look in the mouth.
ӹӹ Swallowing water. IX is the sensory limb, X is the motor limb (Figure 146). ӹӹ Cough. Not a very sensitive test, as multiple factors affect cough, e.g. respiratory muscle strength, but tests vagal nerve function as well (Figure 146). ӹӹ Opening the mouth and saying “aaahh”. Look for uvula deviation, and symmetrical rising of the soft palate (both
FIGURE 145 A
B
C
A. Weber’s test. Tap the tuning fork, and place the base at the centre of the forehead. Ask the patient if they hear it loudest in the middle (normal), to the left, or the right. In a conductive deafness, Weber’s test is louder in the affected ear, but in sensorineural deafness, it is loudest in the unaffected ear. B/C. Rinne’s test. Tap the tuning fork, and place it on the bony mastoid process behind the ear (B), and then over the ear (C). In normal hearing and sensorineural deafness, conduction over the ear (air conduction) is better, whereas bony conduction is better in conductive deafness.
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NEUROLOGICAL EXAMINATION
testing vagus nerve function). Deviation to the right suggests paralysis on the left (Figure 146).
FIGURE 148 A
B
EXAMINATION
CN XI (Accessory Nerve) ӹӹ Shrugging and turning head against resistance. This is tested as shown in Figure 147.
CN XII (Hypoglossal Nerve) ӹӹ Tongue inspection. Look for tongue wasting and fasciculation while the mouth is open and the tongue is still in the mouth (relaxed). Ask the child to stick out their tongue. Look for any deviation (Figure 148). ӹӹ Tongue movement. Get the child to move their tongue from side-to-side, and look for any weakness. Ask them to push the tongue into their cheeks (Figure 149).
Inspection of the tongue sticking out and in the mouth.
FIGURE 147 A
FIGURE 149 B
A. Ask the child to shrug their shoulders against resistance. This will show any weakness in the trapezius muscle. B. Ask the child to turn their head against your hand. This will show any weakness in the sternocleidomastoid muscle.
Tongue pushed into the cheek to assess power.
Cerebellar Examination
General Inspection
Cerebellar examination would be required in the child who appears to have “lost their balance”. The commonest reason for this is a post-viral cerebellitis, often post-varicella infection, but it is important to look for signs of other causes such as inherited ataxias or a space occupying lesion. A helpful mnemonic for cerebellar signs is “DANISH Pastry”: D - dysdiadochokinesia A - ataxia N - nystagmus I - intention tremor S - slurred/staccato speech H - hypotonia P - past-pointing
ӹӹ Walking aids. These may suggest gait abnormalities. ӹӹ Posture and baseline movements. There may be truncal ataxia and tremor. ӹӹ Look for clues suggesting an underlying aetiology. ›› Chronic liver disease, e.g. spider naevi, palmar erythema. ›› Alcohol intoxication. ›› Ataxic telangiectasia. Look for telangiectasia, particularly on the face/eyes.
Eye Signs ӹӹ Extraocular muscle movements. Use H testing (Figure 1325). Get the child to hold their gaze in the lateral and vertical positions to try and illicit nystagmus.
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EXAMINATION
ӹӹ Rapid eye movements. Not routinely done, but may pick up nystagmus (Figure 70).
Speech ӹӹ Dysarthria. Slurring of speech can be identified by saying phrases such as “baby hippopotamus” or “British constitution.” Staccato speech will be apparent, meaning each sound/note is sharply detached from the others.
Upper Limb Examination ӹӹ Tone. Hypotonia is associated with a cerebellar lesion. ӹӹ Power. Normally not significantly affected, but poor coordination can impair this. ӹӹ Reflexes. There may be diminished reflexes in Friedreich’s ataxia. ӹӹ Intention Tremor. Ask the child to pick up an object. A tremor may become obvious on “intention” or “movement”. ӹӹ Pronator drift. (Figure 105). ӹӹ Cerebellar rebound. (Figure 150). ӹӹ Past pointing (dysmetria) and intention tremor. (Figure 106). ӹӹ Dysdiadochokinesis. (Figure 107).
FIGURE 150
FIGURE 152 Head shapes.
ӹӹ Tone. Hypotonia is associated with a cerebellar lesion. ӹӹ Power. Power is not normally significantly affected. ӹӹ Reflexes. There may be diminished reflexes in Friedreich’s ataxia, along with an upgoing plantar reflex. ӹӹ Heel-shin test. (Figure 125).
Gait ӹӹ Ataxic gait. An ataxic gait with a broad base, and difficulty balancing, particularly on heel-toe walking is seen. ӹӹ Romberg’s test. Romberg’s test is positive in sensory ataxia, but it is negative in cerebellar ataxia (Figure 128).
Examination of the Head Head examination is not routinely done in older children, but is more relevant to babies/toddlers and those that have undergone surgical procedures.
Inspection ӹӹ Head size. (Figure 151). ӹӹ Head shape. (Figure 152). ӹӹ Dysmorphic features.
FIGURE 151
Cerebellar Rebound. With the patient’s hands held out in front of them, press one arm down and then let go. Ask the child to try and put the arm back in the original position. In cerebellar rebound, this is very difficult, and the arm “overshoots” on correction.
Normal
Lower Limb Examination
Plagiocephaly
Plagiocephaly. An asymmetric and oblique deformity of the head.
Measuring head circumference. Measure with a tape measure, going around the occiput, and above the eyebrows.
Brachycephaly
Brachycephaly. Short wide head, with flattened occiput, i.e. a relatively short anteroposterior diameter.
Dolichocephaly
Dolichocephaly. Disproportionately long anteroposterior diameter.
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NEUROLOGICAL EXAMINATION
The Child in the Wheelchair
ӹӹ Palpate the sutures and the fontanelles (Figure 153). Fusion of the sutures (craniosynostosis) may occur prematurely. It is usually isolated, but it may occur as part of a syndrome, e.g. Apert syndrome or Crouzon syndrome. ӹӹ Fontanelles can be: ›› Sunken. This is most commonly due to dehydration. ›› Bulging. This is associated with raised intracranial pressure, e.g. meningitis, hydrocephalus or crying. ›› Delayed closure. e.g. congenital hypothyroidism, trisomy 21, rickets. ›› Premature closure. This is associated with craniosynostosis and microcephaly. The posterior fontanelle usually closes before the child is twomonths-old. The anterior fontanelle between 4 and 26-months-old. ӹӹ Palpate for any surgical procedures. ›› Ventriculoperitoneal (VP) shunt. This is usually a U shaped incision, 3 cm posterior/superior to the pinna of the ear, but it may vary depending on age. The catheter is palpable going to the abdomen/thorax. Look for an associated scar in the abdomen/thorax as well. The abdominal site is usually a vertical incision at the right subcostal margin.
A child may need to be examined whilst in a wheelchair. Most information can be deduced without putting the child onto an examination couch. The other neurological examinations or slightly adapted versions of them can be performed on the child who is wheelchair bound.
FIGURE 153
Inspection Wheelchair ӹӹ Type of wheelchair (Figure 24). ›› Manual self-propelled. This needs good strength and co-ordination in upper body for operation. ›› Electric power wheelchairs. There are two broad types: ӽӽ Controlled via a hand device, e.g. joystick. This requires relatively good strength in hands, and reasonable hand-eye co-ordination. ӽӽ Controlled by a headswitch, chin joystick or breath assisted controls. This indicates a severe motor deficit, as the child cannot use their hands to control the joystick. ӹӹ Wedges or braces used to support, and if so, where? Truncal, neck and head supports indicate hypotonia; e.g. a child with cerebral palsy may be hypertonic in their arms and legs but have truncal hypotonia requiring a brace. Duchenne Muscular Dystrophy patients may require spinal support.
EXAMINATION
Palpation
Face and Head ӹӹ Head size. e.g. a child with macrocephaly may have hydrocephalus, particularly if a shunt is present. ӹӹ Shunts. VP shunt in hydrocephalus. ӹӹ Dysmorphic facies. ӹӹ Obvious nystagmus or squint.
Arms ӹӹ Tremor. ӹӹ Abnormal posturing. ӹӹ Dystonic movements. e.g. chorea, athetosis.
A Posterior fontanelle
Lambdoidal suture
Parietal bones
Sagittal suture Coronal suture
Anterior fontanelle B
Frontal bones
Metopic suture
A. Palpating the skull sutures and fontanelles in an infant. Keep the head well supported while palpating and employ the parent’s assistance if needed. B. The underlying anatomical landmarks.
Abdomen ӹӹ Scars. These can be seen from VP shunt placement. ӹӹ Gastrostomy or jejunostomy. These may either be in situ or scars may be visible from removal. ӹӹ Urinary catheter in place. This is common in spina bifida.
Palpation Head ӹӹ Head circumference. ӹӹ Shunts. Palpate for any shunts or reservoirs.
Arms/Legs ӹӹ Tone and power can be grossly assessed by holding the child’s hands/feets. ӹӹ Reflexes can also be assessed in the wheelchair.
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EXAMINATION
Completing the Examination ӹӹ Perform a general paediatric examination. ӹӹ If the child is in a wheelchair, ideally perform a full neurological examination of the patient’s arms and legs with the patient out of the wheelchair and on the bed.
Bedside tests include: ӹӹ Review the observation chart. ӹӹ Plot the height and weight on an age and sex appropriate chart.
PRESENTING YOUR FINDINGS
CASE SCENARIOS
Joe is a 7-year-old boy who I examined in clinic today.
SCENARIO 11 Joe is a 7-year-old boy who has come to the outpatient clinic for review. He was born prematurely and has been regularly seen in the clinic. Please examine his lower limbs.
EXAMINATION CHECKLIST Domain
Findings
Evolving thought process
Global assessment of child on approach.
• Alert, looks well, no supports.
• Not acutely unwell.
Inspection.
• Walks with legs turned inwards, more on tiptoes. • Scar over Achilles. • Loss of muscle bulk in the legs.
• Appears to have a diplegic gait. • May have had tendon release surgery. • Pathology likely to be chronic.
Tone.
• Increased.
Power.
• 4/5 throughout.
• Likely upper motor neurone lesion, but with good function.
Reflexes.
• Brisk, with upgoing plantars.
Pronator drift.
• Positive.
Coordination.
• Good.
Function.
• Good.
Sensation.
• Normal to light touch, and joint position sense.
• Isolated motor pathology.
He appears well and comfortable at rest. He has no obvious dysmorphic facies and no mobility aids. Joe has a diplegic gait. He has scars over his Achilles, consistent with tendon release surgery. Joe has signs of an upper motor neurone lesion affecting both his lower limbs, with increased tone, brisk reflexes and upgoing plantar reflex. Power is 4/5 throughout. His arms do not appear affected but I would need to formally examine them. These findings are consistent with a spastic diplegia, which may be a result of cerebral palsy or an acquired brain injury. To complete my examination, I would like to review the observation chart, perform a musculoskeletal and vascular examination of the lower limbs, examine the arms and examine cranial nerves. I would also like to plot his height and weight on a growth chart.
SCENARIO 12 Phoebe is an 8-year-old girl who presents to ED with drooping of the right side of her face. Please examine her cranial nerves. EXAMINATION CHECKLIST Domain
Findings
Evolving thought process
Global assessment of child.
• Appears well, not in pain, well grown for age.
• Lesion not impacting on general health.
Inspection.
• Drooping of right side of face. No dysmorphic features.
• Possible facial nerve palsy.
Individual cranial nerves.
• Cannot close right eye tightly. • When smiles, right side does not lift.
• Appears to have a unilateral facial palsy affecting all of that side of the face.
• Normal gait on entering room.
• Rest normal.
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RESPIRATORY EXAMINATION
PRESENTING YOUR FINDINGS
Phoebe appears well grown for her age and grossly does not appear to have any other neurological signs. Her facial movements are asymmetrical, with lack of movement on the right side of her face. The rest of her cranial nerves are intact. I believe that Phoebe has a right-sided facial nerve palsy caused by a lower motor neurone lesion. The most common cause for this would be a Bell’s Palsy. To complete my examination, I would like to perform fundoscopy, perform a cardiovascular exam, including blood pressure, examine the spine, and perform a formal neurological examination of her arms, legs, and gait.
TABLE 34: Common acute respiratory presentations Disease
Possible clues
Bronchiolitis.
• Usually under 6-months-old, but up to 1- to 2-years-old. • Increased work of breathing +/- oxygen requirement. • Crackles and wheeze on auscultation. • Reduced feeding.
Viral induced wheeze.
• • • •
Exacerbation of asthma.
• School-aged children. • Increased work of breathing, wheeze +/oxygen requirement. • Breathless on speaking in severe exacerbation.
Lower Respiratory Tract Infection.
• Fever. • Increased work of breathing. • Crackles mainly, but there may also be wheeze.
Anaphylaxis.
• • • •
RESPIRATORY EXAMINATION The lungs are not the only organs in the thorax and other systems can produce respiratory signs, e.g. wheeze and crackles due to fluid overload secondary to cardiac failure. Do not look for solely a respiratory diagnosis when asked to perform a respiratory examination. Particular incidences when a more detailed assessment might be required include: 1 Does the child in respiratory failure need immediate intervention? See Box 8 for signs of respiratory distress. Be aware of signs of a tension pneumothorax, as this only requires clinical diagnosis before treatment is initiated. 2 Are there signs of chronic underlying respiratory disease or other chronic medical diseases that may impact the respiratory system? For example, a child with four limb cerebral palsy and scoliosis is likely to have reduced reserve for coping with a chest insult such as a lower respiratory tract infection.
Box 8: Signs of respiratory distress • Head bobbing in infants. • Nasal flaring. • Grunting. This is a result of a child breathing through a closed glottis in order to increase their end expiratory pressures.
• Subcostal recessions.
Trigger may be known. May be airway compromise. Wheeze. May be associated rash and facial swelling.
Disease
Possible clues
Cystic fibrosis.
• • • • •
Asthma.
• Harrison’s sulcus. • Hyperexpanded chest. • May be no signs to see at all!
Bronchiectasis. • Congenital, e.g. CF, PCD • Acquired, Post infective, Obstruction e.g. foreign body, Connective tissue disease
• • • • • •
Wet cough. Productive sputum. Crackles. Wheeze. Faltering growth. Clubbing.
Chronic lung disease (due to prematurity).
• • • • •
Younger child. Venepuncture scars on hand. Chest drain scars. Home oxygen. Other signs of prematurity, e.g. head shape, developmental delay.
Kartagener syndrome.
• • • • • •
Bronchiectasis (as above). Dextrocardia. Situs inversus. Nasal polyps. Sinusitis. Rhinorrhea.
• Stridor. This is a high pitched sound, indicating airway obstruction. It may be inspiratory, expiratory or biphasic. • Intercostal recessions.
Preschool-age children. Associated coryzal symptoms. Wheeze responding to salbutamol. May be family/personal history of atopy.
TABLE 35: Common patients that may appear in clinical examinations
• Too breathless to speak.
• Tracheal tug.
EXAMINATION
Phoebe is an 8-year-old year old girl who has presented to ED today with drooping of the right side of her face.
3 Does this child with wheeze/respiratory distress require further treatment, or are they improving? This is one of the commonest assessments required in paediatrics, and often involves determination of whether bronchodilator therapy needs to be escalated, or whether it can be reduced. Common acute presentations are shown in Table 34. Common cases for exams are shown in Table 35.
May be underweight. Central line/gastrostomy visible. Pancreatic enzymes around the bed. Nasal polyps. Clubbing.
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EXAMINATION
Summary Checklist Global assessment of child on approach. Inspection. Hands, face, chest, abdomen. Palpation. Lymph nodes, apex, chest expansion. Percuss. Lungs, front and back. Auscultate. Chest sounds throughout the lung fields. Completing the examination. ENT assessment. Bedside tests. Observation chart, peak flow, inhaler technique, plot weight/head circumference, inspect sputum sample.
Box 10: Grading of clubbing Stage 1. Fluctuant bogginess of nail bed. Stage 2. Nail fold angle lost. Stage 3. Nail bed has an increased curve. Stage 4. Distal phalanx of finger enlarged.
FIGURE 154 A
B
Starting the Examination The examination begins by observing the child. Begin with a broad assessment: 1 Does the child look unwell? Is the child playing happily or too breathless to even speak? Is there a cough or stridor? Any colour change to the skin? Is there an altered respiratory rate? 2 Are there any signs of chronic ill health? Determine if the child looks cachectic, whether they have any medication around the bed, and if they have any medical devices, longterm lines or feeding tubes in place. Is the child attached to a portable oxygen device? Is there a sputum pot? 3 Has treatment already been started? Are they attached to oxygen? Is a nebuliser currently being administered?
Inspection Hands ӹӹ Clubbing. For causes and grading, see Box 9 and 10. In an older child, ask them to hold their thumbs together, nailto-nail, and look through the diamond shaped space that is formed to assess loss of this normal curvature (Figure 154). ӹӹ Peripheral cyanosis.
Box 9: Causes of clubbing Respiratory. • Bronchiectasis (inc CF). • Empyema/lung abscess. • Pulmonary TB. • Fibrosing alveolitis. Cardiac. • Cyanotic congenital heart disease. • Bacterial endocarditis. • Atrial myxoma. GI. • IBD. • Biliary cirrhosis. Other. • Malignancy.
Clubbing. A. Normal. B. Clubbed. Normal
Clubbed
ӹӹ Radial pulse. Check this in an older child. Feel for 30 seconds and count the pulse rate. Tachycardia can be caused by sepsis, respiratory distress, or secondary to salbutamol use.
Face ӹӹ Dysmorphic features. ӹӹ Central cyanosis. This is best seen by asking the patient to open their mouth and lift their tongue. Cyanosis is seen in advanced cystic fibrosis, bronchiectasis or cyanotic congenital heart disease. ӹӹ Nasal polyps. Look in the nose. Bilateral polyps are often seen in patients with cystic fibrosis. ӹӹ Ask the child to cough and to huff. This will give an indication of whether there is a prolonged expiratory phase, or if there are significant lung secretions, e.g. cystic fibrosis. ӹӹ Hearing aids. Children may have associated hearing impairment with primary ciliary dyskinesia.
Neck ӹӹ Cervical lymphadenopathy. Palpate the anterior and posterior chains. Lymphadenopathy may indicate an acute illness or chronic inflammation. ӹӹ Tracheal position. (Figure 155).
Abdomen ӹӹ Gastrostomy. This may be present or there may be a scar from previous gastrostomy. It is used in children with cystic fibrosis with faltering growth to increase calorie intake and aid growth. ӹӹ Palpate liver. Is there hepatomegaly or has the liver been pushed down by hyperexpansion? Usually this would be performed at the end of the examination after the chest has been auscultated.
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RESPIRATORY EXAMINATION 1 FIGURE 155
FIGURE 156
2 3
1 Tracheal palpation. Demonstrate the examination technique first. Place one finger either side in the sternal notch to see if the sternum is in the midline. Do not press too hard as it can be uncomfortable.
4
3
Chest Start with anterior chest examination first. ӹӹ Respiratory rate. Count it over 30 seconds. ӹӹ Chest wall shape. ›› Pectus excavatum. Sternum pushed in, hollowed chest. ›› Pectus carinatum. Sternum pushed out, “pigeon chest”. ӹӹ Obvious hyperinflation. This could be due to bronchiectasis, chronic asthma, cystic fibrosis, or chronic lung disease. ӹӹ Harrison’s sulci. This refers to grooves along the lower border of the chest. It is seen in chronic airway disease and poorly controlled asthma. ӹӹ Scoliosis. This is particularly exaggerated in children with cerebral palsy, but it may not be obvious until the child is seen from behind. ӹӹ Central venous access device. This may be present in children with cystic fibrosis or bronchiectasis, who require prolonged courses of intravenous antibiotic treatment for exacerbations. ӹӹ Chest wall recessions. This indicates increased work of breathing. ӹӹ Scars. These may be from cardiac or lung surgery (Figure 156). Remember to lift up each arm and look in the axilla, as lateral thoracotomy scars may be hidden.
Palpation ӹӹ Chest expansion. Assess from the side. In asthma, the anteroposterior diameter may be increased (indicating obstructive airway disease)
4
Chest drain scar May be in multiple sites in axillary triangle. Usually small scars similar to venepuncture scars Left thoracotomy Blalock-Taussig shunt, lobectomy, coarctation repair, PDA ligation, pulmonary artery banding
EXAMINATION
2
Right thoracotomy Right sided, may be axillary only. Could indicate a modified Blalock-Taussig shunt, a lobectomy performed due to congenital pulmonary airway malformation or repair of a tracheooesophageal fistula
Median sternotomy Bypass surgery e.g. correction of major cardiac defects
Common chest wall scars in children. FIGURE 157
A
B
Both AP expansion and lateral expansion can be tested. A. AP expansion. Place the palms of the hands on the chest. Note how much the hands are raised when the child breathes in and out. B. Lateral expansion. Place the palms of both hands on the anterolateral chest wall either side of the midline with thumbs apart and pointing towards the head. Note how far apart the thumbs move as the child breathes in and out.
(Figure 157). In school-aged children, chest expansion is usually 3-5 cm. ӹӹ Feel for apex beat of heart. Altered position of apex beat may indicate mediastinal shift or dextrocardia.
FIGURE 158
Percussion Percussion is not so useful in infants, but in older children it is performed. Percuss over the clavicles, in the upper, middle and lower zones on each side of the chest (both front and back), and in both axillae (Figure 158). Listen for the differences in the two sides. The note may be: ӹӹ Dull. This may be the case in consolidation or effusions (stony dull). ӹӹ Hyperresonant. This occurs with pneumothoraces.
Place the middle finger of left hand over the site to percuss. With the middle finger of the right hand, tap 2-3 times onto the other finger. The wrist must be kept loose.
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EXAMINATION
Auscultation
FIGURE 159
Work down from top to bottom comparing right to left. Auscultate in six zones on the anterior chest (upper left, upper right, middle right, middle left, bottom left, bottom right), both axillae, and repeat on the back (Figure 159). Abnormal sounds may include: ӹӹ Crackles. This indicates consolidation, collapse, bronchiectasis, pulmonary oedema or fibrosis. ӹӹ Wheeze. This is an expiratory sound due to airway narrowing, commonly due to bronchiolitis, viral-induced wheeze or asthma. If focal, consider an inhaled foreign body, particularly in preschool aged children. ӹӹ Reduced or absent air entry. This may be due to consolidation, effusion or poor lung expansion. ӹӹ Pleural rub. This is caused by inflamed pleura rubbing together. It is a creaking sound that can be heard in expiration and inspiration. Causes include pneumonia and pulmonary emboli. Tactile vocal fremitus may be tested for at this stage, if relevant (suspected consolidation/collapse) (Figure 160).
Auscultating the chest.
FIGURE 160
Completing the Examination ӹӹ Repeat the above inspection through to auscultation, for posterior chest. This is less disruptive than palpating the front of the chest and then the back of the chest, and then listening to the front of the chest, listening to the back of the chest, etc. ӹӹ Examine the ENT system. Bedside tests include: ӹӹ Review the observation chart. ӹӹ Plot the height and weight on an age and sex appropriate chart. ӹӹ Perform a peak flow. ӹӹ Collect sputum sample for culture. Place the medial, ulna border of the hand on the chest wall, palm side up. Feel for how the sound is transmitted to your hand. In older children, you can get them to say “ninety-nine”. It will be increased in consolidation.
CASE SCENARIO SCENARIO 13 Millie is a 14-year-old girl who has attended clinic today for review. Please perform a respiratory examination.
EXAMINATION CHECKLIST Domain
Findings
Evolving thought process
Global assessment of child on approach.
• Alert, active, and looks well. • Looks underweight.
• Unlikely to have an acute pathology. • May have a chronic underlying disease.
Inspection.
• • • • •
• May be malnourished. In keeping with a chronic underlying systemic disease, could this child have cystic fibrosis?
Palpation.
• Normally placed apex. • Hyperinflated.
