OXFORD MEDICAL PUBLICATIONS Oxford Handbook of Pediatrics 00_McClure_Prelims.indd i 11/14/2012 11:16:23 PM Published and upcoming Oxford Handbooks Oxford Handbook for the Foundation Program 3e Oxford Handbook of Acute Medicine 3e Oxford H andbook of Anesthesia 3e Oxford Handbook of Applied Dental Sciences Oxford Handbook of Card iology 2e Oxford Handbook of Clinical and Laboratory Investigation 3e Oxford Handbook of Cl inical Dentistry 5e Oxford Handbook of Clinical Diagnosis 2e Oxford Handbook of Clinical Ex amination and Practical Skills Oxford Handbook of Clinical Hematology 3e Oxford Handbook of Clinical Immunology and Allergy 3e Oxford Handbook of Clinical Medicine \200\223 Mini Edi tion 8e Oxford Handbook of Clinical Medicine 8e Oxford Handbook of Clinical Pathology Oxfor d Handbook of Clinical Drugstore 2e Oxford Handbook of Clinical Rehabilitation 2e Oxford Ha ndbook of Clinical Specialties 9e Oxford Handbook of Clinical Surgery 4e Oxford Handbook of Complementary Medicine Oxford Handbook of Critical Care 3e Oxford Handbook of Dental Patie nt Care 2e Oxford Handbook of Dialysis 3e Oxford Handbook of Emergency Medicine 4e Oxford H andbook of Endocrinology and Diabetes 2e Oxford Handbook of ENT and Head and Neck Surgery O xford Handbook of Epidemiology for Clinicians Oxford Handbook of Expedition and Wilderness Medicine Oxford Handbook of Gastroenterology & Hepatology 2e Oxford Handbook of General Pra ctice 3e Oxford Handbook of Genetics Oxford Handbook of Genitourinary Medicine, HIV and AID S 2e Oxford Handbook of Geriatric Medicine Oxford Handbook of Infectious Diseases and Micro biology Oxford Handbook of Key Clinical Evidence Oxford Handbook of Medical Dermatology Oxf ord Handbook of Medical Imaging Oxford Handbook of Medical Sciences 2e Oxford Handbook of M edical Statistics Oxford Handbook of Nephrology and Hypertension Oxford Handbook of Neurolo gy Oxford Handbook of Nutrition and Dietetics 2e Oxford Handbook of Obstetrics and Gynecolo gy 2e Oxford Handbook of Occupational Health 2e Oxford Handbook of Oncology 3e Oxford Handb ook of Ophthalmology 2e Oxford Handbook of Oral and Maxillofacial Surgery Oxford Handbook o f Pediatrics 2e Oxford Handbook of Pain Management Oxford Handbook of Palliative Care 2e Ox ford Handbook of Practical Drug Therapy 2e Oxford Handbook of Pre-Hospital Care Oxford Hand book of Psychiatry 3e Oxford Handbook of Public Health Practice 2e Oxford Handbook of Repro ductive Medicine & Family Planning Oxford Handbook of Respiratory Medicine 2e Oxford Handbo ok of Rheumatology 3e Oxford Handbook of Sport and Exercise Medicine Oxford Handbook of Tro pical Medicine 3e Oxford Handbook of Urology 3e 00_McClure_Prelims.indd ii 11/14/2012 11:16:23 PM Oxford Handbook of Pediatrics Second Edition Edited by Robert C. Tasker Professor of Neurology and Anesthesia (Pediatrics), Harvard Medical School ; Chair in Neurocritical Care, Children \200\231s Hospital, Boston, USA Robert J. McClure Neonatologist, Pediatrician and Anatomical Pathologist Queen Elizabeth Me dical Center, Nedlands, Perth, Western Australia Carlo L. Acerini University Senior Lecturer, Cambridge University Clinical School, Cambridg e, UK 1 00_McClure_Prelims.indd iii 11/14/2012 11:16:23 PM 3 Great Clarendon Street, Oxford, OX2 6DP, United Kingdom Oxford University Press is a department of the University of Oxford. It furthers the Univer sity \200\231s objective of excellence in research, scholarship, and education by publishin g worldwide. Oxford is a registered trade mark of Oxford University Press in the UK and in certain other countries Oxford University Press, 2013 The moral rights of the author have been asserted First edition published 2008 Second edition published 2013 Impression: 1 All rights reserved. No part of this publication may be reproduced, stored in a retrieval s ystem, or transmitted, in any form or by any means, without the prior permission in writing of Oxford University Press, or as expressly permitted by law, or under terms agreed with t he appropriate reprographics rights organization. Enquiries concerning reproduction outside the scope of the above should be sent to the Rights Department, Oxford University Press, a t the address above You must not circulate this book in any other binding or cover and you must impose the same condition on any acquirer British Library Cataloging in Publication Data Data available ISBN 978 \200\2230 \200\22319 \200\223960830 \200\2230 ( \202 exicover: alk.paper) Printed in China by C&C Offset Printing Co. Ltd. Oxford University Press makes no representation, express or implied, that the drug dosages in this book are correct. Readers must therefore always check the product information and c linical procedures with the most up-to-date published product information and data sheets p rovided by the manufacturers and the most recent codes of conduct and safety regulations. T he authors and the publishers do not accept responsibility or legal liability for any error s in