• Respiratory pathology identified, no evidence of Kartagener’s syndrome.
Percuss.
• Normal.
• No additional pathology identified.
Auscultate.
• Few scattered crackles.
• May have a chest infection.
Small cervical lymph nodes, non-tender. Little subcutaneous fat. Normal RR. Pancreatic enzymes by bed. Empty sputum pot by bed.
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THYROID EXAMINATION
PRESENTING YOUR FINDINGS
Millie is a 14-year-old girl seen in the paediatric outpatients.
These findings are consistent with a diagnosis of cystic fibrosis, particularly supported by the pancreatic enzyme supplements. She does not appear to have an exacerbation at present and there is no evidence of bronchiectasis.
EXAMINATION
Millie appears underweight with little subcutaneous fat. However, I would like to plot her height and weight on a centile chart to confirm this. She appears comfortable at rest, has a normal respiratory rate and is not cyanosed. She has some small cervical lymph nodes palpable and a normally placed apex. Millie’s chest is hyperexpanded, with a few scattered crackles heard on auscultation. I note that she has an empty sputum pot and pancreatic enzymes by her bedside.
To complete my examination, I would like to review the observation chart, plot Millie’s height and weight on a growth chart as aforementioned, obtain a sample of sputum for microscopy, culture and sensitivity, and perform a peak flow test.
THYROID EXAMINATION Thyroid examination may be performed in children with a suspected thyroid neck lump, or with clinical features of thyroid disease (Table 36). Common patients likely to appear in exams are show in Table 37. TABLE 36: Clinical features of thyroid disease Hyperthyroidism
Hypothyroidism
• • • • • • • • • • •
• • • • • • •
Weight loss. Sweating. Heat intolerance. Increased appetite. Diarrhoea. Anxiety. Tachycardia. Hypertension. Hand tremor. Exophthalmos. Lid lag.
Weight gain. Cold intolerance. Constipation. Bradycardia. Slow relaxing reflexes. Short stature. Delayed puberty.
TABLE 37: Common exam patients Disease
Possible clues
Autoimmune (Hashimoto’s) thyroiditis.
• Goitre. • Short. • Patient may be clinically euthyroid or hypothyroid. • Often female.
Graves’ Disease.
• Goitre. • Signs of hyperthyroidism, although may be clinically euthyroid. • Proptosis. • Lid lag.
Thyroglossal cyst.
• Mass in midline. • Mass rises when tongue is stuck out.
Starting the Examination The examination begins on entering the room. Whilst introducing yourself, make a broad assessment: 1 Does the child look well or unwell? Hyperthyroidism can be associated with cardiac arrhythmias. 2 Are they restless and sweaty? Do they have any tremors? These are all associated with hyperthyroidism.
Summary Checklist Global assessment of child. Inspection. Hands, face, eyes, teeth. Palpation. Goitre. Percussion. Sternum. Auscultation. Goitre for bruit. Completing the examination. BP, reflexes, proximal myopathy. Bedside tests. Review observations, plot height and weight.
3 Are they an appropriate size for their height? Hypothyroidism is associated with being short and fat. 4 Are they appropriately dressed for the room temperature or wearing lots of layers? Temperature intolerance is reflected in clothing. Endocrine examinations are often easier to carry out going from one body part to the next, and so this is how the thyroid examination will be presented.
Inspection and Palpation Hands ӹӹ Pulse rate and rhythm. The patient who is hyperthyroid can be tachycardic, or in atrial fibrillation. Bradycardia is associated with hypothyroidism. ӹӹ Palmar erythema. This is seen in hyperthyroidism. ӹӹ Sweaty palms. This is seen in hyperthyroidism. Feel this. ӹӹ Blood pressure. In hyperthyroidism, there may be a high systolic pressure and wide pulse pressure. ӹӹ Thyroid acropachy. This is subperiosteal new bone formation of the digits which manifests as clubbing. It is associated with Graves’ disease. ӹӹ Vitiligo. Look for other signs of autoimmune diseases such as vitiligo. ӹӹ Tremor. There may be a fine tremor if the patient is hyperthyroid (Figure 161).
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EXAMINATION
FIGURE 161
Ask the patient to hold out both their hands, arms outstretched. Look for a tremor. Putting a piece of paper over the hands will help make this more obvious.
Face ӹӹ Signs of thyroid eye disease. Look for these both from the front, the side and stand behind the child and look over and down (Figure 162): ›› Exophthalmos. This describes bulging of the eyeballs out of the orbit. Assess from the side, and by looking over the top of the patient from behind. ›› Ophthalmoplegia. This indicates pathology of cranial nerves III, IV and VI. ›› Lid lag. (Figure 163). ›› Lid retraction. If the eyelids are retracted, the sclera will be visible above the iris. ›› Oedema of the eyelid and conjunctivae. Conjunctival oedema is known as chemosis.
Neck ӹӹ To fully inspect the neck, look from the front, back and from both sides. Exposure of the chest is required to assess the sternal notch.
FIGURE 162
A
B
C
Inspect the eyes from: A. Side. B. Front. C. Above. FIGURE 163
A
FIGURE 164
B
Lid lag. Ask the child to follow the examiner’s finger (positioned horizontally) as it is moved rapidly from an up (A) to a down (B) position. The eyelid will lag behind the movement of the eye and so when the child looks down, the sclera will be more visible.
Palpating for a goitre.
FIGURE 165
ӹӹ Lymphadenopathy. Feel the anterior and posterior cervical chains for lymphadenopathy. ӹӹ Masses. Describe any masses as shown on p574. Palpate any goitre whilst standing behind the child (Figure 164), but warn them beforehand. Particularly assess for: ›› Size. Assessing this requires percussion down onto the chest to identify any retrosternal extension (Figure 165). ›› Shape/Surface/Consistency. There may be nodules. ›› Tenderness. This is suggestive of infection. ›› Transillumination. This is suggestive of a cyst. ›› Bruits. Bruits are associated with hyperthyroidism (Figure 166).
Percussing for retrosternal extension.
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THYROID EXAMINATION
FIGURE 166
FIGURE 167
Auscultating for bruits.
ӹӹ Scars. A scar would indicate previous thyroid surgery. ӹӹ Swallow test. Thyroid goitres will move up with swallowing (Figure 167). ӹӹ Sticking out tongue. Ask the patient to stick out their tongue (Figure 168). A thyroid mass will remain in its position on tongue protrusion, whereas a thyroglossal cyst will move up.
Completing the Examination ӹӹ Reflexes. These may be slow-relaxing in hypothyroidism. Test one, e.g. knee jerk (Figure 169). ӹӹ Pretibial myxoedema. This is seen in Graves’ disease. Bilateral, firm, nonpitting, asymmetrical plaques/nodules. ӹӹ Proximal muscle weakness. This is associated with hypothyroidism (Figure 170). ӹӹ Perform a general paediatric examination. Bedside tests include: ӹӹ Review the observation chart. ӹӹ Plot the height and weight on an age and sex appropriate chart.
B
Ask the child to take a sip of water, hold it in their mouth and then ask them to swallow. Does the goitre move on swallowing? Inspect (A) and palpate (B) when doing this.
EXAMINATION
A
FIGURE 168
A
B
Assessing for thyroglossal cyst. Observe from the side (A), and palpate (B) to see if the mass rises on tongue protrusion.
FIGURE 169
FIGURE 170
Assessing reflexes.
Proximal myopathy. With the child seated in a chair, ask them to stand up without using their arms (e.g. ask them to cross their arms whilst doing it).
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EXAMINATION
CASE SCENARIO SCENARIO 14 Judy is a 14-year-old girl who has come to outpatient clinic with a neck mass. Please examine her neck and proceed as necessary. EXAMINATION CHECKLIST Domain
Findings
Evolving thought process
Global assessment of child on approach.
• Appears well, obvious neck mass in midline. • Appears overweight.
• Not acutely unwell. Could the mass be a goitre?
Hands.
• Normal pulse rate. No peripheral stigmata of thyroid disease.
Face.
• No lymphadenopathy.
• May not be a thyroid mass, or could be thyroid disease well controlled.
Neck.
• Solid 2 cm x 2 cm mass, non-tender, diffusely enlarged, smooth, no bruits, not fluctuant, and not extending into the chest. • Mass moves up on swallowing, does not move on sticking out tongue.
• Normal eye examination. • Likely thyroid mass. • Only sign suggesting it is hypothyroidism is that she appears overweight.
PRESENTING YOUR FINDINGS
Judy is a 14-year-old girl who has presented to clinic today with a neck mass. Judy appears well but overweight. She has a neck mass in the midline that elevates on swallowing, but not on tongue protrusion. The mass is 2cm x 2cm, smooth, non-tender and not fluctuant. There is no retrosternal extension or thyroid bruit. There are no thyroid eye signs, or other signs of illness. To complete my examination, I would like to plot Judy’s height and weight on an age and sex appropriate growth chart. My impression is that Judy has a thyroid goitre. I believe her to be hypothyroid in view of her short stature but would like to take a history to assess her thyroid status. The commonest cause of hypothyroidism with a goitre is autoimmune thyroiditis. To confirm this diagnosis, I would need to measure her T4 and TSH levels.
REFERENCES AND FURTHER READING General 1 Bedwani SJ, Anderson CJR, Beattie RM. MRCPCH Clinical: short cases, history taking and communication skills. Third edition. Cheshire: PasTest; 2011. 2 Thomson A, Wallace H, Stephenson T. Short cases for the MRCPCH. Edinburgh: Churchill Livingstone; 2005. 3 Barakat N, Buchdahl R. Get Through. MRCPCH Part 2: 125 questions on clinical photographs. Florida: CRC Press; 2005. 4 Stanford School of Medicine. Newborn photo gallery. newborns.stanford.edu/PhotoGallery/ 5 MRCPCH2009. Dysmorphic atlas. www.mrcpch2009.com/ educational-materials/dysmorphic-atlas-1
6 Harris W. Examination Paediatrics. 4th Ed. New South Wales: Churchill Livingston; 2011. 7 Stephenson T, Wallace H, Thomson A. Clinical Paediatrics for Postgraduate Examinations. 3rd edition. Edinburgh: Churchill Livingstone; 2003.
Development 1 Sharma A, Cockerill H. From birth to five years: practical developmental examination. Fourth edition. Abingdon: Routledge; 2014.
Cardiology 1 Samuels M, Wieteska S. Advanced Paediatric Life Support. The Practical Approach. Fifth edition. Chichester: Wiley-Blackwell; 2005.
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REFERENCES AND FURTHER READING
Musculoskeletal
Neurology 1 Palisano R, Rosenbaum P, Walter S, Russell D, Wood E, Galuppi B. Development and reliability of a system to classify gross motor function in a child with cerebral palsy. Dev Med Child Neurol. 1997;39:214-23. 2 Cunningham ML, Heike CL. Evaluation of the infant with an abnormal skull shape. Curr Opin Pediatr. 2007;19:645-51.
EXAMINATION
1 Arthritis Research Council. pGALs. http://www .arthritisresearchuk.org/health-professionals-and-students /video-resources/pgals.aspx. 2 Wheeless’ Textbook of Orthopaedics. www.wheelessonline .com/ortho. 3 Houghton KM.Review for the generalist:evaluation of paediatic hip pain. Pediatr Rheumatlo Online J. 2009;7:10. 4 Soucie JM et al. Range of motion measurements: reference and a database for comparison studies. Haemophilia. 2011;17:500-7.
5 Bandy W and Reese N. Joint Range of Motion and Muscle Length Testing. Second edition. Missouri: Saunders Elsevier;2013.
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3.03
COMMUNICATION ANNA CHADWICK AND MICHAEL MALLEY
CONTENTS
605 Introduction 605 Theories of Communication
605 Five Components of Communication 605 Ideas, Concerns, Expectations
605 Key Components of Communication 605 Preparation and Location 606 Creating Rapport 606 Acknowledging Agenda 606 Verbal Skills 606 Non-Verbal Skills 606 Active Listening
606 Communicating with Children of Different Ages
606 Baby 607 Pre-verbal Child 607 Verbal Child 607 Adolescent/Young Person
607 Parents
607 Communication Challenges 607 Clinical Cases
607 Case 1: A newborn baby presenting with ambiguous genitalia 608 Case 2: A 3-day-old baby with a new diagnosis of Down Syndrome 609 Case 3: A woman at 29 weeks gestation requiring counselling for pre-term delivery 609 Case 4: A 4-year-old boy inadvertently given the wrong dose of paracetamol. 610 Case 5: A 15-year-old girl non-compliant with her diabetes medication 611 Case 6: A 6-month-old boy who has not been vaccinated due to parental choice
612 References and Further Reading
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KEY COMPONENTS OF COMMUNICATION
INTRODUCTION
TABLE 1: Communication with children and adults Children
Adults
Often indirect, through a parent/ carer.
Usually directly from patients.
Children may not know why they are in hospital.
Adults usually self-present to medical care.
Child-focused language is required to explain complex ideas. It may be difficult to convince a child of the importance of something, and distraction/restraint techniques are often necessary.
More likely to understand medical terminology, and appreciate the need for procedures such as examination or blood taking.
Establishing rapport often involves being fun, playful and friendly towards the child.
Establishing rapport may involve a more serious, conservative approach.
THEORIES OF COMMUNICATION It is important to recognise the limitations of applying communication models in practice. Patients are unlikely to interact well with a doctor who recites information or comes across as formulaic, so intuition and adaptation are key to
Five Components of Communication This theory helps frame the purpose and manner of the communication scenario (Example 1).
Ideas, Concerns, Expectations (ICE) This theory helps identify any initial thoughts the patient might have, allowing communication to be tailored to this (Example 2). In paediatrics, this tool must incorporate the concerns of both the parents and the child.
COMMUNICATION
Throughout medical training, the importance of communication skills is continually emphasised. Paediatrics has the additional challenges of communicating with children across all age groups, with parents and carers, and especially with children who cannot verbalise. Table 1 shows the key differences between paediatric and adult communication. Teenagers can be treated more like adults. This chapter is designed to illustrate common components of effective communication in the paediatric setting.
communicating well. However, the foundations of good communication can be recognised within models and theory. Two models are described below.
KEY COMPONENTS OF COMMUNICATION Communication with any patient, carer or staff member can benefit from addressing the following factors.
Preparation and Location Choose an appropriate setting. Ideally, for a sensitive conversation, choose a quiet side-room, free from interruptions. Remove potential distractions, such as a pager, and read the medical notes and any test results prior to the discussion if possible. Similarly, think about the seating arrangements. Creating a comfortable setting that implies equality amongst the participants is likely to encourage effective communication. Get down to the child’s level, rather than standing over them. Decide who should be present. Some discussions are best held with both parents present, while others may not be appropriate in front of the child. It is good practice to have an allied health professional present when breaking bad news, and it is always useful to establish beforehand what the patient or carers already know.
EXAMPLE 1 An illustrated example of the ‘five components of communication’ model.
Element
Explaining the need for oral antibiotics for a 5-year-old with an ear infection in the Emergency Department (ED)
1
Whom – By whom is the communication initiated?
Here, the paediatric doctor will initiate the discussion.
2
What – What content is to be communicated?
The content to be explained is the need for antibiotics and how the prescription should be administered.
3
How – How is the content delivered?
The content is communicated verbally with written prompts (i.e. prescription on the bottle of antibiotics), and non-verbal skills are used throughout.
4
Who – Who is receiving the content?
The parent and child are the recipients of the content.
5
What effect – What is the outcome of the communication?
The hoped for outcome is that the parent will understand why antibiotics are needed and will feel reassured and confident in giving them.
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EXAMPLE 2 An illustrated example of the ‘Ideas, Concerns and Expectations’ model. Explaining the diagnosis of type 1 diabetes to a 12-year-old girl and her mother.
Type 1 diabetes: new diagnosis
Possible questions to ask
Child
Parent
Ideas
What do you think might be going on?
May not know about diabetes. May not appreciate they are unwell.
May know this is diabetes already. May be more concerned about weight loss or other features.
Concerns
Is there anything in particular you are concerned about?
May be worried about injections or changing diet.
May be concerned her daughter won’t know how to manage hypoglycaemia episodes. May have a relative with diabetes, and be worried about diabetic complications.
Expectations
What do you think might be the best plan of action?
She may want to be the one in charge of managing her diabetes. She may need more time to come to terms with the diagnosis.
She may expect tertiary specialist care. She may expect a cure. She may expect regular contact from diabetic specialists.
Creating Rapport A simple introduction with a handshake and a smile or kneeling down to chat to a toddler before taking a history can create a comfortable rapport. In some settings, establishing a rapport may take longer — for example, when breaking bad news to a patient or carer you have not met before. Making an effort to put the patient and their carers at ease will improve the dialogue and working relationship.
Acknowledging Agenda When entering into a new scenario, recognise the agendas of each of the people involved, as this may affect the outcome of the situation. For example, a parent who brings their child with a cough and fever to ED may be expecting a prescription for antibiotics: this is their agenda. However, the clerking doctor will be assessing the child’s health and making a tailored decision about the appropriateness of antibiotics. Without recognising the parent’s agenda, the doctor may miss an opportunity to discuss the lack of a role of antibiotics in a viral infection. If the parent feels their agenda has not been acknowledged, they may feel their expectations have not been met and may lose confidence in the doctor’s clinical judgement. The agenda underlying each interaction may not be obvious, but quick assumptions of culture or values impact the overall interaction with patients, carers and colleagues. Using non-judgmental and open questioning, providing clear information and offering the chance to ask questions should eliminate the risk of missing any key concerns.
Verbal Skills Avoid beginning with closed questions and limit the use of jargon. If jargon is unavoidable, explain any such terms. When covering sensitive or confrontational topics, have an appropriate tone of voice. Recognise the effect of language barriers on good communication and use an impartial interpreter if needed. Although occasionally unavoidable in an
emergency, using a bilingual paediatric patient to communicate to their non-English speaking parent can risk incorrect translation.
Non-Verbal Skills Eye contact encourages trust and the appropriate use of body contact can be empathic. Don’t be afraid of silence. Pauses in a conversation may feel uncomfortable, but are useful for processing information, particularly in the context of breaking bad news, and they may lead readily to the next stage of the conversation. Paediatricians often need the ability to interpret the non-verbal skills of their patients, whether limited by age or cognitive ability. The appreciation of basic posture and behaviours (e.g. grimacing, back arching) may give essential information that cannot otherwise be communicated by the patient. The use of expert skills, such as occupational therapy (i.e. sign language, picture cards) and play therapy, can optimise communication in the absence of verbal skills.
Active Listening Active listening not only involves critically analysing what is being said, but also letting the speaker know that they are being heard, both through encouraging words and behaviours. Similarly, it is important to ensure that any information or advice offered is registered. A useful tool is to ask the recipient to summarise in their own words the information imparted. This strategy can also be used when taking a clinical history or handover to a colleague.
COMMUNICATING WITH CHILDREN OF DIFFERENT AGES Baby In newborns, variations in the tone of a cry can indicate different needs. A high-pitched cry may signify pain or distress.
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CASE 1: A NEWBORN BABY PRESENTING WITH AMBIGUOUS GENITALIA
Pre-verbal Child Toddlers may have stranger anxiety or be miserable if unwell. A direct introduction to the patient is important, crouching down to their level. Use a toy for distraction. Place a stethoscope on the parent’s chest/the child’s arm before formal auscultation. This helps introduce the instrument as being playful and non-painful. Cheerful smiles and a relaxed disposition can calm young children, but separation anxiety is common at this age, so allow parents to comfort the child as they wish. Avoid disturbing the child if they are nervous or agitated. Play therapists can be invaluable in communicating with children in these younger age groups.
the techniques for pre-verbal children is insufficient, as the child’s understanding may be greater than suggested by their behaviour. Allow more time to assess the likely cognitive age of the patient. Ask the parents how they normally communicate with their child, or if there is anything the child particularly dislikes. Some children with autism, for example, do not like to be touched on certain parts of their body. The use of sign language, such as Makaton or picture cards, can be helpful. A play therapist may assist when performing essential tasks, such as examination or venepuncture. Always maintain eye contact where appropriate and try to involve the child in the consultation as much as possible. Do not be afraid to give them a break and return later, when discussion may be less distressing and information quicker to obtain.
CLINICAL CASES
CASE 1
A newborn baby presenting with ambiguous genitalia
Verbal Child
Candidate Briefing
Ask a few questions; for example, enquire about their age or the names of any dolls they may have with them. Older children are more likely to follow instructions, so talk to them rather than their parents during examination. Try to continue the dialogue, using age-appropriate language (such as “sore” rather than “painful”), and ask the child to point to or describe any symptoms they have. The child may be able to describe symptoms or localise pain.
You are a junior doctor on the labour ward and you are paged to review a baby immediately after delivery. On arrival, you find the baby is clinically stable and is wrapped in a towel and being warmed on the resuscitaire. The midwife asks to speak to you outside the labour room and tells you that she thinks the baby’s genitalia are abnormal. She explains that the firsttime parents are expecting a baby girl and are unaware of the abnormality so far. You return to the room to examine the infant, and find a well baby with ambiguous genitalia. The midwife asks you to explain the findings to the mother, as the father has popped outside to tell family members about the new arrival.
Adolescent/Young Person This age range provides a variable challenge, as some teenagers will be open to communication and others less so. Talk directly to the patient. Work out whether the parents need to be present for the whole consultation. Offer to see the young person on their own. Some questions may be difficult to answer in front of parents, such as those involving sexual activity, drug and alcohol use, feelings of low mood and suicide attempts. If appropriate, find common ground to help develop a rapport, but maintain professionalism and appreciate the young person’s boundaries.
Parents The key here is to maintain an “adult” conversation whilst also simultaneously adapting to the child. Use empathic language to acknowledge concerns, and give clear, concise information where appropriate. Ensure understanding by asking the parent/ carer to feed back the main points of the conversation.
COMMUNICATION CHALLENGES Communication can be challenging when a child has developmental delay, autistic spectrum disorder (or other disorders of communication) or mental health problems. Using
COMMUNICATION
Parents/carers are often able to recognise cries due to hunger or tiredness. A happy baby may coo and babble or offer a smile. When communicating with a baby, use exaggerated non-verbal skills to reassure and comfort, like big smiley faces, gentle soothing noises and good eye contact. Use the parents to help console the infant, or consider examining during a feed if the baby is crying.
Patient/Actress Briefing You are Sarah Thompson, a first-time mother who has just had a normal delivery at 39 weeks. The pregnancy was generally fine, although you were taking some medication for depression in the first few months before you realised you were pregnant. All scans and blood tests have been normal and the anomaly scan revealed you were having a baby girl. You have never smoked and stopped drinking when you found out you were pregnant. You work as a teaching assistant and live with your partner, the baby’s father. Your partner is supportive and although the baby was unplanned, you are both excited about becoming parents. You are yet to hold your child as the midwife has said she would like the doctor to take a quick look, although she hasn’t explained why. Initially, you are worried your baby is unwell following the birth, and you are anxious to hold her. You don’t understand how there can be any question over the gender, especially as you were told you were having a girl by the sonographer at your 20 week scan.
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Questions to ask: ӹӹ How can the gender be ambiguous? ӹӹ What tests will be needed? Will the baby need surgery? ӹӹ Was there anything I did that could have caused this? ӹӹ Why was I told I was having a girl? ӹӹ Should I raise the baby as a boy or a girl for now? Only if specifically asked, tell the doctor that you’d ideally like to wait for your baby’s father to come back before having the conversation, but that he’s told you that he’s held up and you must speak alone for now.
EXAMINER CHECKLIST Domain
Comments
Introduction
• Introduces self by name and role. • Checks identity of the mother/infant. • Explains need to discuss an important finding and asks if the mother would like anyone else present.
Explaining Findings
• Establishes what the mother has been told already. • Clearly explains that the infant’s genitalia are ambiguous. • Explains that the anomaly scan is not a definitive method of predicting infant gender. Apologises for the upset this may have caused. • Explains that this means the gender of the infant cannot yet be confirmed. • Explains the need for senior review. • Explains that this may be the result of a genetic abnormality or a medical condition, i.e. congenital adrenal hyperplasia. • Reassures that this is not the parents’ fault. • Explains the need for further investigations, such as genetic testing. • Acknowledges the need for possible medical or surgical intervention at a later date.
Verbal Skills
• • • • •
Non-Verbal Skills
• Maintains good eye contact throughout. • Promotes active listening and responds to the actor’s cues.
Summary
• • • •
Speaks clearly and concisely when giving information. Uses compassionate, caring language throughout. Does not use medical jargon. Uses silences appropriately. Allows time for questions.
Summarises what has been said. Ensures understanding of the main issues. Clarifies the next step. Offers further support and a follow-up meeting.
CASE 2
A 3-day-old baby with a new diagnosis of Down Syndrome Candidate Briefing You are a junior doctor in the neonatal outpatient clinic. Mrs Jones is waiting to talk to you about her 1-day-old baby, Jamie. Jamie was floppy at birth and has mild dysmorphic features. Karyotyping has confirmed a diagnosis of Down syndrome (Trisomy 21). Mrs Jones does not know the diagnosis. Please inform Mrs Jones that the karyotype is positive and answer any questions she may have.
Patient/Actress Briefing You are Mrs Jones. You are 32-years-old and gave birth to your first baby, Jamie, four weeks ago. The pregnancy was smooth and unremarkable. You gave birth at 38 weeks via a normal vaginal delivery. You were told you had a low risk of Down Syndrome, and antenatal scanning revealed no abnormalities. You first thought something was wrong when Jamie struggled to breastfeed on day one and appeared floppy. The doctor mentioned that Jamie might have some “special facial features”, but you do not really see them. You are currently breastfeeding but requiring bottle top-ups. You live with Jamie’s father and have a good support network. You work as a park attendant. You do not drink or smoke. You have no involvement with social services. You were informed at the time of Jamie’s birth that he might have Down Syndrome. You don’t know much about it and think children with Down syndrome “look different” and need “lots of support”. Questions to ask: ӹӹ What is Down Syndrome? ӹӹ How do children get it? ӹӹ Why did this happen? ӹӹ Is it my fault? Is there anything I could have done differently? ӹӹ Why did the antenatal scans not pick this up? ӹӹ If I have any more children, will they also have Down Syndrome? ӹӹ What support is available to help me? If specifically asked, you are worried that Jamie will never live a “normal life” and that he might “die young”.
EXAMINER CHECKLIST Domain
Comments
Introduction
• Introduces self by name and role. • Checks identity of the mother/infant. • Explains the need to discuss the results of genetic tests and asks if the mother would like anyone else present.
Discussing the Situation
• Explains that the test for Down syndrome is positive. • Explains Down syndrome is a chromosomal/genetic abnormality. Avoids jargon. • Mentions at least two complications of Down’s syndrome when asked (e.g. learning difficulties, cardiac, bowel or neurological issues, leukaemia). • Explains the high likelihood of developmental delay (in over 90% of patients). • Explains Down syndrome is a life-long condition with no cure. • Reassures that many children with Down syndrome live a fulfilling life and can live to middle age. • Mentions medical support that is available: multidisciplinary team [MDT; e.g. occupational therapist (OT), physical therapist (PT), dietitian]. • Mentions non-medical support that is available (e.g. support groups, Down Syndrome Association, special schools). • Explains that testing in pregnancy gives a probability, but is not a guarantee. (Continued)
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CASE 4: A 4-YEAR-OLD BOY INADVERTENTLY GIVEN THE WRONG DOSE OF PARACETAMOL
EXAMINER CHECKLIST Comments
Domain
Comments
• Explains the relationship between maternal age and risk of Down Syndrome. • Reassures that it is not mother’s fault. • Explains that the chance of having a second child with Down syndrome is slightly increased.
Introduction
• Introduces self by name and role. • Checks identity of the patient. • Explains the need to discuss a potential preterm delivery and asks if the patient would like anyone else present.
Discussing the Situation
• Establishes what the patient has been told already and confirms the gestation of the pregnancy. • Explains that a senior paediatrician will attend the delivery and that the baby will be taken to a resuscitaire for stabilisation prior to transfer to the neonatal unit. • Explains the need to keep the baby warm using an incubator. • Explains that the baby is likely to require breathing support in the form of either continuous positive airway pressure (CPAP) or intubation. Acknowledges the importance of steroids in foetal lung development. • Explains that the baby will need to have blood tests and a cannula and will require feeding support. • Explains that further treatment will depend on the condition of the baby at birth. • Explains that the mother will be able to visit freely and will be close by on the postnatal ward. • Enquires about family situation, and explores possible ways to support the mother. • Explains the risks associated with preterm delivery but reassures that the chances of survival at this gestation are good. • If asked, offers to discuss the results of studies on outcomes of preterm deliveries.
Verbal Skills
• • • • •
Non-Verbal Skills
• Maintains good eye contact throughout. • Promotes active listening and responds to the actor’s cues.
Summary
• • • •
Verbal Skills
• • • • •
Speaks clearly and concisely when giving information. Uses compassionate, caring language throughout. Does not use medical jargon. Uses silences appropriately. Allows time for questions.
Non-Verbal Skills
• Maintains good eye contact throughout. • Promotes active listening and responds to the actor’s cues.
Summary
• • • •
Summarises what has been said. Ensures understanding of the main issues. Clarifies the next steps. Offers further support and a follow-up meeting.
CASE 3
A woman at 29 weeks gestation requiring counselling for pre-term delivery Candidate Briefing You are the junior doctor and have been asked to see Mrs Patel on the labour ward. Mrs Patel is 29 weeks pregnant and has severe pre-eclampsia, but currently the baby is a good size for gestational age. The obstetric team members are worried she may need an emergency caesarean section if her blood pressure cannot be controlled. The midwife asks you to counsel her on what to expect from a preterm delivery and answer any questions she has.
Patient/Actress Briefing You are Shushma Patel, a 31-year-old accountant. You are 29 weeks pregnant and this is your second pregnancy: two years ago, you had a miscarriage at 10 weeks. You have been told you are having a baby boy. Your husband frequently works abroad and is currently away due to work, and you haven’t yet told anyone else that you are in labour. You have never smoked and don’t drink alcohol. This is a planned pregnancy and you have followed all advice from your midwifery team. You have received two doses of steroids to help develop the baby’s lungs. You are very concerned about the risks to your baby of being born so early and are distressed that your husband won’t be here to support you. Questions to ask: ӹӹ Why is the baby coming early? ӹӹ Will the baby be able to breathe on his own? ӹӹ Will the baby be brain damaged? ӹӹ Will I be able to stay with my baby after he is born? If prompted for further questions, you should ask if there are any studies on preterm delivery outcomes. Only if specifically asked should you explain the situation with your husband.
COMMUNICATION
Domain
EXAMINER CHECKLIST
Speaks clearly and concisely when giving information. Uses compassionate, caring language throughout. Does not use medical jargon. Uses silences appropriately. Allows time for questions.
Summarises what has been said. Ensures understanding of the main issues. Clarifies the next step. Offers further support and a follow-up meeting.
CASE 4
A 4-year-old boy inadvertently given the wrong dose of paracetamol Candidate Briefing You are the general paediatric junior doctor on call. You are asked to see a 4-year-old boy on the ward who has recently been brought up from ED, having been admitted with a first episode of viral-induced wheeze. In the ED, he was given twice the recommended dose of paracetamol for his age and the nurse on the ward has just noticed the mistake on the drug chart. She asks you to explain the mistake to the parents and answer any questions they may have.
Patient/Actress Briefing You are Mr Lemarde, father to Theo, a 4-year-old boy admitted with his first wheezy episode. You are extremely concerned about him as he has had trouble breathing for the last two days. Theo was initially seen by a primary care physician, who sent you home with a salbutamol inhaler. You have brought him
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COMMUNICATION
in overnight as his breathing became more laboured and he developed a fever. So far, Theo has been given some paracetamol and salbutamol nebulisers before being transferred to the ward. Theo is your only child and is usually fit and well. He has no other medical problems and is rarely unwell. He has no allergies. When informed about the drug error, you are initially angry and confused about how this has happened. You are concerned about the effects an overdose may have on Theo and want to make a formal complaint. Questions to ask: ӹӹ Why was the large dose prescribed and given? ӹӹ Why are there no procedures in place to prevent this kind of error? ӹӹ Who is accountable for the mistake? ӹӹ What effect might the overdose have on Theo? Does he need any tests or treatment? Only if specifically mentioned, you should ask how to make a formal complaint. EXAMINER CHECKLIST Domain
Comments
Introduction
• Introduces self by name and role. • Checks the identity of the patient and his father. • Explains the need to discuss a drug error that has occurred involving Theo. • Asks if Mr Lemarde would like anyone else present.
Discussing the Situation
• Establishes what Mr Lemarde has been told so far. • Explains that a drug error has occurred, where Theo was given twice the recommended dose of paracetamol whilst in ED. • Apologises unreservedly for the error and acknowledges the shared responsibility amongst the medical team. • Explains that these errors are uncommon due to protocols in place for checking prescriptions but that these have failed on this occasion. • Establishes whether Theo is more unwell after the paracetamol overdose. • Explains that the amount of paracetamol given is unlikely to have a health impact on Theo, and that the total amount of paracetamol given per kg of body weight will be calculated to decide if any further tests are needed. • Explains that the drug error will be discussed with the members of staff involved, the nurse in charge and the admitting consultant, and will be reported internally via the incident reporting system. • Explains that an adverse clinical incident form will be filled in so that it will be looked into. • Offers advice on making a formal complaint, such as the contact details for the patient advice and liaison service (PALS).
Verbal Skills
• Speaks clearly and concisely when giving information. • Apologises clearly. Uses compassionate, caring language throughout. • Does not use medical jargon. • Uses silences appropriately. • Allows time for questions. (Continued)
EXAMINER CHECKLIST Domain
Comments
Non-Verbal Skills
• Maintains good eye contact throughout. • Promotes active listening and responds to the actor’s cues.
Summary
• • • •
Summarises what has been said. Ensures understanding of the main issues. Clarifies the next steps. Offers further support and a follow-up meeting.
CASE 5
A 15-year-old girl non-compliant with her diabetes medication Candidate Briefing You are a junior doctor in the diabetic outpatient clinic. Dawn Firth is a 15-year-old girl with type 1 diabetes who has come in for her annual review. Her mother is waiting for her outside the clinic room. Dawn has had diabetes since she was 8-years-old and is on a basal bolus regime. Recently, there have been concerns that Dawn is not taking her insulin appropriately. In the past six months, she has had two admissions to hospital for diabetic ketoacidosis. Today, her glycated haemoglobin (HbA1c) is 75 mmol/mol; her other blood results are normal and her urine dipstick is clear. Her previous HbA1c was 53 mmol/mol. You are asked to see Dawn and talk to her about her diabetes control. Please discuss the concerns and provide advice on how to optimise her current treatment in light of her results.
Patient/Actress Briefing You are Dawn Firth, a 15-year-old with type 1 diabetes. You were diagnosed at 8-years-old after a two week history of weight loss, increasing thirst and passing more urine than usual. You are attending the clinic for your annual review with your mother who is waiting outside. You have had some blood and urine tests, with results pending. Your current treatment plan is a basal bolus regime: you take your background insulin at night, but you have been concerned about your weight after hearing some nasty comments made by other girls at school. Having read on the internet that reducing your insulin causes weight loss, you have been omitting some insulin doses around mealtimes and running your blood sugars around 10–12 mmol most days, and sometimes higher. You have been admitted to hospital twice recently, feeling unwell and having high blood sugar levels. You have been advised to increase your insulin doses. Initially, you are reluctant to admit that you haven’t followed this advice and instead feel that you would rather have high blood sugar levels than gain weight. Areas for discussion: ӹӹ Initially be reluctant to discuss your diabetic control. ӹӹ If asked directly, explain that you have been omitting insulin doses in order to control your weight.
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CASE 6: A 6-MONTH-OLD BOY WHO HAS NOT BEEN VACCINATED DUE TO PARENTAL CHOICE
you establish that Bobby has not had any vaccinations. Before discharging Bobby, please discuss this with his mother and answer any questions she may have.
Only if specifically asked about the weight loss, mention being upset by the comments made by other girls at school.
You are Mrs Stevens, a 29-year-old office manager, and have brought your son Bobby to hospital because he developed a runny nose and high fever over the last couple of days. You have been seen by the paediatric team and are expecting to be discharged home today. You have not given Bobby any routine vaccines as you don’t believe that they work and think that they may cause him harm. Your niece had meningitis at 1-year-old, despite receiving the meningitis vaccine as part of the routine schedule, and you are adamant that this proves they are ineffective. You also have many friends whose children have suffered with fever and diarrhoea following the vaccines and have decided that, overall, it is better not to vaccinate Bobby. Bobby was born at term and is usually well. You have already had discussions with your primary care physician regarding vaccinations but believe it is ultimately your right to refuse them because you do not believe they are suitable for your son.
EXAMINER CHECKLIST Domain
Comments
Introduction
• Introduces self by name and role. • Checks identity of the patient. • Explains the need to discuss the diabetes, and asks if the patient would like her mother present.
Discussing the Situation
• Establishes what the patient has been told already and explains the blood and urine results. • Explains that HbA1c is a marker of long-term blood sugar control, and that the latest result is high and has increased since the previous annual review. • Asks whether the patient has any ideas about why this has happened. • Explores the reasons for the patient running high blood sugars and her understanding about the impact this has on her health. • Explains that chronically high blood sugars are likely to cause irreversible damage, such as problems with eyesight, kidney disease, nerve damage, and heart problems. • Explains that acutely high blood sugars can be dangerous and may require admission to a high dependency unit/ intensive care unit (HDU/ITU) for treatment. • Acknowledges that the patient may also experience low sugar symptoms if control is poor. • Explores the psychological impact of having a chronic disease and appreciates the impact of having diabetes on daily life. • Offers to refer the patient to a dietician for more advice on healthy weight control and signposts to a clinical nurse specialist/support groups for young people with diabetes.
Verbal Skills
• • • • •
Speaks clearly and concisely when giving information. Uses compassionate, caring language throughout. Does not use medical jargon. Uses silences appropriately. Allows time for questions.
Non-Verbal Skills
• Maintains good eye contact throughout. • Promotes active listening and responds to the actor’s cues.
Summary
• • • •
Summarises what has been said. Ensures understanding of the main issues. Clarifies the next step. Offers further support and a follow-up meeting.
CASE 6
A 6-month-old boy who has not been vaccinated due to parental choice Candidate Briefing You are the junior doctor in ED. Mrs Stevens has brought her 6-month-old son Bobby to hospital after he developed a fever at home. You diagnose him with a viral upper respiratory tract infection (URTI) and plan to send him home. During clerking,
Patient/Actress Briefing
COMMUNICATION
ӹӹ Ask why having a low HbA1c is important. ӹӹ Explain how upset you are about your weight and that you feel that because you have diabetes you cannot act like a normal teenager.
Areas for discussion: ӹӹ Initially be reluctant to discuss the reasons for refusing vaccines as you have already talked this through with your primary care physician. ӹӹ Explain that you believe vaccines to be ineffective, giving the example of your niece contracting meningitis. ӹӹ Explain that you are also concerned that they may have side effects such as fever. ӹӹ Ask why it matters as you believe conditions like whooping cough are treatable, and he is too old for it anyway. ӹӹ Ask what the common side effects of vaccinations are. Only if asked about your concerns should you state that you are worried about the vaccines being very painful.
EXAMINER CHECKLIST Domain
Comments
Introduction
• Introduces self by name and role. • Checks identity of the patient/parents. • Requests permission to discuss the mother’s refusal to vaccinate, and asks if she would like anyone else present.
Discussing the Situation
• Establishes what the mother understands regarding immunisation schedule. • Explores the reasons for her refusal to vaccinate her son. • Accepts the mother’s concerns as valid and offers to explain the nature of the immunisation schedule to ensure she is making an informed decision. • Explains that many of the diseases infants are vaccinated against have become dormant since the introduction of routine vaccination and as such public health has improved. (Continued)
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COMMUNICATION
EXAMINER CHECKLIST Domain
Comments • Explains that vaccines protect against diseases that can be fatal, e.g. whooping cough, and that these diseases have re-emerged due to the reduced uptake of vaccinations. • Explains that the current meningitis vaccine does not protect against all strains of meningitis, so although the rates of bacterial meningitis have reduced dramatically, it is still possible to develop the disease, although new vaccines are being implemented. • Explains that although the vaccines frequently cause children to develop a mild fever and be unsettled, this is temporary and should not be a reason to avoid vaccination. • Acknowledges the mother’s right to refuse the vaccine schedule but emphasises the clinical importance to protect both her son and other children around him.
Verbal Skills
• • • • •
Non-Verbal Skills
• Maintains good eye contact throughout. • Promotes active listening and responds to the actor’s cues.
Summary
• • • •
REFERENCES AND FURTHER READING 1 Tate P, Tate L. The Doctor’s Communication Handbook. Seventh Edition. London: Radcliffe Publishing Ltd.; 2014
Clear and concise when giving information. Uses compassionate, caring language throughout. Does not use medical jargon. Uses silences appropriately. Allows time for questions.
Summarises what has been said. Ensures understanding of the main issues. Clarifies the next step. Offers further support and a follow-up meeting.
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3.04
PRACTICAL SKILLS MICHAEL MALLEY, ZESHAN QURESHI, ANITA DEMETRIOU AND MARIE MONAGHAN
CONTENTS 614 Introduction 614 Vascular Access
614 Capillary Blood Gas 616 Venous Blood Sampling 619 Arterial Line Sampling 620 Intravenous Cannulation 622 Umbilical Venous and Arterial Catheterisation 626 Emergency Umbilical Venous Catheterisation 627 Intraosseous Needle Insertion
628 Airway Procedures
628 Bag-Valve-Mask Ventilation 630 Nasopharyngeaal Airway Insertion 631 Oropharyngeal Airway Insertion 631 Laryngeal Mask Airway
632 Neonatal Intubation 636 Surfactant Administration
637 Asthma
637 Asthma Inhalers 639 Peak Flow Measurement
640 Additional Procedures
640 Lumbar Puncture 642 Non-Invasive Blood Pressure Measurement 642 Nasogastric Tube Insertion 644 Urinalysis
645 Baby Care
645 Changing a Nappy 646 Dressing and Undressing a Baby 647 Preparing Baby Milk Formula
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PRACTICAL SKILLS
INTRODUCTION
T
he key to success is preparation and practice. The best way to accomplish this is to discuss the procedure with a senior colleague and observe the skill in order to understand the process, before performing it under supervision. In addition to knowing the steps, it is important to know the indication for a procedure and its impact on management. This chapter contains a brief “how to” guide for each of the key competencies expected of junior doctors and paediatric trainees. Important themes common to all procedures include: ӹ Explanation. Explain in lay terms what is going to be done, and why, to the child and parents. Invite questions. ӹ Consent. Gain informed consent and ensure the patient and parents are aware of any serious or common side effects. Consent may be written or verbal depending on the procedure. ӹ Pain relief. This may involve the use of oral/intranasal/ intravenous/topical anaesthesia, cold spray, or oral sucrose. ӹ Distraction. Nearly all procedures are better tolerated with the use of appropriate distraction beforehand. Involve the play specialist team where possible. If unavailable, utilise smartphone apps/videos/toys as appropriate. ӹ Aseptic technique. Observe aseptic or sterile conditions as appropriate. ӹ Keep calm. Ensure adequate preparation, with appropriate support to hand. Being calm increases the likelihood of success. Know what to do in the event of any unexpected events. ӹ Post-procedure. After each procedure, clear the equipment, follow-up results and act on results as necessary.
Assessment Early identification of the need for an invasive procedure. Involve play specialist. Early application of local anasethetic when needed. Depending on age, check child has an understanding of what needs to be done.
Preparation If parents wish to be actively involved, begin to get child into any appropriate holding position. Check that all equipment and team-members are ready. Ensure the child is actively distracted.
Teamwork Distractor (play specialist or holder) should be the lead voice for communicating/coordinating between medical staff and child, reducing unfamiliarity for the child. Check that the team is ready: holder, distractor, person doing the task and parents (if opting to be present). Minimise the number of people in the room.
Implementation Where possible, use a procedure room. If appropriate, ask the child if they would like a warning, e.g. “quick scratch coming" for cannulation.
Post Procedure Thank the child and praise their efforts, e.g. with cheering, a high five, a sticker or a bravery certificate. Explain to the child why any bandage is on and ask parents to supervise it. Explain the ongoing plan again clearly, e.g. time to wait until blood results or admission details. Check if there are any questions. Perform any safety checks afterwards (e.g. flush cannula to ensure patency, request X-ray for position of ET tube) FIGURE 1
A flow chart for performing procedures is shown in Figure 1.
VASCULAR ACCESS
Flowchart for performing an invasive procedure.
EQUIPMENT CHECKLIST
Capillary Blood Gas (“heel prick”) Arterial blood gases are rarely performed in paediatrics unless from a secure arterial line in a high dependency setting. As a result, capillary gases fulfil an important role in the assessment of the ill child. Capillary blood gases are one of the most readily available objective barometers of a child’s health. They are quick and easy to obtain, with almost immediate results.
Indications ӹ Acutely unwell patient, e.g. respiratory compromise, severe sepsis. ӹ Checking glucose, bilirubin, haemoglobin or electrolytes. ӹ Post intubation or for assessing the efficacy of ventilation.
P Skin cleansing solution, e.g. 70% isopropyl alcohol skin disinfectant. P Non-sterile gloves. P Clean tray.
P Lancet. P Capillary gas tube. P Sharps bin. P Plaster.
P Gauze/cotton wool.
P Capillary tube caps/clot catcher (if available).
P Vaseline.
P Blood gas machine.
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VASCULAR ACCESS
STEP-BY-STEP GUIDE
2 3
Introduce oneself to the patient/ family and explain the purpose of the procedure. Wash hands and put on gloves. Identify the best place to perform the test: › In a neonate, this will be a heel prick. This means selecting a spot around the periphery of the inferior aspect of the heel. Steer clear of the centre of the heel, where there are fewer capillaries and more nerve fibres (Figure 2).
8
9
10
11 Lateral plantar artery Medial plantar artery Puncture site
Figure 2. Puncture sites for a heel prick. Avoid the centre of the heel.
12
4
5
6 7
› In a child over six months, it may be easier to use the lateral aspect of the thumb, fingers or toes. Gently rub the site and/or gently push down at the intended site a few times to improve capillary blood flow. Check for any previous site of capillary blood sampling that will still bleed without requiring another piercing of the skin. Place a paper towel under the intended puncture site to catch any spillages. Clean the area and allow it to dry (Figure 3). Apply a thin layer of Vaseline over the area to be punctured (note this is optional and not used in some neonatal units). This will ensure that the blood stays in a bleb around the needle prick site rather than smearing elsewhere, making it easier to collect.
13
14
15
16
17 18
Take a mildly firm hold of the heel or digit and stretch the skin. Use the lancet to pierce the desired area (Figure Figure 4). Gently squeeze the area rhythmically, starting distally from the site of puncture and allowing reperfusion between squeezes. This should produce a steady bleb of blood from the site. Hold the capillary tube horizontal or a little elevated. Holding it at a downhill angle may mean air bubbles are sucked in as the blood is collected quicker than it is produced. Holding it level or “uphill” from the patient will prevent this (Figure 5). Gently move the end of the capillary tube to the bleb of blood and fill it to the end. Aim to only collect blood if it is free flowing so that clots are less likely to enter the capillary tube. If air bubbles enter, empty the capillary tube up until the point of the air bubble on gauze and refill the tube. Once filled sufficiently, put the gas tube on a tray. Wipe the puncture site with some cotton wool or gauze to remove any remaining blood and any Vaseline (Figure 6). Apply a plaster if needed (Figure 7). Note that this will not stick if Vaseline is still present. If available, put a cap on both ends of the capillary tube as this will prevent spillage on transfer to the gas machine. Thank the parents/patient and take the sample to the blood gas machine. Remove the caps from the gas tube and put a clot catcher on the end to be inserted into the gas machine (if required). Place lancet and capillary tube in a sharps bin. Once the gas result has been obtained, immediately record it and make a plan based on the findings.
Figure 3. Paper towel placed under foot and skin surface cleaned.
PRACTICAL SKILLS
1
Figure 4. Tightened skin punctured with lancet.
Figure 5. Collect blood in the capillary tube. Note the capillary tube is held horizontally, not down-slanting.
Figure 6. Compress to stop bleeding with cotton wool.
Figure 7. Apply plaster over site.
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Practical Skills
Note that this technique can also be used for sending blood samples for biochemistry and haematology testing. It is neither suitable for blood cultures as the blood sample is contaminated by the skin, nor for clotting studies as clotting factors are activated following skin contact. Blood bottles can be adapted by having a “scooper” on top. This makes it easier to collect the blood as it emerges.
COMPLICATIONS TOP TIPS
This is generally a safe procedure, but complications may include: • Infection. • Pain, particularly with prolonged squeezing of the heel. • Erroneous results from prolonged squeezing of a poorly perfused periphery (classically, erroneous hyperkalaemia or high lactate). • Soft tissue injury. • Rarely, bone infection/injury (from puncturing the calcaneus, particularly if central heel punctured).
• Vaseline is very helpful at preventing blood spillage and making it easier to “catch” in the capillary tube. However, some hospitals may not use Vaseline because of a possible risk of infection. If Vaseline is used, ideally each baby should have their own tube to minimise this risk. • A common mistake is to hold the foot of a neonate tightly immediately after pricking it. After the initial prick, allow the child to kick their foot a little as this will promote blood flow and greatly settle the patient. • Remember that blood is subject to the laws of gravity. Positioning the foot as low as possible may aid collection.
Venous Blood Sampling Venepuncture is performed by several different techniques in paediatrics. In older children, the technique is exactly the same as for adults. In neonates and younger children, because the veins are much smaller, using adult techniques may cause the veins to collapse. Both methods are described below. Unlike with adults, venepuncture is usually very traumatic for a child. Therefore, in some circumstances, it is worth cannulating the child at the same time, as long as this doesn’t compromise taking the blood sample. Taking the blood via a cannula can be more difficult in some children.
Older Child EQUIPMENT CHECKLIST
Indications Indications for venepuncture are wide-ranging, and include: ӹӹ Performing a wide range of bloods tests as an investigation process for many conditions. ӹӹ Checking electrolytes in a child on intravenous fluid therapy. ӹӹ Measurement of drug levels, e.g. gentamicin. ӹӹ Taking a cross-match sample for blood transfusion.
PP Tray.
PP Blood bottles.
PP Non-sterile gloves.
PP Cotton wool.
PP Disposable tourniquet.
PP Plaster.
PP Skin cleansing solution.
PP Sharps bin.
PP Venepuncture needle/ Vacutainer or butterfly needle.
PP Topical anaesthesia (e.g., cold spray, or Ametop cream).
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Vascular Access
STEP-BY-STEP GUIDE
Apply anaesthetic cream (if being used) 30-40 minutes before the procedure (Figure 8). 2 Wash hands and assemble equipment: i. Attach vacutainer to venepuncture needle/butterfly needle (depending on technique) (Figure 9). ii. Place blood bottles in draw order. iii. Cut a piece of tape to size. 3 Put on gloves. 4 Place the child in a comfortable position. This may involve putting a pillow under their arm. In younger children, positioning requires careful thought. The key is to immobilise the child, prevent them from watching the procedure and to facilitate distraction by having them look in another direction (Figure 10). 5 Place the tourniquet 7-10 cm proximal to the proposed insertion point (Figure 11). 6 Select the vein. This can be done by inspection and palpation. The antecubital fossa may be the best site to use, ideally in the nondominant arm. However, in smaller children, it may be easier to hold their arm extended and access the veins on the dorsum of the hand. This is an easier position for keeping a child restrained. 7 Clean the site using the skin cleansing solution and then leave it to adequately dry. 8 If cold spray is being used, apply this now, in and around the intended puncture site (Figure 12). 9 Puncture the vein, making an effort not to re-palpate the site around the vein which has just been cleaned. 10 Draw blood by placing the blood tubes in the vacutainer (Figure 13). Invert the bottles. A similar technique is used with butterfly needles (Figure 14). 11 Remove the tourniquet (Figure 15). 12 Remove the needle and immediately dispose of any sharps.
13 Apply pressure to the puncture site with cotton wool until bleeding stops. Then secure it with tape. 14 Label bottles at the bedside. 15 Inform the child/parents to let staff know if they have any concerns, particularly with regard to pain or ongoing bleeding. Let them know that the dressing can be removed after a few hours.
Figure 12. Applying cold spray (left), and awaiting crystal formation (right) before inserting the needle.
Practical Skills
1
Figure 8. Ametop cream applied to skin over the site of a vein.
Figure 13. Insert vacutainer needle and then bottle.
Figure 9. Assembling vacutainer (left), and butterfly needle (right).
Figure 10. Ask a parent or a nurse to help with positioning. Sit the child in their lap, being cuddled, and facing away from the arm. Feed the arm under the holder’s armpit so that the arm is isolated. In a particularly active child, a third person might be required to hold the arm still. Be creative with distraction techniques!
Figure 11. Apply the tourniquet.
Figure 14. Inserting butterfly needle and then attaching the bottle.
Figure 15. Remove the tourniquet as soon as last bottle of blood collected.
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Practical Skills
Technique in a Neonate/Infant Note that this technique can be modified by using a butterfly needle attached to a syringe or by using a needle and syringe to draw blood.
STEP-BY-STEP GUIDE
1
2 3
EQUIPMENT CHECKLIST
PP Tray.
PP Blood bottles.
PP Non-sterile gloves.
PP Cotton wool.
PP Skin cleansing solution.
PP Sharps bin.
PP 23 gauge needle.
4
PP Plaster. PP Sucrose (if available).
5
COMPLICATIONS
• Infection. • Pain. • Soft tissue injury, e.g. skin extravasation. • Venous thrombosis. • Anaemia (if large recurrent blood samples are taken).
6
7 TOP TIPS
• In an older child who has had multiple blood tests before, it can be helpful to ask them if there are any sites that are particularly good for blood sampling. • In a sick baby, it may be better to obtain blood samples from heel pricks. This saves the veins for cannulation and central lines. If venous blood sampling is needed, it is better to use smaller peripheral veins rather than larger central veins, as larger veins should ideally be saved for cannulation/central lines. • Ensure that a suitable number of helpers are present before starting. Holding the child’s hand and placing lids on the blood bottles can be difficult to do without adequate assistance.
8 9
Introduce oneself to the parents and explain the purpose of the procedure. Wash hands and put on gloves. Select the vein. This can be done by inspection and by palpation. The back of the hand is usually the best site for neonates, as larger veins are often reserved for central lines. Placing some cotton wool in the palm and gently squeezing the hand gives a tourniquet-like effect and will make the veins more prominent. Clean the site using the skin cleansing solution and then leave it to dry (Figure 16). Administer sucrose to the baby as analgesia if available (Figure 17). This could be done by asking the mother to put some on her finger or by putting it on the end of a dummy. Another option is getting the mum to breastfeed during the procedure. Note that some parents don’t like this, because of the association of blood taking with breast feeding. Puncture the vein with the needle. Once in the vein, blood will drip out of the other end of the needle. Collect blood by placing the blood tubes underneath the needle, so that the blood drips into the tubes (Figure 18). For obtaining blood cultures, a separate needle and syringe should be used to aspirate blood from the back of the needle, and then afterwards put it into a blood culture bottle (follow local blood culture taking protocol). To improve blood flow, the hand may need to be squeezed and released intermittently. Remove the needle, and immediately dispose of all sharps. Apply pressure to the puncture site with cotton wool until bleeding stops (Figure 19), and then apply a plaster if needed (Figure 20).
10 Label bottles at the bedside. 11 Inform the parents to let staff know if they have any concerns, particularly with regard to pain or ongoing bleeding. Let them know that the dressing can be removed after a few hours.
Figure 16. Clean site.
Figure 17. Drip sucrose into the mouth.
Figure 18. Allow blood to gently drip into the bottle.
Figure 19. Apply pressure to stop any bleeding.
Figure 20. Apply plaster.
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VASCULAR ACCESS
Arterial Line Sampling
Indications ӹ Arterial blood gas. ӹ Routine blood tests in patients with an arterial line in place. EQUIPMENT CHECKLIST
P Port cleansing solution, e.g. 2% chlorhexidine.
P 3 x syringes.
P Non-sterile gloves.
P Draw up needle.
P Gauze.
P Sharps bin.
P 0.9% sodium chloride ampoule.
P Tray.
COMPLICATIONS
• Infection. • Haemorrhage. • Thromboembolism. • Hypovolaemia. • Arterial spasm.
TOP TIPS
• Calculate exactly how much blood is required in advance, and then draw up this exact amount from the arterial line. This ensures no blood is wasted, which is particularly important in small preterm babies with relatively small circulating volumes. Smaller babies will often have a chart documenting the volume of all blood taken off the arterial line, so be very careful. • Sometimes, after arterial line sampling, the distal extremity can appear pale/white. Take this seriously and monitor very closely over the next five minutes. Consider asking for senior support or removing the arterial line if the colour has not returned to normal after this time.
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1
Wash hands and put on gloves. Open packaging of syringes. Draw up sodium chloride flush in to one of the syringes using the draw up needle, and then dispose of the needle in the sharps bin. 2 Rotate the three-way tap so that it is open to the sampling port and the patient. The blood pressure reading should disappear from the monitor at this point (Figure 21). 3 Place sterile gauze under the threeway tap of the arterial line. 4 Clean the sampling port with the port cleansing solution. 5 Draw off 2-3 mL of blood from the arterial line into one of the syringes (Figure 22). Draw it off slowly, 0.5 mL at a time. In older children, this blood can be discarded, but in neonates it is replaced. If replacing it, inspect the syringe, ensuring that any air bubbles are expelled. Put the blood-filled syringe into its original packaging, ensuring it remains as sterile as possiblethis blood will be returned to the patient. 6 Draw off the required sample volume in the second syringe. Do this slowly, and keep an eye on the colour of the distal extremities. If they change colour, it may be that blood is being drawn off too quickly and is being “stolen” from the distal blood supply. 7 Replace the first blood sample back into the arterial line, 0.5 mL at a time (Figure 23). 8 Flush the arterial line with the 0.9% sodium chloride, ensuring that no blood is left in the line (Figure 24). 9 Clean the port again with the port cleansing solution. 10 Switch the position of the threeway tap so that the sampling port is closed off again (Figure 25).
Figure 21. Turn three-way tap so that it is open to the sampling port.
PRACTICAL SKILLS
Arterial line sampling is commonly required in NICU/PICU, and represents a common task which junior doctors may be expected to do.
STEP-BY-STEP GUIDE
Figure 22. Draw off blood for replacement, and then, in a separate syringe, draw the blood for sampling.
Figure 23. Return the initial replacement blood.
Figure 24. Give a saline flush, ensuring the line is clear of blood.
Figure 25. Close tap off to port.
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Practical Skills
Intravenous Cannulation
EQUIPMENT CHECKLIST
Although cannulation is done for the same indications in both children and adults, it can be much more difficult in children. This is because, particularly with younger children, co-operation can be an issue. It is important to ensure before the procedure takes place that the child is adequately distracted, a strategy is in place for analgesia and the proposed cannulation site is immobilised.
Indications ӹӹ Intravenous fluids (maintenance, replacement or bolus). ӹӹ Intravenous medications.
PP Skin cleansing solution. PP Draw up needle. PP Syringe. PP 0.9% saline ampoule. PP Sterile adhesive dressing. PP Cannula. PP Disposable tourniquet. PP Cotton wool. PP Non-sterile gloves. PP Tray. PP Sharps bin. PP Splint and splint bandage (depending on age). PP Intravenous extension set (+ bung/connector). PP Gauze.
STEP-BY-STEP GUIDE
Preparing the Equipment 1 2 3 4 5 6
Wash hands and put on non-sterile gloves. Attach the draw up needle to the syringe. Remove the top from the 0.9% sodium chloride and draw it up into the syringe (Figure 26). Expel any air from the syringe by tapping it or advancing the plunger. Discard the needle in the sharps bin and flush through the intravenous extension set (Figure 27). Leave the flush connected to the intravenous extension set and place it in the equipment tray alongside the other cannulation equipment.
Figure 26. Draw up the flush.
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Figure 27. Prime the intravenous extension set.
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VASCULAR ACCESS
STEP-BY-STEP GUIDE
1
2
3 4
5
6 7
8
9
10 11
12 13
Remove the cannula from its packaging and open the packet of sterile gauze. Place the tourniquet approximately 7-10 cm proximal to the site of insertion. Select an appropriate vein. Clean the proposed site using skin cleansing solution and then leave it to dry. Tether the chosen vein beneath the insertion site and insert the cannula at approximately 15⁰ using an aseptic technique (Figure 28). Advance the cannula until flashback is obtained. Once flashback has been seen, advance the cannula slightly further (1-2 mm). This ensures the cannula tubing is within the vein before advancing it forwards, over and off the needle. Hold the needle still and advance the cannula over it, all the way into the vein. No part of the cannula tubing must be seen at the point of entry. Occlude the vein proximal to the cannula insertion site with firm pressure and then gently remove the needle (this will reduce blood loss when the needle is removed from the tubing) (Figure 29). Dispose of the needle straight into the sharps bin. Take off any blood samples needed from the end of the cannula (Figure Figure 30). Release the tourniquet. If the child is very mobile or vigorous, it may help to secure the cannula in place at this stage with one piece of tape as a temporary measure. It can be
14
15 16 17
18
19
20
formally secured once its position is confirmed. Attach the intravenous extension set to the end of the cannula (Figure 31). Slowly flush the cannula through the extension set, 1 mL at a time, checking to see that the fluid is not going into subcutaneous tissue, and that there is minimal resistance to pushing the fluid through the cannula (Figure 32). As the last millilitre goes through, clamp the extension set. Dispose of the syringe in the sharps bin. Wipe away any blood that may have leaked around the cannula with sterile gauze. Apply tape and sterile adhesive dressing (Figure 33). Use tape and cotton wool to further secure the cannula even more if needed. Apply a splint to the cannula and thoroughly bandage it so that it is inaccessible to the child. Remove gloves and wash hands.
Figure 30. Collect blood sample.
PRACTICAL SKILLS
Inserting the Cannula
Figure 31. Attach IV extension set.
Figure 32. Flush cannula.
Figure 28. Insert cannula.
Figure 29. Occlude vein while removing needle.
Figure 33. Secure using tape (top) and sterile adhesive dressing (bottom).
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PRACTICAL SKILLS
COMPLICATIONS
• Infection. • Pain. • Soft tissue injury, e.g. skin extravasation. • Venous thrombosis.
TOP TIPS
• Children will often attempt to pull cannulas out. Do not leave until the cannula is definitely stuck down and covered. • Adhesive dressings may not stick to sweaty skin if the child is hot and vigorous. Using a barrier preparation such as Cavelon can overcome this. Apply the barrier preparation to the skin before sticking the adhesive dressing on top. • One of the most common reasons for failing to insert a cannula is choosing the wrong vein. Spend time assessing the best veins on each limb. Remember also to select a vein which can be easily immobilised – choosing veins overlying joints (e.g. wrist or antecubital fossae) may prove difficult due to the child’s movements.
Umbilical Venous and Arterial Catheterisation Umbilical venous catheterisation (UVC) is a relatively common procedure in neonates and is usually relatively straightforward with practice. UVCs can be inserted in the first week of life, although the earlier the better, due to the declining patency of the umbilical cord. Umbilical arterial catheterisation (UAC), if needed, is usually done at the same time as inserting the UVC. This is often more difficult, as the artery is more muscular, with a smaller lumen.
Indications UVC ӹ For administration of TPN in small babies. ӹ For giving high glucose concentrations to hypoglycaemic babies. ӹ For administration of certain drugs that may be poorly tolerated peripherally, e.g. dopamine. ӹ For performing exchange and partial exchange transfusions. EQUIPMENT CHECKLIST
• If struggling to find a vein, revert to anatomical sites common to almost all children. Consider the long saphenous vein anterior to the medial malleolus or “houseman’s vein” on the lateral border of the wrist. In neonates, remember that scalp veins may yield extra unutilised possibilities. • In a non-emergency situation, after two unsuccessful attempts at peripheral cannulation, ask a more experienced colleague to attempt cannulation. • In emergency situations, after three unsuccessful attempts at peripheral cannulation, attempt intraosseous access instead.
Ensure sterility:
P Umbilical tape.
P Sterile gown.
P Scalpel.
P Sterile towel.
P Sterile drapes.*
P Sterile gloves.
Inserting the UVC and UAC:
Preparing to insert the UVC and UAC:
P Fine non-toothed forceps.*
P Tape measure.
P Blunt end dilator.*
P 2 x appropriately sized umbilical catheters.
P 2 x artery forceps.* P Sterile gauze.*
P 2 x three-way taps.
Securing the catheter:
P 2 x 5 mL syringe.
P 2 x sutures.
P 2 x 0.9% sodium chloride ampoule.
P 2 x Steri-Strips.
P Draw up needle. P Skin cleansing solution, e.g. 0.05% chlorhexidine gluconate aqueous solution (appropriate strength to avoid chemical burns).
P Scissors.* P Suture holder.* *May all be included in a blunt dissection pack
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VASCULAR ACCESS
UAC ӹ Frequent arterial blood gas measurement/blood sampling. ӹ Continuous blood pressure reading. ӹ Exchange transfusion.
Contraindications Exomphalos is a contraindication, but otherwise there are very few absolute contraindications. The process for inserting a UVC and a UAC at the same time is described below.
PRACTICAL SKILLS
ӹ For immediate access in an emergency, when peripheral cannulation is difficult.
STEP-BY-STEP GUIDE
Preparation 1
2
3
Lay the baby supine, and estimate the length of the two catheters (Figure 34). Wash hands, dry them with a sterile towel, and put on a sterile gown. Prepare the UVC and the UAC. i Attach the three-way tap to the catheter. ii Draw up the sodium chloride in the 5 mL syringe using the draw up needle (Figure 35), and prime all the ports of the catheter, making sure it flushes all the way through (Figure 36). Immediately dispose of the draw up needle in the sharps bin.
UVC UAC
Stum Stump length
iii Leave the catheter in the sterile packaging, ensuring that the distal end is not open to the atmosphere.
4 5
6
Figure 35. Drawing up the flush.
Clean the umbilical cord area with the skin cleansing solution. Place the sterile drapes around the umbilicus, leaving the feet/ head exposed. Clean again (Figure 37). Tie the umbilical tape around the base of the umbilicus (Figure 38). This helps keep the cord upright, and also minimises any blood loss. Be careful not to make it too tight, as this may make passing the catheter harder.
Figure 37. Create a sterile field and, while holding the cord with sterile gauze, clean around it.
b a
Figure 34. Cord lengths: UVC: stump length + b. UAC: stump length + 2a + b: a= distance from umbilicus to midinguinal point. b= distance from umbilicus to xiphoid.
Figure 36. Priming the arterial line (red) and the venous line (blue) with saline. Note both ports of both catheters need to be flushed through.
Figure 38. Tying the cord at the base.
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PRACTICAL SKILLS
STEP-BY-STEP GUIDE
Inserting the Umbilical Lines 1
2
3
4
Cut the umbilical cord with the scalpel in one clean motion. Try to make the cut surface as even as possible (Figure 39). It is important that the cord is not too long or too short. The longer the cord, the more difficult it is to insert the catheter. If the cord is too short, then there will not be space for a second attempt (i.e. cutting the cord again). Approximately 2-3 cm from the umbilicus is ideal. Identify the vessels (Figure 40). The umbilical vein is the single, thin walled, peripheral, larger vessel. There are two arteries, which are muscular with a narrower lumen. Massage the cord from the base to the top to remove any debris in the vessels. Use gauze to stop any bleeding. Pick the more favourable umbilical artery to attempt cannulation in first. Arterial forceps can be used to hold the cord upright. Any umbilical artery is likely to require prolonged, deep dilatation before proceeding, so do not rush. To dilate, use (Figure 41): i. Fine non-toothed forceps or arterial forceps. To dilate the opening very superficially. ii. Blunt ended dilators. To dilate the vessel more distally. Advance the first catheter into the umbilical artery up until the premeasured level. Non-toothed forceps can help give greater control (Figure 42). Check that blood aspirates into the syringe (Figure 43). Problems may arise: i. By having the umbilical tape too tight. In this case, loosen the tape, and then re-advance. ii. By the umbilical cord being too mobile. In this case, hold it in place with one or two artery forceps (held by the non-dominant hand).
5
6
7 8
iii. By creating a false passage, with the catheter going through the vessel wall, and into the stump. In this scenario, it is helpful to recut the cord more proximally and try again. iv. At 1-2 cm. Where the umbilical artery turns downwards. Use gentle pressure, and also try turning the umbilical stump towards the baby’s head, to straighten the vessel. v. At 4-5 cm. May be due to spasm/ kinking at the iliac vessels. Overcome with gentle pressure. Inspect the baby’s legs/toes: if they are blue or white, the catheter may be obstructing blood flow, and therefore needs to be removed. Ensure haemostasis has been achieved. A second umbilical tape may be needed in some cases. Secure the catheter (see “Securing the Catheter”). Grasp the umbilical cord with the curved artery forceps to hold it upright and straight again. Reinspect the umbilical vein. It may be that a catheter can be inserted into
Figure 41. Hold the cord up using artery forceps if needed. Dilation of the vessels can be achieved with a blunt ended dilator (top), fine non-toothed forceps (middle), or arterial forceps (bottom).
Figure 42. Advancing the UAC. Note that the syringe is still attached. Figure 39. Cut the cord as close to the clamp as possible, approx 2-3 cm from the skin. 2 x Arteries (Feet end)
1 x Vein (Head end)
Figure 40. Identify the vessels: two narrow muscular arteries (feet end) and one thinner walled vein (head end).
Figure 43. After reaching measured length, aspirate blood.
Continued
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Vascular Access
STEP-BY-STEP GUIDE
1-2 cm beyond the abdominal wall. Gentle traction should overcome this. ii. Getting coiled up/wedged in the portal system. Pull back part of the way and re-advance. 10 Secure the catheter (see “Securing the catheter”), ensuring that the sutures remain entirely separate. As with the UVC, check after insertion that the babies legs/toes haven’t changed colour (if so the catheter
Figure 44. Repeat with UVC, ensuring blood aspirates.
will need to be removed). Ensure haemostasis has been achieved.
Practical Skills
9
the vein with no manipulation of the vessel: it is usually much easier than for the artery. Prepare the umbilical vein for catheterisation if needed, as done with the artery. Advance the second catheter into the umbilical vein, up until the premeasured level, ensuring blood aspirates (Figure 44). Problems may arise: i. Meeting a kink in the umbilical vein. This may occur
STEP-BY-STEP GUIDE
Securing the Catheter 1
2
3
Using the suture holder, pass the suture through the stump of the cord, avoiding the vessels. Tie a secure knot to firmly fix the suture to the stump (Figure 45). Loop the suture around the catheter, and then tie another knot, ensuring there is minimal slack in the suture (Figure 46). Repeat this again, two or three times, at 0.5 cm increments, giving a total of three or four knots (Figure 47). Gently pull on the catheter to ensure that it is secure, and then cut the suture with the scalpel, leaving
Figure 45. Anchor suture to cord with first knot.
4
tails of approximately 1 cm on either end (Figure 48). Use steristrips to tape the knots against the cord, ensuring that
5
the sutures are taught whilst stuck down (Figure 49). Gently pull on the catheter again to check that it is secure.
Figure 46. Tie second knot around base of catheter to secure it.
Figure 48. Cut the loose thread.
Figure 47. Tie knots approximately every 0.5cm.
Figure 49. Use Steri-Strips to fix the knots to the catheter.
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Practical Skills
STEP-BY-STEP GUIDE
COMPLICATIONS
• Infection.
Finishing off 1
2
To keep the lines patent, an infusion of fluid should be set up to run at a slow rate, e.g. 0.5-1 mL/hr of 0.9% sodium chloride. Perform an X-ray to check the position of the two catheters. The UVC goes straight up, whereas the UAC dips down first towards the pelvis, before going up. The UVC should be around T9-T10, and the UAC should be between T6 and T10. A low UAC can be between L3-5, but never leave at L1 as this is the level of the renal arteries (Figure 50).
• Haemorrhage.
T5 T6 T7
UVC
T8 T9
• Vessel perforation. UAC
T10 T11 T12
• Advancing the catheter gently but firmly from the tip will help prevent the creation of a false passage. Figure 50. X-ray showing position of umbilical lines. UVC at T10, and UAC at T8 (Reproduced with kind permission from Radiopedia).
• Be very gentle when dilating the vessels: this will also reduce the risk of false passage. • Most clinicians prefer putting the UAC in first. The UAC is technically more difficult, and the cord may need to be cut multiple times to successfully gain access, which is a lot easier if the UVC is not already in situ.
STEP-BY-STEP GUIDE
1 2 3
4
EQUIPMENT CHECKLIST
PP Umbilical catheter. PP 10 mL syringe of 0.9% sodium chloride.
• Cardiac/hepatic haematoma.
TOP TIPS
Indications ӹӹ For fast, central access to administer drugs and fluids (e.g. adrenaline, bicarbonate, sodium chloride, blood), as peripheral cannulation in this scenario can be challenging.
• Portal vein thrombosis. • Cardiac arrhythmia/perforation/ tamponade (if catheter is advanced into the heart).
Emergency Umbilical Venous Catheterisation Insertion of an umbilical venous catheter is a key life-saving skill that may be required during the resuscitation of a neonate. In the emergency setting, this is not a sterile procedure, but once the baby has been transferred to the neonatal unit, this can then be replaced in a sterile manner with a new catheter and secured properly.
• Air/catheter tip embolism.
5
Flush the catheter with sodium chloride. Tie the umbilical tape loosely around the base of the umbilicus. Hold the umbilical cord straight up by holding the cord clamp (which will already have been placed by the midwife/ obstetrician). Using the scalpel, make a single cut across the cord proximal to the clamp. If there is significant blood oozing from the stump, tighten the umbilical tie. Use a piece of gauze to dab at any oozing, and identify the umbilical vein. Dilate the umbilical vein superficially if needed. Insert the
6
catheter 4-5 cm past the umbilical ring and ensure blood can be aspirated before it is used. Make a note of how far the catheter has been advanced and tape it to the abdomen. This can now be used to administer emergency drugs, fluids or blood. If ongoing umbilical access is required, then a definitive umbilical catheter should be inserted in a sterile manner and sutured into place. If not required, the umbilical venous catheter can be gently withdrawn, with the umbilical cord-tie still in situ.
PP Umbilical tape. PP Scalpel. PP Fine non-toothed forceps. PP Gauze. PP Tape.
TOP TIPS
• Do not wait for X-ray confirmation before using this line. It is an emergency and position is confirmed by aspirating blood back.
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VASCULAR ACCESS
Intraosseous Needle Insertion
EQUIPMENT CHECKLIST
P Manual intraosseous (IO) needle or battery operated IO drill. P IO needle (15 mm needle (pink) for patients under 40 kg, or 25 mm needle (blue) for more than 40 kg). P Non-sterile gloves. P Skin cleansing solution. P Adhesive dressing (for securing IO needle). P 5 mL syringe. P Specimen bottle. P Sharps bin. P Equipment tray. P Intravenous extension set: ◊ Intravenous extension set. ◊ Three-way tap. ◊ 0.9% sodium chloride ampoule. ◊ Draw up needle. ◊ 5 mL syringe.
1
Wash hands. Put on nonsterile gloves. 2 Remove the top from the 0.9% sodium chloride and draw it up into the syringe. 3 Expel any air from the syringe by tapping it/advancing the plunger. 4 Discard the needle in the sharps bin and flush through the intravenous extension set (Figure 51). 5 Leave the flush connected to the intravenous extension set, and place it in the equipment tray. 6 Chose the site of access. Usually the anteromedial tibia is the first choice and presents a stable, flat surface (Figure 52). The anterolateral femur is the next most commonly selected site. Avoid areas of infected skin/wounds. Other contraindications include: › Fracture in targeted bone or proximal to site of insertion. › Excessive tissue or absence of adequate anatomical landmarks. › Previous, significant orthopaedic procedure at site (e.g. prosthetic limb/joint). › IO access in targeted bone within past 48 hours. 7 Clean with skin cleansing solution. 8 Immobilise the relevant limb, ensuring that the hand immobilising the limb is not placed under the limb in the process. 9 Insert the IO needle at 90° to the surface of the skin. There should be a loss of resistance (a “give”) on entering the cortex of the bone. This may be done manually (Figure 57) or with a drill (Figure 53). If successful, the needle should be firmly attached. 10 After ensuring the needle is stable, remove the trocar from the needle: immediately dispose into the sharps bin. 11 Attach a syringe, and aspirate bone marrow if possible
(Figure 54). Samples cannot be put through a blood gas analyser and should be sent to the laboratory marked as bone marrow aspirate. Tests that may be performed include: › Red blood cell count. › Chloride. › Haemoglobin and haematocrit. › Total protein.
PRACTICAL SKILLS
Intraosseous needle insertion is the quickest way to give rapid fluid resuscitation in a peripherally shut down child. It also functions as central access, allowing inotropes to be administered in emergency situations. It is usually done in an emergency situation, and this is the process that will be described below. In a conscious child, use of local anaesthetic should be considered before the procedure.
STEP-BY-STEP GUIDE
Figure 51. Prime the intravenous extension set.
Figure 52. Palpate the anatomical landmark.
Figure 53. Insertion of intraosseous needle using the easy IO drill.
Figure 54. Aspirate bone marrow (attempt).
Continued
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PRACTICAL SKILLS
STEP-BY-STEP GUIDE
› › › › ›
Glucose. Albumin. Urea. Lactate. Creatinine. 12 Fix the IO needle with the adhesive dressing (Figure 55). 13 Attach the pre-primed intravenous extension set, and flush through with sodium chloride, checking there is no extravasation (Figure 56). 14 Administer medication/fluids as needed. Infusion of fluids is often
COMPLICATIONS
painful for patients responsive to pain; therefore, following the IO needle insertion, IO anaesthetic (2% preservative-free lignocaine) may be considered for use. 15 Continue at least hourly observations of the access site for possible complications.
• Infection. • Pain. • Skin necrosis. • Fractures.
TOP TIPS
• If a child is coming to ED following cardiac arrest or is significantly compromised, have everything set up to establish intraosseous access, as it is highly likely that it will be needed. • There is nothing to prevent multiple IO needles being put in the same child at various sites; indeed multiple ports of access will frequently be required in an emergency.
Figure 55. Secure with dressing.
Figure 56. Attach intravenous extension set.
• Extravasation of fluid/medication, which can lead to compartment syndrome.
Figure 57. Same procedure using the manual intraosseous needle. Inserting the needle by twisting and pushing downwards (top), removing the trocar (middle) and attaching the intravenous extension set (bottom).
AIRWAY PROCEDURES
• Intraosseous samples should never be run in a blood gas machine (the machine will break). If sending to the lab, the sample must be very clearly labelled so the laboratory staff can process it appropriately (if they think they are looking at a blood film, they are likely to report a leukaemic picture with the large number of blasts present!).
EQUIPMENT CHECKLIST
Bag-Valve-Mask Ventilation Bag-Valve-Mask (BVM) Ventilation is a life-saving method of administering non-invasive positive pressure to inflate the lungs. The BVM’s greatest attribute is that it is portable and does not require a gas supply to function.
Indications ӹ Respiratory arrest. ӹ Hypoventilating patient, i.e. a patient who is breathing either too slowly or too shallowly to be effective.
P Face mask (sizes 0, 1 and 2). P Self-inflating bag, with valve and sometimes a
reservoir bag (which can be inflated by attaching to an oxygen supply if available).
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AIRWAY PROCEDURES
STEP-BY-STEP GUIDE
2
3
Pick the correct size mask (Figure 58). A tight seal is crucial. The mask should cover the nose and mouth, without pressing on the eyes (which may promote a vagal response or cause local trauma) or overhanging at the chin. Pick the correct size bag. There are generally three sizes of self-inflating bag; infant, child and adult. In larger adolescents, use the adult size. Two possible techniques can be used: a One person technique (Figure 59):
i Place the face mask over the nose and mouth, creating a good seal and being careful not to press over the eyes. ii With the left hand, create a C-shape with the thumb and forefinger. Place this on top of the mask, pressing it downwards onto the face. iii The third, fourth and fifth fingers should be used to support the mandible. Be careful not to compress the soft tissues of the upper airway when performing this manoeuvre, by ensuring that fingers are placed on the bones of the jaw only (Figure 59). Some people are able to simultaneously perform a jaw-thrust; this needs practice. 4
Figure 58. Sizing the mask: too small (top), correct size (middle), too big (bottom).
COMPLICATIONS
• Pneumothorax. • Aspiration (from stomach inflation).
Figure 59. C (thumb and index finger) and E (little, ring and middle finger) grips, while ventilating a baby using a bag-valve-mask (top), and using a T-piece (bottom).
iv Compress the bag with the right hand. Watch for chest wall rise and listen for leak around the edge of the mask. The bag may only need to be compressed by a third or half of its volume for each breath. Adjust positioning as required. b Two-person technique: this makes it easier to adequately create a seal, and to utilise the jaw thrust (Figure 60): i Using both hands, press the mask down onto the face with the thumbs (+/- index fingers). ii The other fingers can then perform a jaw-thrust on both sides. iii The second person can then compress the bag. Consider the need for a definitive airway or other forms of ventilatory support (invasive or non-invasive). If the patient is spontaneously breathing, they may require high flow oxygen, or alternatively in the acute newborn situation, they may recover very quickly and selfventilate in air.
PRACTICAL SKILLS
1
Figure 60. Two person technique.
TOP TIPS
• It can be helpful to place an oropharyngeal airway (Guedel airway) first, as this will draw the tongue forwards and prevent it from obstructing the airway. This will only work if the patient is sufficiently unconscious to not have a gag reflex. • Bag-valve-mask ventilation can be a tiring process, so ask a colleague to take over when necessary. • Make sure that when holding the mask over the patient’s face, the bag is actually being compressed; otherwise no air/oxygen will be delivered to the patient, effectively suffocating them (the valve is closed at rest).
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Practical Skills
Nasopharyngeal Airway Insertion Airway adjuncts are used in children with reduced consciousness and those that are difficult to ventilate. They are simple, effective ways to open the airway. Below,
nasopharyngeal (NP), oropharyngeal (OP) and laryngeal mask airways are described. A NP airway comprises a flange, body and bevelled opening (Figure 61).
STEP-BY-STEP GUIDE
1
Correctly size the NP airway (Figure 62). The size is indicated by the number printed on the side of the airway. Make sure the flange is large enough to anchor the NP airway in the nose. To be correctly sized, the bevelled opening should fit comfortably into the nostril, and the length should approximate the distance from the tragus of the ear (or angle of the jaw) to the nostril. The other way of sizing the tube is choosing a diameter that
2 3
4
is approximately the same size as the diameter of the patient’s little finger. Put lubricant on the bevelled end of the NP airway (Figure 63). Introduce the NP airway (bevel end first) into one of the nostrils and advance it along the floor of the nasal passage (Figure 64). Continue until only the flange remains at the opening of the nasal passage.
Figure 61. An NP airway, and lubricant.
Figure 63. Lubricate the end of the NP airway.
Figure 62. Sizing an NP airway from the nostril to the angle of the jaw (above) and comparing to size of little finger (below).
Figure 64. Inserting the NP airway.
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Airway Procedures
Oropharyngeal Airway Insertion
STEP-BY-STEP GUIDE
1
2
Correctly size the OP airway (Figure 66). They are colour coded according to size. To be correctly sized, the OP airway should measure from the angle of the jaw to the middle of the incisors. Insert the OP airway, holding it by the flange, into the mouth
Figure 65. Range of sizes of OP airways.
(Figure 67). Initially, advance it upside down to avoid pushing back the tongue. Once in, rotate by 180 degrees. In neonates/small children, insert it with a laryngoscope/tongue depressor pushing down the tongue, with the OP airway in the direction it will sit, i.e. pointing downwards.
3
This also provides the opportunity to examine the oropharynx for foreign material. Advance it into the mouth until the bit block rests between the teeth.
Practical Skills
An OP airway comprises a curved tube with a bit block and a flange (Figure 65).
Figure 66. Sizing a guedel. Left: too short. Right: still a little short of the angle of the jaw, but much better fit.
Figure 67. Inserting the OP airway: in an older child, insert it upside down (left) and then twist round 180 degrees to put in final resting position (middle). Once in, ventilate as normal (right).
Laryngeal Mask Airway A laryngeal mask airway (LMA) is an example of an advanced airway (Figure 68). It is a supraglottic airway
device. These airway devices offer increased airway protection compared to NP/OP airways, as they sit above the larynx.
STEP-BY-STEP GUIDE
1 2
3 4
Deflate and lubricate the cuff (Figure 69). Insert the LMA, holding it like a pen, until resistance is felt at the back of the pharynx (Figure 70). Inflate the cuff using room air (via a syringe) (Figure 71). Ventilate with the LMA in place, assessing for breath sounds and chest movement to ensure the LMA is in a good position (Figure 72).
Figure 68. LMA (inflated) attached to syringe.
Figure 69. Deflate the cuff before starting.
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Practical Skills
STEP-BY-STEP GUIDE
Another example of a supraglottic airway device is the iGel. It is inserted held like a pen, until resistance is felt at
Figure 71. Inflate the cuff.
the back of the pharynx. An iGel has the advantage of allowing an NG tube to be passed through it.
Figure 72. Ventilate.
Figure 70. Insert the LMA like a pen.
Neonatal Intubation Neonatal intubation of term and pre-term neonates is a key skill for any paediatric trainee. It can be performed with or without premedication. Premedication is preferred to make the procedure easier, and to reduce the cortisol response in the neonate. Common pre-medications include morphine, atropine and suxamethonium. Sometimes, in an emergency, there is no time to retrieve, prepare and give drugs in advance.
Indications ӹӹ Failure of oxygenation. ӹӹ Failure of ventilation (CO2 clearance). ӹӹ To facilitate the administration of surfactant. ӹӹ Specific conditions requiring immediate intubation at birth, such as congenital diaphragmatic hernia. ӹӹ To secure the airway during resuscitation (if not able to move the chest despite airway manoeuvres or if the resuscitation is prolonged).
EQUIPMENT CHECKLIST
PP Correctly fitting facemask. PP T-piece (attached to pressure limited gas flow device, e.g. neopuff), or selfinflating bag. PP Endotracheal (ET) tube (ideally three sizes, to cover a possibly larger and smaller baby than expected). PP Introducer (stylet). PP Appropriately sized straight blade (00, 0, 1) attached to a working laryngoscope. PP Suction circuit and suction catheter. PP Stethoscope. PP Oxygen saturation monitor. PP Scissors. Method for securing tube (one example is shown below, but many exist): PP Hat with strings either side. PP Soft plastic flange, with a hard plastic clip. PP Forceps (to close the clip). For premedication: PP IV access.
PP Premedication drugs: note that these vary between units, but one combination commonly used is: a. Atropine (to treat any vagal response to intubation). b. Suxamethonium (muscle relaxant). c. Fentanyl or morphine (analgesia). An assistant is essential to: PP Keep an eye on any monitoring. PP Pass the suction catheter. PP Pass the ET tube. PP Administer cricoid pressure if needed. PP Confirm placement of the tube with a stethoscope. Capnometer (end-tidal CO2) may help confirm the position of the ET tube. However, it may be misleading, particularly if there is reduced cardiac output or failure of adaptation of the neonatal circulation.
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Airway Procedures
STEP-BY-STEP GUIDE
1
2
3
4
Put the introducer in the ET tube, ensuring that the introducer is not protruding from the end of the tube (where it may cause tissue damage). Wrap the introducer around the hard plastic connector of the ET tube to ensure it is secure (Figure 73). Ensure all equipment is working, including laryngoscope, light source, air/O2, and suction circuit. Check that the cannula is working. Draw up all premedication in advance. If there is an NG tube in place,
5
6
aspirate the stomach contents. Position the neonate on a flat surface, head in the midline, with the neck slightly extended. In a non-emergency intubation, optimise ventilation by using
7
intermittent positive pressure ventilation (e.g. with a bag-valvemask) and aim to fully saturate the baby before beginning. Suction any visible secretions from the mouth.
Practical Skills
Preparation
Figure 73. Putting introducer into ET tube.
STEP-BY-STEP GUIDE
Inserting the ET tube 1
2
3
4
5
Place the laryngoscope into the oropharynx. Initially place it low, putting it over the oesophagus. Gently pulling out anteriorly should reveal the vocal cords in the superior part of the view (Figure 74). An assistant pushing on the cricoid may be needed to bring the cords into view. Suction any blood/secretions that may be present around the vocal cords. Ask for the ET tube, and advance it through the cords. There is a black mark on the ET tube, which should just go through the cords (Figure 75). Hold the tube firmly against the hard palate so that it does not move while removing the introducer (Figure 76). Look for chest wall movement, and ask an assistant to listen for equal air entry. If there is more marked air entry on one side, gently withdraw
ET tube Laryngoscope
Epiglottis
Trachea Oesophagus Lungs
Figure 74. Visualising the vocal cords (with ET tube ready) (top left). Note the white vertical cords on either side of the airway (top right). Aim straight between these with the ET tube, with the tube finishing below the cords (bottom).
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PRACTICAL SKILLS
STEP-BY-STEP GUIDE
the tube by half a centimetre at a time until the air entry is equal. If the saturations improve, this is a good indication of correct position. Be careful not to extubate the baby.
Figure 75. Inserting the tube.
Figure 76. Holding the tube against the hard palate whilst the introducer is removed.
STEP-BY-STEP GUIDE
Securing the ET Tube Described below is one common method of securing an ET tube. Different units use different techniques, including specially designed adhesive tapes and, in older children, the Melbourne strapping technique using Elastoplast. 1 Keep a secure hold on the tube whilst an assistant passes the soft plastic flange over the ET tube. Use the forceps to clamp the clip on the flange tight around the ET tube (Figure 77). 2 Attach a capnometer (if available) to the ET tube to check that CO2 is being expelled (Figure 78). 3 Attach the ET tube to a ventilator circuit (or to a bag and mask). 4 Cut the tube to a reasonable length to reduce dead space (and resistance of the tube). 5 Tie the strings of the hat to the flange, securing the ET tube to the head (Figure 79). The tube is now secure and it no longer has to be held against the hard palate. 6 Auscultate again to check the tube is still in the correct position (Figure 80).
Figure 77. Placing flange over the tube, and tightening it in place.
Figure 78. Attaching CO2 monitor, and then the T-piece to connect to the ventilator. Note that the tube is firmly pushed against the hard palate as it is still not secure.
Figure 79. Tying the ET tube to the cap, and then releasing grip on the hard palate once secure.
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Airway Procedures
STEP-BY-STEP GUIDE
Arrange a chest X-ray to assess the position of the tube. The tip of the ET tube should be 1 cm above the carina. If the carina cannot be visualised, then a position of T2-3 is adequate (Figure 81).
Practical Skills
7
ET tube
T4 T5 T6 T7 T8 T9 T10
NG tube
T11 Figure 80. Final position confirmation by auscultation.
T12
ET tube T2 T3 T4 T5 T6
COMPLICATIONS
T7
• Laryngospasm.
T8
• Pneumothorax.
T9
• Oesophageal intubation.
T10
• Haemorrhage.
T11
• Hypotension (from vagal stimulation).
T12
NG tube
• Lung collapse (from ET tube being too far advanced). • ET tube obstruction (e.g. from blood/secretions). • Oedema of the cords. • Cord paralysis.
Figure 81. X-ray confirming ET Tube position. TOP: Low position at T4 (although may be acceptable as head flexed during X-ray). BOTTOM: High position at T1.
• Lung damage from barotrauma.
TOP TIPS
• Don’t rush the procedure. Take time trying to visualise the cords before taking the ET tube. Have an assistant on hand to watch the monitor and keep a close eye on the saturations. • Take no more than 30 seconds for each intubation attempt before withdrawing and re-oxygenating. • The person holding the tube against the palate should have no other job. Otherwise, the tube is at risk of slipping in either direction. • If possible, pass an NG tube first, as this will make the position of the oesophagus clear and can be used as a guide (as well as deflating the stomach).
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• It may be tempting to try to advance the tube without having a good view of the cords. Do not do this, and instead ask a more experienced colleague to attempt the procedure. It will risk laryngospasm, local airway trauma and potentially a prolonged period of hypoxia. • Consider “plan B” regarding any difficult airway. Can a smaller tube be used? Does a different size laryngoscope blade need to be used? Identify who can be called for help (PICU, anaesthetics, paediatric registrar covering another area of the hospital). Have an appropriately sized bag-valvemask ready.
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Practical Skills
Surfactant Administration Surfactant is administered via an ET tube in neonates. It significantly improves outlook in respiratory distress syndrome by increasing the compliance of premature lungs. Usually it is done in babies that are already intubated, but some centres will intubate purely to give surfactant and then extubate. Other indications are shown below.
Indications ӹӹ Respiratory distress syndrome. ӹӹ Meconium aspiration. ӹӹ Pulmonary haemorrhage.
EQUIPMENT CHECKLIST
PP Surfactant vial. PP Surfactant administration kit (surfactant tube, syringe, vial adaptor). PP Sterile gloves. PP Sterile scissors. PP Bag-valve-mask/T-piece for ventilation.
STEP-BY-STEP GUIDE
Preparation 1 Confirm ET tube position, place the baby supine, and check that the bag-valvemask or T-piece is working correctly. 2 Suction through the ET tube if needed to clear any secretions. 3 Calculate and prescribe the appropriate surfactant dose. 4 Check the length of the ET tube, and note this down.
STEP-BY-STEP GUIDE
Drawing up the Surfactant 1 2 3
4
Place all equipment in the sterile area. Wash hands and put on sterile gloves. Cut the surfactant tube to the required length for the ET tube. It should be long enough to reach the bottom of the ET tube. The surfactant tube can be folded in half to enable measurement from markings already on it. Attach the vial adapter to the surfactant vial, and ask an assistant to turn this upside down. Draw up the required surfactant volume into the syringe (Figure 82).
5 6
Attach the syringe to the tube (Figure 83). Check again that the baby is optimally ventilated, with a good heart rate and good oxygen saturations.
Figure 82. Drawing up surfactant with assistant.
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Asthma
STEP-BY-STEP GUIDE
8
9
Ask the assistant to disconnect the ventilator from the baby, and quickly feed the surfactant tube down the ET tube. Rapidly put the surfactant tube down the ET tube, and administer the surfactant (Figure 84). As soon as it is administered, ask the assistant to give intermittent positive pressure ventilation to the baby. Once the baby is stable, reattach them to the ventilator. Ensure a blood gas is repeated within an hour of giving any surfactant, as the ventilator requirement can rapidly change as the surfactant takes effect. The ET tube should also not be suctioned for at least an hour post surfactant administration, unless there is a clinical concern of obstruction.
• If giving a large volume of surfactant, divide it into two doses, and give intermittent positive pressure ventilation in between.
Figure 83. Surfactant ready to administer.
Practical Skills
7
TOP TIPS
Figure 84. Administering surfactant.
ASTHMA
EQUIPMENT CHECKLIST
Asthma Inhalers The different varieties of inhalers can be confusing. A good practical summary can be found on most asthma association websites. Most inhalers in hospital will be the traditional metered dose inhalers, so their use will be described here. They should be used in conjunction with a spacer to optimise delivery, in all age groups.
Indication Treatment of reversible obstructive airways disease (predominantly asthma, viral-induced wheeze and bronchiolitis).
PP Inhaler. PP Spacer +/- mouth piece. PP Assistant to help hold younger less cooperative children.
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Practical Skills
STEP-BY-STEP GUIDE
1
2
Shake the inhaler well and place into the end of the spacer (Figure 85). The next steps depend on the age of the child:
Figure 85. Assembling the spacer.
›› In an older child, ask them to exhale completely and to then put their mouth firmly over the mouthpiece with a tight seal. Press the canister to release a dose into the spacer and then ask the patient to take one slow breath in and hold for ten seconds (or as long as comfortable) (Figure 86).
Figure 86. Taking one slow breath in after the canister has been pressed.
›› In a younger child, a face mask may be required (Figure 87). This should be placed over the child’s mouth and nose. Expel one puff into the chamber and count five breaths before releasing a second puff.
Figure 87. Using a facemask to administer inhaler.
STEP-BY-STEP GUIDE
Complications Steroid inhalers: ӹӹ Oral candidiasis. Advise the child to wash their mouth with water after use. ӹӹ Poor growth with prolonged use. However this risk is very small compared to oral doses.
Beta agonists: ӹӹ Tachycardia. ӹӹ Tremors. ӹӹ Headaches.
TOP TIPS
• Try to make the process fun, if at all possible. Use active distractions. The child may be comfortable watching a TV or smart phone videos. Make the spacer into a toy, offer stickers to make it more accessible, and let children play with it first to familiarise themselves with it. • Do not get disconcerted if the child screams violently, as very few small children tolerate inhalers/spacers well. It can help to reassure the parents that this reaction is normal, and that when children cry they take deeper breaths and may actually open up their lungs to the medication as a by-product! • If children are very difficult to engage, holding them in the same manner as one would for an ENT examination can be helpful: ask the parent to give them a big hug with one hand across both their arms and another on their forehead. • Remember that when spacers are cleaned they should be left to drip-dry to avoid the build-up of static which can prevent medication reaching the patient.
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Asthma
Peak Flow Measurement
Indication
1 2
3
4
ӹӹ Acute asthma exacerbation. ӹӹ Asthma control monitoring in clinic.
Assemble the peak flow meter, and mouth piece (Figure 88). Explain the task. Often the technique will be likened to “blowing out the candles on your birthday cake” and it may help to pretend to be ”blown away” by how hard the child can blow. Make it into a competition to see if they can beat their score with each attempt, and at every stage be encouraging and fun. Ask them to stand up tall and blow as hard as they can into the mouthpiece, keeping their fingers clear of the measuring marks and movable parts (Figure 89). Congratulate them on their performance but tell them you think they can do better. Try the same process three times and take the best score. Plot the score on the chart of expected peak flow values for height (Table 1) or against the child’s personalised previous scores.
Practical Skills
Peak flow can be a useful though crude measurement of respiratory function. It can be particularly useful in obstructive airway disease where the rate of exhalation is impaired by narrowed airways. Peak flow is about the maximum rate of flow generated, not the capacity of the lungs. The lower the peak flow reading, the greater degree of obstruction predominantly in the large airways (e.g. bronchoconstriction), which prevents effective exhalation. The test is very effort-dependent as forcing the maximal exhalation requires the use of numerous accessory skeletal muscles. Children have to be old enough to understand how to use the peak flow meter (at least 5-years-old).
STEP-BY-STEP GUIDE
Figure 89. Blowing long and hard into the mouth piece.
TABLE 1: Interpreting peak flow values by height (adapted from www.peakflow.com, for use with EU/ EN13826 peak flow meters). Height (m)
Predicted PEFR (L/min)
0.85
87
0.90
95
0.95
104
1.00
115
1.05
127
1.10
141
1.15
157
1.20
174
1.25
192
1.30
212
PP Height of the patient.
1.35
233
PP Expected peak flow chart/peak flow calculator.
1.40
254
1.45
276
1.50
299
1.55
323
1.60
346
1.65
370
1.70
393
EQUIPMENT CHECKLIST
5
PP Peak flow meter. PP Mouth piece.
PP Patient peak flow diary (if available).
TOP TIPS
• The score is all about the velocity of expiration rather than the capacity, so emphasise that the child needs to blow out as hard and fast as possible. • Be familiar with the British Thoracic Society asthma management guidelines which use peak flow percentages as one factor in delineating the severity of an asthma exacerbation.
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Figure 88. Assembling the peak flow meter and mouthpiece.
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ADDITIONAL PROCEDURES Lumbar Puncture Lumbar punctures (LP) are most commonly done in patients with suspected meningitis. The technique in children is similar to that in adults, and is actually often easier since the intervertebral spaces are relatively larger. The technique in a neonate is described below. Older children are positioned in
a similar manner to adult patients, and cold spray or lignocaine infiltration can be utilised as analgesia if desired.
Indication ӹӹ A clinical suspicion of meningitis/ encephalitis in the absence of contraindications. ӹӹ Tertiary neurological or metabolic investigations.
1
2 3 4 PP Sterile drapes. PP Sterile gauze. PP Sterile gloves. PP Non-sterile apron. PP Skin cleansing solution, e.g. chloroprep. PP Lumbar puncture needle.
5
PP Glucose bottle (the same blood bottle used to test blood glucose in the lab). PP Plaster.
• Suspicion of raised intracranial pressure and/or space occupying lesions (e.g. reduced consciousness level, focal seizures/neurology, papilloedema, Cushing’s triad). • Deranged clotting factors. • A clinically decompensating or unstable patient.
STEP-BY-STEP GUIDE
EQUIPMENT CHECKLIST
PP Minimum of three universal collection pots (sterile white containers).
CONTRAINDICATIONS
6
Ensure an experienced colleague is present to hold the child effectively.
7
Check the patient’s blood glucose level. This is required to accurately interpret the CSF glucose. Wash hands, put on sterile gloves and apron. Ensure you have consent for the procedure. Position the patient effectively by ensuring: i The head is to the left side (if the doctor is right handed). ii The child is as close to the edge of the bed as possible. iii The child’s back is parallel with the edge of the bed and at a right angle to the mattress (i.e. not tilted towards or away from the doctor). Clean the area around L3-L4 in a circumferential manner. Also clean the area around the uppermost iliac crest. Identify the iliac crest. Draw an imaginary line between the iliac crests: this will act as the anatomical marker. Identify a vertebral space one above or one below this imaginary line: this will be the site for the lumbar puncture (Figure 90). Insert the spinal needle in the centre of the disc space, angled slightly proximally to slip between the vertebral bodies. Insert this parallel with the flat surface of the bed. It may be helpful to keep a finger on the iliac crest while doing this, for stability and as an anatomical landmark. It can
8
be helpful to aim towards the umbilicus (Figure 91). A slight “give” may be felt on progression through the spinal ligaments. At this stage, remove
Figure 90. Clean the back, and identify a space above or below the line of the iliac crest.
L2 L3
Skin Dura mater and arachnoid Vertebral disc
L4 L5 S1
Lumbar puncture needle Supraspinous ligament Cauda equina
Figure 91. Inserting the needle whilst curving the spine (top) with anatomical landmarks shown (bottom).
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Additional Procedures
STEP-BY-STEP GUIDE
puncture site as required (Figure 96). Note that use of a transparent quick drying film spray (e.g. Opsite) is an alternative. 11 Thank the patient and/or parents. 12 Carefully label the samples, send them to the lab and chase the results.
Figure 93. Drip CSF into bottles held by assistant.
• Infection. • Swelling/bruising. • Headaches. • Unsuccessful procedure (dry tap/ bloody tap).
TOP TIPS
• The LP can look like a traumatic procedure and observers can put the person performing it under undue pressure. Advising the parents to wait outside is beneficial for all parties. Figure 94. Reinsert the stylet.
Figure 92. Remove the stylet while holding the lumbar puncture needle firmly.
• Bleeding.
Practical Skills
the stylet of the needle and look for CSF in the same way you might look for flashback from a cannula (Figure 92). If no CSF is present, replace the stylet and reposition the needle. Continue to check for flashback each time. 9 When CSF is found, catch approximately eight drops in each of the three universal containers and approximately six drops in the glucose container (Figure 93). 10 Replace the stylet before removing the entire needle (Figure 94) and compressing the site with gauze (Figure 95). Place a plaster over the
COMPLICATIONS
• Correct positioning is key. Do not insert the needle until happy that the child is lying parallel with the bed and at a right angle to the mattress. Do not be afraid to ask the person holding the child to reposition as required. • Adjust the bed to the most convenient height for the person doing the LP.
Figure 95. Remove the needle and quickly compress with gauze.
Figure 96. Place a plaster over the entry site.
• The patient may flinch as the needle touches the skin. Be confident with this first movement, as being tentative may cause the patient more discomfort and make the procedure more difficult. • When counselling the parents in advance, it is helpful to advise them that the procedure may not be successful. It is not uncommon to obtain a bloody tap or a dry tap, despite appropriate technique. • If the CSF is flowing very slowly, rotating the needle gently so the bevel faces the child’s head can sometimes yield a more effective flow. • If a bloody tap is obtained, it can be helpful to wait a few seconds to see if the blood clears slightly. Some taps start off looking like whole blood but clear slightly to leave blood stained CSF.
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Practical Skills
Non-Invasive Blood Pressure Measurement Blood pressure measurement can be challenging in a child who is upset, particularly since this may artificially increase the blood pressure. Distracting and calming the child is key.
STEP-BY-STEP GUIDE
1
2
Indication Blood pressure should be checked as part of the routine observations in any patient seeking hospital care. It is particularly important in: ӹӹ Sepsis. ӹӹ Shock. ӹӹ Suspicion of raised intracranial pressure. ӹӹ Suspicion of renal impairment. ӹӹ Suspicion of congenital cardiac disease (four limb blood pressures).
3
Place the cuff around the bicep muscle if measuring on the upper limb (preferred) or around the calf on the lower limb (Figure 97). Place the stethoscope over a distal pulse. If in the arm, this will be the brachial pulse, and in the leg, the posterior tibial or dorsalis pedis pulse. Ensure that the valve on the sphyg is closed. Inflate the cuff until the pulse is no longer heard (Figure 98). Inflate a further 20 mmHg beyond this.
4
5
6
7
EQUIPMENT CHECKLIST
PP Sphygmomanometer ”sphyg”. PP Appropriate size of blood pressure cuff (inner surface of cuff should encompass 90-100% of the arm).
Figure 97. Place BP cuff in appropriate position.
PP Stethoscope. TOP TIPS
• Attempt to settle the patient as much as possible. If attempting the procedure on a small baby, it may help if they are fed either during or just before the procedure and kept warm throughout. • Ensure the right size cuff is used, as a slightly missized cuff can yield significantly erroneous results.
8
Figure 98. Inflate cuff until pulse is no longer heard through the stethoscope.
9
Gently open the valve of the sphyg and allow the cuff to deflate in a consistent and controlled manner. Continue to auscultate the distal pulse. Mark the point at which turbulent blood flow (Korotkoff sounds) can be heard. This represents the return of the arterial flow and is the systolic blood pressure. Continue deflating the cuff slowly. The sounds may become slightly more blowing in nature before returning to a harsh tapping. The sounds will become abruptly muffled and subsequently disappear altogether. Classically (especially in adults), this disappearance is recorded as the diastolic pressure. However in children under 13-years-old, these sounds may not disappear altogether until the cuff is completely removed. In this case, the diastolic pressure should be recorded at the point the sounds became significantly muffled. Estimate the mean blood pressure from these values: Mean Blood Pressure (MAP) = Diastolic Blood Pressure + –13 (Systolic Blood PressureDiastolic Blood Pressure) Record the reading in the medical notes/observation chart.
EQUIPMENT CHECKLIST
Nasogastric Tube Insertion Nasogastric tubes are commonly used, particularly in neonates, where the more premature babies invariably require a period of NG feeding. Insertion is more challenging in children due to compliance difficulties. Placement into the lungs is a possibility, but feeding through this should be a never event.
Indication ӹӹ Gastric drainage/relief of bowel obstruction. ӹӹ Feeding. ӹӹ Prevention of vomiting.
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PP Non-sterile gloves.
PP Sticky tape.
PP Disposable apron.
PP Drainage system.
PP Lubricant gel.
PP Cup of water (in older children).
PP NG tube. PP 60 mL syringe and pH strip.
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Additional Procedures
STEP-BY-STEP GUIDE
3
4 5
6
Ideally have the child sitting up. Wash hands, and put on gloves and an apron. Estimate the length of the tube by measuring from the tip of the child’s nose to the xiphisternum, passing the tragus of the ear (Figure 99). Lubricate the end of the NG tube (Figure 100). Slide the tube along the floor of the nasal cavity, initially aiming towards the occiput. There is usually slight resistance as the tube passes into the oesophagus (Figure 101). In an older child, ask them to repeatedly swallow or offer them
sips of water. This aids advancement of the NG tube. 7 Advance the tube to the predetermined distance. 8 Attempt to aspirate gastric fluid into the syringe (Figure 102) and test it on a pH strip (Figure 103). 9 Securely tape the NG tube to the nose (Figure 104). 10 Attach the tube to a drainage system or spigot, as directed.
The most definitive way to check is with an X-ray (e.g. if stomach fluid cannot be aspirated or has a pH>5.5): a correctly positioned NG tube should be seen passing below the diaphragm (Figure 105). Feeding through an incorrectly placed NG tube is entirely avoidable through appropriate checks as above.
NG tip-too short
ET Tube
Checking the position of the NG tube can be done by several methods. First, the pH of the NG aspirate can be checked. If the pH is 92%.
Route. Nasal Cannula.
06 10
Start. 1/1/15. Signature
A.Jones
Indication. Pneumonia.
Stop/review. 2/1/15.
14
JONES 2134
18 22
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PRESCRIBING
3 4 5
Route of administration. Rate of infusion and likely the acceptable range of a variable infusion (e.g. 5-20 micrograms/kg/min). Patient’s weight and age.
From this, the infusion can be calculated as per Example 3. EXAMPLE 3 Prescription of a salbutamol loading dose and maintenance infusion in a severely asthmatic 5-year-old boy. Weight =20 kg.
Date
Drug
Dose
Route
1/1/15
SALBUTAMOL (200 micrograms/mL)
250 micrograms (1.25mL)
IV
Signature
A. Jones
Given
Batch
JONES 1234
Date
Fluid
Additives
Volume
Route
Rate
1/1/15
0.9% SODIUM CHLORIDE
SALBUTAMOL 10mg
50mL
IV
6mL-30mL/hr (60-300 micrograms/kg/ hr)
Signature
A. Jones
Given
Batch
JONES 1234
Loading dose:
15 micrograms/kg (maximum dose 250 micrograms) = 15 micrograms/kg x 20 kg = 250 micrograms (300 micrograms, rounded down to the maximum dose). Using a standard solution of 200 micrograms/mL, this is equivalent to 250 micrograms ÷ 200 micrograms/mL = 1.25 mL. It is subsequently prescribed on the once-only section of the prescription chart.
Maintenance dose:
Lower limit= 60 micrograms/kg/hr (1 microgram per kg per minute) Upper limit= 300 micrograms/kg/hr (5 micrograms per kg per minute) For this case, 10mg of salbutamol is diluted in 50mL of 0.9% sodium chloride, at a concentration of 200 micrograms/mL to generate a standard solution. This solution is then used to deliver the required dose. Infusions are prescribed at a rate of millilitres per hour (or minute); therefore, the maintenance dose has to be converted to millilitres. In this case: Lower limit = 60 micrograms/kg/hr ÷ 200 micrograms/mL = 0.3mL/kg/hr, i.e. 0.3mL of salbutamol contains 60 micrograms of salbutamol. Upper limit = 300 micrograms/kg/hr ÷ 200 micrograms/mL = 1.5mL/kg/hr These figures are then multiplied by the weight to get the tailored dose for the child: Lower limit = 0.3 mL/kg/hr x 20 kg = 6mL/hr Upper limit = 1.5 mL/kg/hr x 20 kg = 30mL/hr It is subsequently prescribed on a fluid chart.
FLUID PRESCRIBING Fluids are prescribed very differently in paediatrics than in adults. However, the formula is very simple once you know it. Fluids are almost always prescribed in 500mL bags. Prescription has three essential components: 1 The fluid constituents and bag size e.g. NaCl 0.9% + dextrose 5% + KCl 10mmol (500mL). 2 The rate at which to administer it, in mL/hr to achieve the desired 24-hourly fluid requirement. 3 The signature of the prescriber.
There should also be a record of: 1 Body weight, including weight change over previous 24 hours. 2 Fluid input (oral, nasogastric, intravenous) and output (urine, vomiting, drains, stomas, diarrhoea, insensible losses-particularly with fever/dehydration/ hyperventilation) over previous 24 hours. 3 Assessment of fluid status (dehydrated, oedematous, euvolaemic). 4 Relevant blood results [full blood count (FBC), urea & electrolytes (U&Es), chloride, glucose, and urinary electrolytes, if measured].
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FLUID PRESCRIBING
Neonatal Fluid Management Neonatal fluid requirements can be defined as enough fluid to keep the baby euvolaemic (i.e. replacing measured and insensible losses). As a rule of thumb, use the fluid requirements in Table 1. These figures come from research with babies born at term, but are often used for babies of all gestations. These volumes also apply when giving nasogastric feeds. Factor in the clinical state of the baby, which includes urine output, weight gain and plasma sodium level. The fluid usually prescribed to neonates is 10% dextrose, which addresses glucose requirements as well as fluid requirements. After the first one to two days, it is important to provide electrolyte support as well. This involves the addition of
TABLE 1: Fluid requirements in a neonate Day of life
Maintenance fluid requirements
1.
50-60mL/kg/day.
2.
70-80mL/kg/day.
3.
80-100mL/kg/day.
4.
100-120mL/kg/day.
5.
120-150mL/kg/day.
sodium (3mmol/kg/day) and potassium (2mmol/kg/day). Some neonatal units will also add calcium (1mmol/kg/day). Calculation of electrolyte doses can be tricky at first, as the correct amount has to be added to the 500mL 10% dextrose bag in order to get the daily requirement. The key is to first work out the daily requirement of electrolytes (total mmols/ day), and the daily requirement of fluids (mL/day). Then use the ratio between the total daily requirement of fluids and 500mL to work out how much goes into each bag. If the daily requirement of fluid is 250mL, ensure that the 500mL bag has the requirement for two days of electrolytes in it (Example 4). Table 2 summarises the number of mmol of electrolytes to be
PRESCRIBING
Note that fluid and electrolyte calculations are almost always based on weight. However, if accurate calculation of insensible losses is critical (e.g. with acute or chronic kidney disease, with cancer, or with weight above the 91st centile), consider using body surface area for the calculations instead (insensible losses (300–400 mL/m2 /24 hours) plus urine output). Surface area is calculated based on height and weight, and can be looked up on a reference chart.
EXAMPLE 4 Fluid requirement calculation: A 4-day-old baby, weighing 3.5kg, with normal electrolytes and requiring maintenance fluids.
Date
Fluid
Additives
Volume
Route
Rate
Signature
1/1/15
10% DEXTROSE (120mL/kg/ day)
12.5mmol NaCl (3mmol/kg/day)
500mL
IV
17.5mL/hr
A. Jones
Given
Batch
JONES 1234.
8.3mmol
KCl (2mmol/kg/day) Fluid
= 120mL/kg/day = 120 x 3.5 = 420mL/day = 17.5mL/hr
Potassium
= 2mmol/kg/day = 2 x 3.5
Sodium
= 3mmol/kg/day = 3 x 3.5
= 10.5mmol/day = 7mmol/day
The correct amount of potassium and sodium needs to be added to the 500mL dextrose bag. If 420mL of fluid is given in 24 hours, then every 420 mL of fluid needs to contain 10.5mmol sodium and 7mmol potassium. Therefore: Sodium per 500mL bag= 10.5 x 500/420 = 12.5mmol Potassium per 500mL bag= 7 x 500/420 = 8.3mmol
TABLE 2: Number of mmol of electrolytes to add to a 500mL bag of dextrose for different daily fluid and electrolyte requirements Fluid requirement
Electrolyte requirement.
40 mL/kg/day
60 mL/kg/day
90 mL/kg/day
120 mL/kg/day
150 mL/kg/day
1 mmol/kg/day.
12.5 mmol
8.3 mmol
5.6 mmol
4.2 mmol
3.3 mmol
2 mmol/kg/day.
25.0 mmol
16.7 mmol
11.1 mmol
8.3 mmol
6.7 mmol
3 mmol/kg/day.
37.5 mmol
25.0 mmol
16.7 mmol
12.5 mmol
10.0 mmol
4 mmol/kg/day.
50.0 mmol
33.3 mmol
22.2 mmol
16.7 mmol
13.3 mmol
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PRESCRIBING
added to a 500mL bag of 10% dextrose for different maintenance fluids infusion rates. Note that this is the same, regardless of the weight of the baby, as it is based on the ratio of fluid to electrolyte requirements, which is independent of weight. This is a useful reference on any neonatal job. The above guidance is the generally employed practice across most neonatal units for most babies; however, local guidelines may be slightly different and should always be checked. Always consider every baby’s individual fluid considerations before prescribing fluids and check with a senior colleague if in doubt. Be aware also that some babies, particularly on surgical units, may be given total parenteral nutrition (TPN) for some time, instead of routine fluids. This is a specialist fluid regime and each patient will generally have an individualised regime calculated carefully between senior doctors and the pharmacy team, and ordered from a central supply accordingly.
Paediatric Fluid Management After the neonatal period, children normally receive a mixture of sodium and dextrose – the typical combination is a premade bag of “0.9% Sodium Chloride + 5% Dextrose”. Hypotonic fluids are avoided, as they have the potential to cause sudden fluid shifts into cells from the plasma, as well as risking hyponatremia. Glucose is required as children are more vulnerable than adults to hypoglycaemia. Potassium is usually added, adjusted according to serum levels – normally as a starting point 10 mmol potassium chloride is added to each 500 mL bag. Table 3 shows how maintenance fluids over 24 hours are calculated in this age group. This means that for the first 10 kg of ANY child’s weight, they will require 100 mL/kg. For the second 10 kg, they require 50 mL/kg and they will need 20 mL/ kg for every kg after this. Examples 5 and 6 demonstrate how this is done in practice. A shortcut is knowing that children require 1500 mL for the first 20 kg of weight inclusive plus “20 x the remaining weight”.
TABLE 3: Fluid requirements in children. Be aware that over a 24 hour period, females rarely need more than two litres, and males rarely need more than 2.5 litres, so adjust fluid prescriptions accordingly. Weight
Fluid requirement
0-10 kg
100 mL/kg
10-20 kg
50 mL/kg
Above 20 kg
20 mL/kg
Note that in cases of respiratory distress (e.g. lower respiratory tract infection), often only two-thirds of the normal fluid intake are given due to a theoretical possibility of the syndrome of inappropriate antidiuretic hormone secretion (SiADH) and fluid overload.
Fluid Boluses For children, the typical fluid bolus is 20 mL/kg of 0.9% sodium chloride. Typically, boluses are given over less than ten minutes, although recent World Health Organisation (WHO) guidelines have suggested that it may be advisable to give boluses over a longer duration, due to the potential risk of fluid overload, particularly in developing world settings. Smaller boluses of 10 mL/kg are used in special groups e.g. neonates, those with cardiac disease, diabetic ketoacidosis and trauma. Be extremely cautious with fluid boluses in situations where rapid shifts of fluid can have disastrous consequences (e.g. cerebral oedema in diabetic ketoacidosis or pulmonary oedema in heart failure/ myocarditis). Always reassess the child’s fluid requirements after administration of a bolus. Generally, if a child has required three boluses (i.e. 60 mL/kg), call for anaesthetic support in case the child deteriorates into pulmonary oedema. The patient may still require more fluid if significantly shocked, but may also require invasive respiratory support. Dextrose boluses in the hypoglycaemic child are usually given at 2 mL/kg of 10% dextrose. The glucose level should then be rechecked and the dose can be repeated, or a dextrose
EXAMPLE 5 Fluid requirement calculation: maintenance fluid in a 13kg child.
Date
Fluid
Additive
Volume
Route
Rate
1/1/15
0.9% SODIUM CHLORIDE + 5% DEXTROSE + 10mmol KCl
-
500mL
IV
48mL/hr
= 50 mL/kg/day
Given
Batch
A. Jones JONES 1234
First 10 kg = 100 mL/kg/day = 100 x 10 Next 3 kg
Signature
= 50 x 3
= 1000 mL
= 150 mL
Total = 1150 mL in 24 hours = 48 mL per hour
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FLUID PRESCRIBING
EXAMPLE 6 Fluid requirement calculation: maintenance fluid in a 26kg child.
Fluid
Additive
Volume
Route
Rate
Signature
1/1/15
0.9% SODIUM CHLORIDE + 5% DEXTROSE + 10mmol KCl
-
500mL
IV
67.5mL/hr
A. Jones
First 10 kg = 100 mL/kg/day = 100 x 10 Second 10 kg = 50 mL/kg/day = 50 x 10 Next 6kg = 20 mL/kg/day = 20 x 6 Total
Given
Batch
JONES 1234 = 1000 mL. = 500mL. =120 mL. = 1620 mL in 24 hours. = 67.5 mL per hour.
infusion started. Remember that, in persistently hypoglycaemic children, a full hypoglycaemia screen should be sent whilst they are actually hypoglycaemic. This is because hypoglycaemia may be a result of a transient change, e.g. in insulin levels.
Replacement and Redistribution Fluids Often, patients with surgical drains in place will require replacement fluids for ongoing losses. For example, nasogastric (NG) losses in a child with pyloric stenosis may be replaced mL for mL with 0.9% sodium chloride and 10% KCl. Gastric fluid is rich in chloride and potassium; hence, both must be replaced. Some replacement fluids will discount “normal losses”; for example, the decision might be made that stoma losses will be replaced only above 20mL/kg/day in a neonate. Additional fluid may also need to be prescribed on top of maintenance for redistribution of fluids, e.g. tissue oedema in sepsis.
Sodium Balance Hyponatremia is common, particularly with hypotonic fluids. If it is asymptomatic, change any hypotonic fluid to isotonic fluid. Restrict IV maintenance fluids to 50-80% of normal if the child is hypervolaemic or at risk of hypervolaemia (e.g. SiADH). Acute symptomatic hyponatraemia (headache, nausea/vomiting, confusion/disorientation, irritability, lethargy, reduced consciousness, convulsions, coma, apnoea) requires immediate expert input, but may be treated with 2mL/kg boluses of 2.7% sodium chloride (given over 10-15 minutes), which can be repeated up to three times if the child is still symptomatic and the sodium is still low. When the child
PRESCRIBING
Date
becomes asymptomatic, it is important to ensure that the sodium does not go up by more than 12mmol/L per day, as over-rapid correction can precipitate cerebral demyelination and brain damage. Hypernatraemia is a less common complication of fluid therapy. Management depends on the fluid status. In hypernatraemic dehydration, the fluid deficit should be replaced (with 0.9% sodium chloride) over 48 hours. If the patient is not dehydrated, then consider going from hypertonic fluid (e.g. 0.9% sodium chloride/5% dextrose) to hypotonic fluid (e.g. 0.45% sodium chloride/5% dextrose). Urinary sodium/osmolality can help assess dehydration if it is unclear: urinary sodium is low and osmolality is high in hypovolaemia. It is important to ensure that the sodium does not go down by more than 12mmol/L per day: over-rapid correction can precipitate cerebral oedema.
Blood Products Blood products are prescribed much less frequently for children than for adults. Cytomegalovirus (CMV) negative blood should be considered in those who may become compromised by CMV infection (which is normally harmless), e.g. neonates or the immunocompromised. Irradiated blood cells (where white blood cells are irradiated) may be required in those with a high risk of transfusion-related graft-versus-host disease (GvHD), e.g. neonates requiring exchange transfusion or the immunocompromised. Blood products are summarised in Table 4, and Example 7 shows an example prescription.
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PRESCRIBING
TABLE 4: Blood products and indications Blood product
Indication
Prescription
Additional considerations
Red blood cells.
• Acute blood loss. • Transfusion dependent children, e.g. those with thalassaemia major. • Post chemotherapy. • Paediatric intensive care patients.
• Unlike with adults, red cells are prescribed in mL, not in units. • Packed red cell volume (mL) = desired rise in haemoglobin (g/dL) x 4 x weight (kg). • The maximum infusion rate is normally 5 mL/kg/hr, up to a maximum of 150 mL/hour.
• There is a potential risk for fluid overload, so furosemide should be considered halfway through the transfusion.
Fresh frozen plasma.
• Patients with coagulopathy (e.g. from chronic liver disease). • Before an invasive procedure. • If there is active bleeding.
• 10-15 mL/kg. • Given at a rate of 10-20 mL/kg/hr.
• Rarely, cryoprecipitate may be needed, for example, if there is active bleeding associated with a low fibrinogen and a raised D-dimer. • This might happen in disseminated intravascular coagulopathy (DIC).
Platelets.
• Low platelets have a variety of causes, including sepsis, iatrogenic (e.g. chemotherapy), and malignancy.
• Platelets are prescribed not in “pools” like in adults, but in mL. • Platelet volume= 15mL/kg over two to three hours.
• In idiopathic thrombocytopenic purpura (ITP), platelets are generally not prescribed, as they are likely to be destroyed by the disease process.
EXAMPLE 7 A 6-year-old sickle cell patient has a haemoglobin of 6 g/dL. Her normal haemoglobin is 8 g/dL. She requires a transfusion. Note that a different formula is used specifically for sickle cell transfusions (as below). Increasing the haematocrit excessively increases the risk of hyperviscosity, fluid overload and vaso-occlusive events. The maximum rate of transfusion in sickle cell is 250mL/hr, and this is not exceeded. However since blood is normally given over a minimum of two hours, the above prescription would be prescribed as 60mL/hr not 100mL/hr.
Date
Fluid
Additive
Volume
Route
Rate
1/1/15
PACKED RED CELLS
-
120ml
IV
60mL/hr
Signature
A. Jones
Given
Batch
JONES 1234 Packed red cell volume (mL) = = = = Maximum Rate (mL/h) = = =
Desired rise in haemoglobin (g/dL) x 3 x weight (kg) (8-6) x 3 x 20 2 x 3 x 20 120mL Weight (kg) x 5 20 x 5 100 mL/hr
Table 5 gives a summary of common prescribing errors. This chapter contains many ‘dos and don’ts’, but the take home message is that prescribing is one of the most important
jobs performed by a clinician. A thoughtful and conscientious approach coupled with a willingness to ask for help will ensure safe, considerate and effective prescribing.
TABLE 5: Classic prescribing errors Drug/situation
Type of Error
Wrong weight.
• Do not always assume the documented weight is accurate. • If a patient is unable to be weighed on admission, a weight may be estimated. • Have a marker or “normal” weight of children of different ages and question if it seems abnormally high or low.
Calculation errors.
• Check and double check calculations, particularly if complex. • Factor of 10 errors are common but can have catastrophic effects if administered. continued
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REFERENCES AND FURTHER READING
TABLE 5: Continued Type of Error
Misinterpreting dosing information.
• Be careful to read the whole monograph. For example, is the dose 100 mg/kg three to four times a day or 100 mg/kg/ day in three to four divided doses? • Another common mistake is that formularies may break down different doses for different indications, e.g. amoxicillin IV dosing for listerial meningitis is listed after the IV section for ”susceptible infections.” The latter is subtherapeutic for listeria but is commonly prescribed as it is the first dose listed.
Prescribing drugs in “mL”.
• Wherever possible, medicines should be prescribed in mg or the most appropriate dose units. • Liquid medicines may come in a range of licensed and unlicensed forms and strengths. A common example is furosemide liquid which is available in 5 mg/mL, 20 mg/mL, 40 mg/mL and 50 mg/mL formulations. Writing 5 mL may mean 5-50 mg, depending on the strength of liquid available. • Some medication may be prescribed in mL, e.g. lactulose, co-amoxiclav. However the compound strength must be specified.
Penicillins.
• When prescribing “Tazocin” or “Augmentin” to penicillin-allergic patients. • Tazocin = Piperacillin (a penicillin) + Tazobactam. • Augmentin = Amoxicillin + Clavulanic acid.
Methotrexate.
• This is a very toxic drug used in inflammatory or neoplastic conditions. • It is a once WEEKLY drug. The classic mistake is to prescribe it daily.
Ibuprofen.
• Avoid prescribing this in asthma due to risk of bronchospasm.
Primary and secondary care interface.
• Information flow between primary and secondary care is a common source of omissions and errors. Ensure the drug history on admission is as complete as possible, and that any additional necessary information is acquired as soon as possible. • Ask about inhalers, topical preparations, vitamin supplements and vaccinations. • For discharge prescriptions, complete all medications, including previous regular medication. Specify liquid strengths if possible. Ensure that the discharge letter includes information about any medication stopped or changed during the admission.
G6PD.
• Numerous drugs may precipitate glucose-6-phosphate dehydrogenase deficiency (G6PD) crises. • Classic examples are ciprofloxacin, nitrofurantoin and the sulphonamides.
Drug-drug interactions.
• Remember the inducers and inhibitors of CYP450 enzymes. A common scenario is the prescription of clarithromycin, an enzyme inhibitor, which will increase the plasma concentration of anti-epileptics like carbamazepine and valproate.
Copying a previous incorrect prescription.
• Always recalculate prescriptions when re-prescribing. There may be a change in weight, or there may have been an error in the previous prescription.
PRESCRIBING
Drug/situation
REFERENCES AND FURTHER READING 1 Paediatric Formulary Committee. BNF for Children 2015. London: BMJ Group, Pharmaceutical Press and RCPCH Publications; 2015. 2 NICE guideline NG29: Intravenous fluid prescription in children and young people in hospital. 2015. https://www.nice.org.uk /guidance/ng29. 3 Maitland K et al. Mortality after fluid bolus in African children with severe infection. New EnglJ Med.2011; 364:2483-95 4 WHO. Paediatric emergency triage, assessment and treatment. 2016. http://apps.who.int/iris/bitstr eam/10665/204463/1/9789241510219_eng.pdf. 5 TOXBASE. www.toxbase.org. 6 The Cochrane Collaboration. www.cochrane.org. 7 Food and Drug Administration (FDA). www.fda.gov. 8 NHS Evidence. www.evidence.nhs.uk. 9 Joint United Kingdom Blood Transfusion and Tissue Transplantation Services Professional Advisory Committee (JPAC). 2015. Effective Transfusions in Paediatric Practice. http://www.transfusionguidelines.org.uk/ transfusion-handbook/10-effective-transfusion-in-paediatric-practice/10-3-transfusion-of-infants-and-children.
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4.01
UNDERGRADUATE AND POSTGRADUATE ASSESSMENTS IN PAEDIATRICS MICHAEL MALLEY AND MARIE MONAGHAN
EXPECTATIONS OF A MEDICAL STUDENT
CONTENTS 658 Expectations of a Medical Student 659 Work Based Assessments
659 Selecting an Assessor 659 Case-Based Discussions 659 Mini-Clinical Evaluation Exercises 659 Directly Observed Procedural Skills
659 OSCEs
659 History Taking 660 Examination 662 Communication 664 Practical Skills
Although most medical students will not become paediatricians, doctors encounter children in most specialties, such as primary care, ENT and other surgical specialties, and emergency medicine, amongst others. For undergraduates, senior clinicians want the student to approach the rotation in “good faith.” This means feeling that the student has tried their best, made the most of their time on the ward and shown a measurable improvement in performance. This is not demonstrated in a one-off assessment, but continually throughout the attachment. In terms of competence, it is important the student is confident that they: ӹӹ Are aware of the basic management and recognition of common paediatric problems. ӹӹ Appreciate the differences between the management of children compared to adults, including communication challenges. ӹӹ Understand the differences in adult and paediatric basic life support. ӹӹ Are aware of child protection issues and start to understand their management. ӹӹ Have completed the requisite range of assessments (which may require organisation on the part of the student). Before a supervisor meeting at the end of the placement, it is important to think about the main learning points from the rotation, as well as what has gone well, and not so well. Demonstrate evidence of engagement with the children, and the paediatric team. Be aware that supervisors will usually take advice from other paediatric staff to make a holistic assessment of each student. For postgraduates, educational supervisors have a long list of criteria to “tick off ”. Become familiar with this list. Other factors to consider include evidence of quality improvement projects (e.g. audits), managerial experience (e.g. rota co-ordination), teaching experience, exam success and reflective practice. This should all be apparent in the trainee’s learning portfolio. More generally, remember assessments are increasingly recognised as learning opportunities. Prepare
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OBJECTIVE STRUCTURED CLINICAL EXAMINATIONS (OSCEs)
OBJECTIVE STRUCTURED CLINICAL EXAMINATIONS (OSCEs)
WORK BASED ASSESSMENTS
History Taking
Work-based assessments are changing at the time of writing, with new assessments added frequently. The basis of undergraduate and postgraduate assessment, however, remains CBDs (case-based discussions), CEXs (clinical evaluation exercises) and DOPS (directly observed procedural skills). The key is to empower the trainee to complete assessments that aid development and reflection. To ensure the best feedback, aim to have any assessment form filled out contemporaneously. This sounds simple but a surprising number of assessments occur opportunistically, but never get formally documented.
General Approach
Selecting an Assessor In practice, the quality of assessments varies greatly. Some supervisors will expect too little and others may expect too much. Do not choose someone because they are an “easy target”. Instead, choose a familiar doctor, who is aware of the assessment process, will be fair, and will give detailed, honest feedback. It might be tempting to opt for a better grade over an honest appraisal, but in reality, constructive feedback is more beneficial in the long-term.
Case-Based Discussions Case based discussions (CBDs) are an opportunity to discuss a case at length, from initial presentation to examination to management. CBDs encourage discussion of the approach to each part of the patient journey, with the aim of improving the student’s knowledge and thought process along the way. When done properly, they can be time-consuming, so arrange to meet with a willing consultant or supervisor at a mutually convenient time. Ensure patient notes and investigation results are accessible. Present a pre-prepared overview of the case.
Mini Clinical Evaluation Exercises Mini clinical evaluation exercises (Mini-CEXs) are designed to give juniors the opportunity to be observed in a clinical setting, e.g. communication, history taking or examination. Be opportunistic and take advantage of situations where natural observation occurs, e.g. on a ward round/in clinic. If appropriate, ask for an opportunity to repeat the assessment later to demonstrate improvements in practice.
Directly Observed Procedural Skills Directly observed procedural skills (DOPS) are more common at the postgraduate level. They involve observation performing a clinical procedure, from blood taking to more advanced procedures, like chest drain insertion. Again, prepare in advance and make sure you get dedicated time to debrief after the procedure is complete.
History taking stations are common. Check what is expected in advance. These stations should be straightforward. There is a clear rubric to follow, and plenty of opportunities to practice in advance. There are three “S’s” in SucceSS: ӹӹ Structure. Replicate the same format for every history station. ӹӹ Smoothness. Practise fluently moving from one section of the history to the next. ӹӹ Sensitivity. Remember that the parent is potentially anxious/ scared. Elicit their main concerns and give them a chance to speak – don’t just ask closed questions. Before entering the station, decide whether to take a focused history of a particular condition or a more general history. Use the information in the briefing to inform an initial differential diagnosis. Write down any key questions that spring to mind. Elicit all the main concerns of the patient/carer (this may be an actor for the OSCE). One way to ensure nothing is missed is to ask, Is there anything I’ve missed or anything else you are concerned about? Do you have any questions for me now? To end, summarise the key points and state what will happen next. So to summarise, Jamie has had three previous admissions with asthma and has come in today with wheeze and difficulty breathing over the last 12 hours. You are worried he might have pneumonia. I’ll examine Jamie and then we can discuss what is going on in more detail.
ASSESSMENTS IN PAEDIATRICS
for them thoroughly and ask for extensive and personalised feedback on how to improve afterwards.
Presenting Findings The presentation is key and this short soliloquy can dramatically alter the final grade. One suggestion is to stand with hands behind the back. Speak slowly and deliberately. Look at the examiner and speak with confidence. Open with a simple introduction. For example: I met Mrs. Brown today, who presented with her son Alex. Alex presents with a history of … followed by the headline symptoms. Then state the important positive and negative findings of the history. It is not a recollection of every word, but of information gathered. Ensure that the summary is no longer than one minute. After relaying the history, suggest the next steps. This will usually involve a full examination of the relevant systems and an investigation strategy. Think of any other information which may be helpful, for example the red book/growth chart, previous discharge summaries or medication lists, and request them as well. If there is still additional time, finish with a one sentence summary. An example presentation is shown in Box 1, alongside a mark scheme in Table 1.
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Examination
Box 1: Presenting a history I met Mrs. Brown today, who brought her 7-year-old son Alex to ED with a 24 hour history of difficulty breathing and wheeze. Alex is known to have asthma. He has had a cough for two days and has developed progressive difficulty in breathing and wheeze over the last 24 hours. He has a low grade temperature and a dry cough. He has been using two puffs of his salbutamol inhaler every four hours without improvement. Alex has had many previous admissions to hospital, the most serious of which required IV salbutamol two years ago. His last admission was two months ago. His known asthma triggers are pets and upper respiratory tract infections, and he also has a history of eczema. His normal peak flow reading is around 300 mL/min. He misses about two days of school per month due to his asthma. Alex was born at term without complication and there have been no concerns about his development. Alex takes two puffs of a beclomethasone inhaler twice per day and uses his salbutamol inhaler when required. He takes no other medications. He has no known drug allergies and his immunisations are up-to-date. Alex’s mother has a history of asthma and his father has hay fever. He lives in a flat with his parents and his 5-year-old brother. There are no smokers in the house and the family have never had any involvement with social services. In summary, Alex is a 7-year-old boy who presents with a likely acute exacerbation of his asthma. I would like to proceed to examine Alex, view his observations chart, perform a peak flow measurement and initiate bronchodilator therapy.
TABLE 1: History taking mark scheme Domain Conduct of the consultation. To include: • Introduction, clarifies role. • Rapport. • Empathy and respect. History taking. To include: • Clear history of presenting complaint. • Appropriate style. • Fluent and comprehensive history. • Explores and responds to concerns/feelings. • Summarises and checks understanding. Differential diagnosis and initial management. To include: • Appropriate range of differentials appropriate for age. • Accuracy of information/ clinical knowledge. FINAL GRADE.
Mark
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
Examination stations cause anxiety amongst undergraduate and postgraduate candidates alike. What if the child isn’t co-operative or cries? What if signs are missed? Candidates often think they will be heinously penalised for forgetting pallor or lymphadenopathy or missing grade one clubbing. They aren’t! Remember that the overall conduct of the examination counts more than the sum of its individual minutiae. Be professional, sensitive, structured and observant. The examiner will be asking the question: Is this person appropriate for the next level? Students should act like a junior doctor; junior doctors should act like a middlegrade doctor.
Before the Examination Practise. A lot. Become familiar with the important components of each examination, and practice under supervision with real patients. This will ensure technique is committed to muscle memory, making it easier to concentrate on picking up relevant clinical signs. The first and last minute of each station are crucial. Therefore, practise the introduction and take time to put the patient at ease. Then practise presenting the examination findings in one minute. Practise presenting both real and imaginary patients. Practise to the mirror, to friends, and to colleagues. Patients suitable for examinations nearly always have chronic conditions and are clinically stable. Contemplate what signs they may have and make a list of the common underlying diagnoses (Table 2). Ask senior students what they encountered in their exams. It may be helpful to create maps of common differential diagnoses to consolidate key findings. Figure 1 is an example for abdominal examination.
Starting the Station Do simple tasks well. Remember: the first minute is critical. Hands should
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OBJECTIVE STRUCTURED CLINICAL EXAMINATIONS (OSCEs)
Observing the Patient
Examining the Patient
Make a show of this – and excuse it by saying: Right before I look at you, I’m just going to have a good look around. Don’t worry—I’m not ignoring you! Look for obvious clues, like wheelchairs/walking aids/medication/modified shoes and scars. Look carefully at the armpits and the back. Learn the meaning of surgical scars carefully; know at least two underlying causes per scar.
Examining children requires a balance between systematic and opportunistic examination. In clinical practice, opportunistic examination is often the way forward, but in timepressured exams, be as structured as possible, working from peripheral signs to central examination. However, when examining an uncooperative child, do the components requiring
TABLE 2: Common examination findings in paediatric exams System
Key Conditions
Key Signs
Key Knowledge
Cardiology.
• Congenital heart disease (especially coarctation, tetralogy, transposition). • Valvular abnormalities. • Septal defects. • Marfan syndrome. • Down syndrome (with ventricular or atrioventricular septal defect; VSD/ AVSD).
• Cyanosis (not common in exams). • Clubbing. • Scars (sternotomy/ thoracotomy/ central lines). • Heart murmurs. • Signs of failure (e.g. hepatomegaly; not common in exams).
• • • •
Respiratory.
• • • •
• Clubbing. • Hyperinflation (chronic obstructive disease, e.g. asthma). • Lung sounds. • Extrapulmonary signs, e.g. clubbing • Finger prick marks for diabetes in CF. • Medication (e.g. inhalers). • Failure to thrive. • Portacaths.
• Causes of clubbing. • Extrapulmonary signs of chronic respiratory diseases. • Management of asthma and cystic fibrosis.
Abdominal.
• Haemoglobinopathies. • Polycystic kidneys. • Ex-premature babies post necrotising enterocolitis (NEC) surgery. • Crohn’s/Ulcerative colitis. • Nephrotic syndrome. • Post kidney transplant. • Antegrade colonic enema (ACE) stoma.
• • • • • •
• Differentials of hepatomegaly, splenomegaly and hepatosplenomegaly. • How to differentiate a spleen and a kidney on examination. • Management of haemoglobinopathies, inflammatory bowel disease, nephrotic syndrome.
Neurological.
• Muscular dystrophies. • Cerebral palsy (hemiplegia, diplegia, quadriplegia, ataxia). • Seventh cranial nerve palsy. • Neurofibromatosis. • Stroke. • Brain tumour.
• • • • • • • • •
Increased/decreased tone. Weakness. Cerebellar signs. Past pointing. Brisk or absent reflexes. Abnormal sensation. Abnormal gait. Gower’s sign. Congenital neurocutaneous markers (e.g. café-au-lait spots, freckling, Shagreen patches). • Wheelchairs/walking aids. • Modified shoes/footwear.
• Differentiate upper vs. lower motor neurone lesions. • Causes of upper vs. lower motor neurone lesions. • Dermatomal and myotomal innervations. • Reflex origins. • Management of cerebral palsy, and role of multidisciplinary team in any neurodisability.
Common multi-system conditions.
• • • • •
• Many signs depending on the condition.
• Learn the main associated signs for each condition so that they can be recognised if they appear in combination.
Cystic fibrosis (CF). Bronchiectasis. Asthma. Chronic lung disease.
Down Syndrome. Turner Syndrome. Neurofibromatosis. Tuberous Sclerosis Complex. Achondroplasia.
Clubbing. Organomegaly. Abdominal scars. Transplanted kidney. Oedema. Stomas.
Types of murmurs and their positions. Grades of murmurs. Operation scars. Broad overview of common congenital defects. • Management of common cardiac defects (conservative/medical/surgical).
ASSESSMENTS IN PAEDIATRICS
be washed before touching the patient. Give a cheery introduction to the child and parent, acknowledging everyone in the room. For older children, it is appropriate to introduce yourself to the child first. Ensure the patient is adequately positioned and appropriately exposed from the start. This will reduce awkwardness later and make important signs more apparent. This also helps build initial rapport with the patient.
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FIGURE 1
HEPATOMEGALY Infective: Hepatitis, EBV HEPATOSPLENOMEGALY Infiltration: Malignancy Cystic fibrosis Obstructive: Right heart EBV/infection failure Thalassaemia Cirrhosis: Wilson’s disease, alpha -1 antitrypsin deficiency Leukaemia/Lymphoma Mucopolysaccharidoses Storage disorders: Gaucher’s disease, Glycogen storage disease
SPLENOMEGALY Myeloproliferative disorders ITP/SLE (autoimune) Spherocytosis Sickle Cell disease EBV Dodgy Liver (portal hypertension)
ASCITES Cirrhosis Heart failure Nephrotic syndrome
Example map of signs and diagnoses (abdominal system).
quiet (e.g. listening for chest sounds) in the most convenient order. Explain to the examiner: As Katie is currently settled, I would like to start centrally and examine peripherally afterwards. Equally, leave anything potentially painful or distressing to the end of the examination (e.g. ENT assessment or palpation of a tender abdomen). Always get down to the level of the patient when examining, particularly when performing exams which may cause discomfort, such as palpating the abdomen. Most importantly, be kind, be gentle, be considerate and treat the child as exactly that — a child and not an exam prop. Talk to the child, comment on their clothes, interact a little and the child is more likely to be at ease!
Concluding Tell the child and the parent when the examination has finished and offer to help them re-dress. State the next steps in the assessment. For example: To finish my abdominal examination I would like to check the external genitalia, the hernia orifices and take a urine dip. I’d also like to take a full history, perform a full multi-system examination and get a set of observations.
Presenting Findings Present the case to the examiner. A general opening could go along the lines of: I examined Katie who is 3-years-old and appears well-grown, although I would like to confirm this on an appropriate growth chart. Emphasise positive and important negative findings. Try to build a picture of related findings rather than saying them in isolation. For example: Katie has a pansystolic murmur. There are, however, no signs of hepatomegaly, oedema or basal crepitations to suggest heart failure. After presenting findings, offer a differential diagnosis, by stating: These findings may be consistent with…. Then, offer a simple management plan. Start with investigations that can be done immediately (examination, observations, bedside tests), then state more complicated management (bloods, imaging, specialist referrals). Finish with a one line summary: To summarise, Katie is a 3-year-old girl who may have a ventricular septal defect (VSD), with no evidence of heart failure. An example presentation is shown in Box 2, alongside a mark scheme in Table 3.
Communication Communication skills are vital in clinical practice. Thus, communication
has become more important in clinical examinations at both undergraduate and postgraduate level. Each scenario will vary, but some general advice is applicable to most settings.
Possible Scenarios ӹӹ Patients. Likely to be a teenager in paediatric exams (as a younger patient is less likely to be able to replicate the scenarios) or an actor playing a carer. Consider: ›› Explaining medical diagnoses, e.g. epilepsy, diabetes. ›› Consent issues, e.g. contraception/ Gillick competence. ›› Psychological issues, e.g. suicidal ideation. ӹӹ Parents. The most common scenario. Consider: ›› Breaking bad news, particularly chronic conditions, e.g. trisomy 21, cystic fibrosis, diabetes. ›› Information giving, e.g. first fit/ febrile convulsion, management of chronic conditions (e.g. asthma, eczema), breastfeeding in a HIVpositive mother. ›› Complaints, e.g. parent unhappy with service. ›› Ethical dilemmas, e.g. confidentiality and a child. ›› Safeguarding concerns, e.g. non accidental injury, Gillick competence. ›› Drug errors, e.g. double dose of gentamicin. ӹӹ Nurses. An increasingly popular station in undergraduate examinations. As a junior doctor, your relationship with nurses will determine your success and happiness! Consider: ›› Handover of a patient being admitted to the ward. ›› Discussion of a difficult case, e.g. withdrawal of care. ӹӹ Medical students/junior colleagues. Teaching skills are increasingly sought-after and examined. Be confident delivering concise teaching on core topics. A classic example is explaining the audit cycle.
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OBJECTIVE STRUCTURED CLINICAL EXAMINATIONS (OSCEs)
I examined Adeolu today, a 4-year-old-boy, Afro-Caribbean in origin, who presented with his father. He appears well grown for his age, although I would like to plot this on the appropriate growth chart. He is comfortable and pain-free at rest. On peripheral examination, he has a resting heart rate of 92 bpm with mild pallor of the conjunctivae and jaundice. On examination of his abdomen, he has a three centimetre non-tender hepatomegaly and a palpable spleen, with no other organomegaly or lymphadenopathy. I note a scar under his right clavicle which may represent a previous central line insertion. To finish my examination, I would like to examine his hernial orifices and external genitalia. I’d like to ensure there was no bony tenderness anywhere, or any signs of respiratory compromise. These findings would be consistent with a haemoglobinopathy – most likely sickle cell disease. I would also like to exclude any infectious or lymphoproliferative processes.
TABLE 3: Examination mark scheme Domain
Mark
Conduct of the examination. To include • Rapport, putting child at ease. • Appropriately professional. • Empathy and respect. • Appropriate communication.
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
Clinical Examination. To include: • Appropriate technique. • Identifies appropriate signs. • Appropriate confidence. • Able to demonstrate signs.
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
Discussion. To include: • Differential diagnosis. • Appropriate investigations. • Management considerations. FINAL GRADE.
General Approach Before starting, check which role the candidate is taking (e.g. medical student, junior doctor, senior doctor) and learn the patient’s name. Write this down if needed. Wash hands, give an introduction and check the identity of the patient. Perhaps say at the outset that: I’ve left my beeper with a colleague so we shouldn’t be interrupted. During the consultation, remember: ӹӹ Be human! If someone has just had a baby, say congratulations at
the beginning! Empathise with the patient’s story. ӹӹ Check what the patient knows first. It’s easy just to say: Before we get started, can you tell me where you’re up to so far? Use this to plan the station. ӹӹ Set your agenda. I’m here to discuss the test results today, if that’s okay. Feel free to jump in and ask any questions along the way or Would it be helpful if I explained a bit about this condition and address any concerns you might
have? This allows for the formation of a mutually agreed plan. ӹӹ Don’t give a monologue. Actively interact. Stop at intervals and check that the patient is following the conversation. It is easy to ask: Am I making sense so far? Do you have any questions at this point? Remember: this is a two-way conversation and not a viva. ӹӹ Prepare for common complex stations. Many candidates don’t know where to start with complex conditions like trisomy 21 or cystic fibrosis. Write down four sentences about each chronic, complex topic phrased in a way that is absolutely clear. Then ask: Would it be helpful if I told you a little bit about X condition? and deliver the information, modifying it slightly based on the expectations/age of the patient. For example, four sentences on Trisomy 21 might look something like: Trisomy 21 is commonly called Down syndrome. This is caused when babies inherit a bit of extra genetic material from their parents. This is no one’s fault and can’t be predicted. This has many effects ranging from mild learning difficulties to more significant effects on the heart and internal organs. It’s a common genetic condition and there is a huge amount of support available including medical teams, special schools and parental groups. Someone will be with you every step of the way. With this support, many children live happy, fulfilled lives well into their fifties and sixties.
ASSESSMENTS IN PAEDIATRICS
Box 2: Presenting examination findings
Finishing Off Have a plan for the warning bell. Think about common things that are missed, or things that are best left to the end: ӹӹ Ideas/concerns/expectations. Ask Do you have any other concerns that you’d like to mention? This may uncover an important point that has been completely missed. ӹӹ Summarise the key points of the consultation into two sentences. This is an important component of a highscoring station.
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ӹӹ Offer leaflets, mention parent support groups, make a plan for follow up. For example: I know this has been a lot to take in, so why don’t we meet up again tomorrow to discuss this further? Provide contact details. Make a positive plan to move forward – for example after discussing a difficult case with a medical student, suggest they spend time in a specialist clinic to learn more. A possible mark scheme is shown in Table 4.
Practical Skills Obtain clinical experience in the relevant skills before the assessment. Step-by-step guides to common procedures are shown on p613. Other practical skills that may be assessed are listed below.
Plotting a Growth Chart Growth charts are simple but must be learned prior to the exam. Be aware that there are a number of different growth charts. These are primarily age-related, including neonatal, 0 to 4-years-old, and 2 to 18-years-old. However, there are also conditionspecific charts, including Down syndrome and achondroplasia. Be prepared to plot serial growth measurements on these charts and be prepared to talk about differentials for faltering growth (p247).
Prescribing Prescribing is increasingly being recognised as a neglected skill in medical education. Know how to calculate fluid requirements. Know how to use common reference sources (e.g. BNF) to calculate appropriate drug dosages (these do not need to be
TABLE 4: Communication mark scheme Domain Conduct of the consultation. To include: • Introduction, clarifies role. • Agrees aims and objectives. • Rapport. • Empathy and respect. Explanation and information. To include: • Clear explanation, no jargon. • Assesses patient’s prior knowledge. • Appropriate style. • Explores and responds to concerns/feelings. • Summarises and checks understanding. Accurate information giving. To include: • Appropriate selection of information, including level/ age-appropriateness, etc. • Accuracy of information/ clinical knowledge. FINAL GRADE
Mark
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
Clear Pass
Pass
Bare fail
Clear fail
Unacceptable
memorised). This station is not just about the drug prescription itself, but also the rest of the drug chart e.g. patient details, such as allergies, hospital number, address, name and date of birth. More detailed prescribing guidance is shown on p648.
DR ABCDE (acute assessment and resuscitation of the unwell child) In an acute scenario, remember to be systematic. Assess airway, breathing, circulation, disability and exposure. This is done in the same way for every case, so practice and be confident. Ensure each letter of the alphabet is addressed before moving to the next. Treat each problem as soon as it is identified, and reassess from the beginning after each intervention. A common structure within each letter is to “look, feel, listen, measure, treat”. Practise this structure for the airway, breathing and circulation components. Key points to consider include: ӹӹ Ask for the observations at the very beginning. The main benefits of this are two-fold. It offers a crude idea of what system is affected, depending on which observations are most deranged. It also provides an opportunity to call for help early in response to abnormal observations. ӹӹ If appropriate, give oxygen during the assessment of “Airway”. ӹӹ Remember the scenario puts the candidate in the position of team leader. Others in the scenario will be role-playing so use them wisely. ӹӹ Remember the parents in the scenario. They may help calm the child down, but they also need to be updated with progress throughout. ӹӹ Reassess thoroughly after every treatment. ӹӹ Never forget to check the glucose, as this is a common yet reversible cause of acute deterioration. A detailed guide on the assessment of the acutely unwell child is shown on p7.
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A GUIDE TO BEING A JUNIOR DOCTOR IN PAEDIATRICS MARIE MONAGHAN AND MICHAEL MALLEY
GENERAL PREPARATION
P CONTENTS 665 General Preparation 668 General Paediatric Wards
668 Completing Ward Rounds 668 Reviewing a Patient 670 Discussing a Patient with Another Hospital or Subspecialty 670 Discharging a Patient and Arranging a Follow-up Review 670 Updating Parents and Caregivers about Progress
670 Neonatal Units
671 Morning Neonatal Unit Handover and Night Summaries 671 Attending Deliveries and Neonatal Crash Calls
672 Postnatal Wards
672 Performing Baby Checks 673 Reviewing Babies on the Postnatal Ward
aediatrics is a fulfilling and stimulating career, offering the potential to bring joy to children and their families. A unique specialty with its own diverse range of conditions, paediatrics requires a flexible approach, an appreciation for the breadth of medicine, and a dextrous hand for the wide range of procedures involved. As much as it demands, it rewards. Paediatricians laugh, play games and most certainly become familiar with the most popular Disney films and children’s TV shows! Although exciting for those already familiar with paediatrics, not surprisingly, those starting out can find this field incredibly daunting after an education focused on adult-centred medicine (Table 1). Before starting a general paediatric or neonatal job, practitioners may benefit from attending a paediatric life support course. These are usually incorporated into paediatric induction training. Being confident in resuscitation will lead to increased confidence when facing the most demanding situations. This training can also lend a structured approach to the care of any child and provide advice on the most effective way to communicate and interact in resuscitation scenarios. Induction training will also offer a good opportunity to explore the specific working environment of the paediatric department. Table 2 shows the key areas in Paediatric units, and Table 3 shows those areas that aid in coordinating patient care. Multidisciplinary teamwork is extremely important in paediatrics, meaning that effective communication is paramount for ensuring all parties are up to date with developments in a child’s care. Being open with colleagues about previous levels of experience will help them tailor teaching, day-to-day task allocation and senior support. The main chapters of the book give detailed information on key conditions encountered in paediatrics and key skills required for assessing and treating children. What follows is additional practical information to help with adaptation to life on paediatric wards.
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TABLE 1: Examples of similarities and differences between adult and paediatric medicine Similarities
Differences
Certain conditions that affect adults also affect children; for example, infection, asthma, diabetes and fractures (although the management of these are often specific to children).
In paediatrics, many conditions, such as diabetes or asthma, are diagnosed for the very first time in a patient’s life, rather than forming part of their past medical history. Many rare conditions can also occur, like those with a genetic, developmental or metabolic origin, which are diagnosed in the paediatric population but are not associated with survival to adulthood.
Resuscitation training is a requirement in both adult and paediatric medicine.
Children, unlike adults, rarely undergo resuscitation due to a primarily cardiac cause. Most severe illnesses stem from respiratory or systemic causes, like asthma or sepsis. These contrasting causative factors mean that resuscitation is focussed on supporting the airway and breathing in the sick child. In terms of resuscitation outcomes, children are regarded as being better able to compensate for illness, respond to resuscitative efforts and recover after significant physiological insults. Similarly, ward admissions are often brief for children (one to two days) following response to appropriate medical intervention. The increasing use of ambulatory care, where patients are cared for on a ward–attender basis or are given medical care in the home, can potentially avoid the need for inpatient admission.
A structured examination approach and comprehensive history-taking are required in adult and paediatric medicine.
In paediatrics, history-taking has a broader social emphasis, and it includes paediatric-specific topics such as birth history, developmental history, immunisation status and safeguarding concerns. The source of the history is generally caregivers rather than the child, but a history may also be taken directly from the child, depending on their age and level of development. In paediatric examinations, while a thorough examination is required, the course of the examination is often opportunistic and adaptive – for convenience and to maintain the ease of the child. An example of this might be the examination of a child’s gait as they walk into an examination room, unaware of observation, or the auscultation of an infant’s heart sounds as they lie asleep. The majority of paediatric inpatient admissions are to a non-specialist, general paediatric ward, where care is managed by an admitting general paediatric consultant with an accompanying medical team, plus any necessary specialist input required. Planned simple procedures in paediatrics, when invasive, require preparation (for example, topical analgesia) and often the assistance of nursing staff and play specialists. Junior doctors in paediatrics are not expected to be accomplished in intravenous cannulation and are not expected to perform these procedures independently at the outset. Procedural skills are developed with senior support. Paediatrics also has a wider range of advanced procedures, such as endotracheal intubation, chest drain insertion, cranial ultrasounds, and central line insertion (umbilical or long line access).
TABLE 2: Locations in paediatric hospital care Location
Function
Playroom.
Full of many toys and games, with activities organised by play therapists. Often a place where children are hiding when they can’t be found!
Teenage room.
May contain age-appropriate activities for teenagers, such as books and computer games.
Treatment room.
Procedures are done here: this helps the bed space remain a safe, comfortable environment for the child and means that a distressed child can be managed in privacy.
School room.
Dedicated teachers provide ongoing education for inpatients.
Parents’ room/ accommodation.
A room is often provided for parents to stay in. This may be within the ward, or it might be in a separate nearby building. A room with basic facilities for sitting and relaxing may also be available.
Hospital at home.
Children may remain under hospital care, but be visited by “hospital at home” nurses for ongoing treatment, such as intravenous antibiotics. These patients are discussed at a daily virtual hospital ward round.
Sensory room.
Contains interesting lights and colours, and soft floor padding. Provides a relaxing space, particularly great for younger children and those with learning difficulties.
Short stay unit.
For children being admitted for 24- 48 hours, although some allow for slightly longer durations of stay.
Day care unit.
Usually for patients not requiring an overnight stay or attending for a short period; for example, attending for allergy testing, for antibiotics, or for a sleep study or day surgery. Often primarily nurse-led.
Milk room.
For preparation and storage of milk, both formula and breast. Continued
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TABLE 2: Continued Location
Function
Labour ward/Obstetric theatre.
Where pregnant women go to deliver.
Postnatal ward.
Where mother and babies go after delivery.
Cubicle.
For infectious/immunocompromised patients to isolate them from other patients.
Paediatric Emergency Department.
Often there is a separate dedicated area of the Emergency Department (ED) just for paediatrics.
Resuscitation area.
The ED will have a dedicated paediatric resuscitation (resus) bay, as well as resus areas on the postnatal and paediatric wards. Incubator spaces in the NICU contain most resuscitation equipment, but a separate resus area is available where the more stable babies can be brought if they deteriorate.
TABLE 3: People available to help deliver care. Units vary in which personnel are actually available and their roles.
Paediatric ward.
Colleague
Role
Child protection (safeguarding) nurse/doctor.
There is an allocated child protection nurse and doctor in every paediatric unit.
Children’s community nurse.
Works alongside families to provide nursing support and monitor the effectiveness of any care package. May be important in discharge planning.
Clinical support worker.
Works alongside allied health professionals (e.g. physiotherapists) to prepare for and deliver therapy.
Dietician.
Gives advice on feeding and nutrition.
Health visitor.
Their role primarily involves visiting families in their homes, and they routinely visit all new parents. Concerns about vulnerable children identified in the hospital may be followed up by a health visitor.
Healthcare assistant/ Auxiliary nurse.
Works under the supervision of a nurse/midwife to help deliver care, including things like taking observations and washing/dressing patients.
Occupational therapist.
Helps provide practical solutions for any child with a physical or psychiatric impairment. This may involve providing equipment or home adaptation.
Orthoptist.
Helps manage eye movement problems, e.g. squints, alongside ophthalmologists.
Paediatric nurse practitioner/Site manager.
Responsible for coordinating care through all paediatric wards and the ED. Helps facilitate discharges and achieve conflict resolution.
Parent.
A parent usually resides most of the time with a sick child in hospital. The parent will often help to deliver care and may provide a vital observation indicating that the child has deteriorated.
Police.
May be involved in child protection cases. Present in the ED for out-of-hospital cardiac arrests.
Pharmacist/Pharmacy technician.
Can provide advice on specific dosing and formulation of drugs. Can also liaise with families and primary care physicians on exact regular medications. Vital source of help for doing difficult prescriptions, and they will check all prescriptions regardless. Pharmacists need to be alerted early about any discharges so they can prepare medications.
Physician’s assistant.
Trained to support doctors in the medical management of children. Roles vary depending on experience.
Physiotherapist.
Helps patients with balance and mobility problems. Chest physiotherapy is particularly helpful in cystic fibrosis.
Play specialist.
Experts in child-appropriate play. Vital support in distraction for practical procedures.
Psychologist.
Clinical psychologists will assist in management of mental health problems, behavioural problems and patients with difficulty adjusting to a new diagnosis/illness. Educational psychologists (usually working in the community) address psychological issues to enhance a child’s learning ability.
A GUIDE TO BEING A JUNIOR DOCTOR
GENERAL PREPARATION
Continued
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TABLE 3: Continued
Labour ward and post-natal ward.
Colleague
Role
Specialist nurse.
Nurses have wide ranges of potential specialities, including common diseases such as diabetes, cystic fibrosis and asthma, as well as more esoteric areas like small bowel transplants. Specialist nurses may review patients on the ward and see them in follow-up clinics independently.
Social worker.
Provides support to vulnerable children/families. For any child protection concerns, it is worth checking to see if they are known to social services already. Social workers are crucial in coordinating child protection investigations.
Speech and language therapist (SALT or SLT).
Helps those with speech and language problems, wider communication difficulty and feeding problems.
Staff nurse.
Doctors work more closely with the nursing staff than any others. The staff nurses deliver overall care for the patient, including administering medication, performing observations/ investigations and highlighting the deteriorating patient.
Nurse-in-charge.
The nurse-in-charge actively manages the activity on the ward. Regular updates are helpful for the nurse-in-charge regarding management of the ward patients, particularly discharges.
Ward sister.
Supervises the work of the nursing/health care assistant team. As experienced paediatric nurses, they are a great source of advice if you are stuck!
Ward clerk.
Able to retrieve notes, book appointments, answer phone calls, and welcome patients/ families to the ward.
Midwife.
Leads delivery of low risk pregnancies and supports resuscitation of sick newborns. Trained in newborn life support (NLS). Able to provide advice on many aspects of neonatology, particularly normal variations in newborns and feeding issues.
Obstetrician.
Will make decisions on any urgent deliveries. May request counselling for a mother before a preterm delivery, and may hold discussions about bed status in the NICU before a delivery is planned or pregnant women with high risk deliveries are admitted to the ward.
Anaesthetist.
Present in theatre for Caesarean sections. May often help in neonatal resuscitations, but aren’t necessarily qualified in NLS.
Breastfeeding advisor.
Able to provide specific guidance to mothers with difficulty establishing feeding.
Nursery nurse.
Often available on the postnatal ward and will help deliver basic care to newborn babies.
GENERAL PAEDIATRIC WARDS Completing Ward Rounds Ward rounds take place daily on a paediatric ward and are led by the consultant or the registrar. The average length of stay for children is two days, and about 50% of patients stay less than 24 hours. The role of the junior doctor on the ward round is to: ӹӹ Ensure blood test/investigation results are available. ӹӹ Ensure patient details are written on every sheet of the written patient notes. ӹӹ Document information gathered (history, examination, observations, blood results, parental/patient thoughts). ӹӹ Document action plans. ӹӹ Document who was responsible for decisions. ӹӹ Ask questions if anything appears to have been missed/ is unclear. A typical ward round entry is shown in Box 1. Parents, carers and nurses should be present on ward rounds wherever possible to help effectively communicate the care of the paediatric patient. Multidisciplinary ward rounds also
take place, and ensure coordinated care from the multiple professionals involved in any child’s care.
Reviewing a Patient Junior doctors in paediatrics are neither intended nor expected to manage acutely unwell children independently. However, they are often the first members of the medical team to initially assess a patient. If asked to review an unfamiliar patient, ask for a brief SBAR (Situation, Background, Assessment and Response) format handover (Box 2), as this aids the prioritisation and management of the review. Abnormal observations will often have independently generated a medical review based on the recommendations of the now widely used Paediatric Early Warning Score systems in use on nurses’ observation charts. One common reason for review is to consider “stretching” a wheezy patient on salbutamol therapy. This is carried out to determine whether a patient is improving or deteriorating on current therapy. In cases where the patient’s breathing is improving, the aim is to reduce the frequency of the medication (e.g. moving from two-hourly to three-hourly salbutamol).
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Box 1: Typical ward round entry. Note patients details usually provided with a pre-printed sticker. There is also often a separate parent communication sheet to document discussions. 29.6.15
WR Fred Smith (Consultant)
10.54 a.m.
Sarah King P54030493 29.3.15
3 month old boy, admitted 28.6.15 Diagnosis:
D3 RSV+ve Bronchiolits Ex prem (32 weeks)
Investigations: CRP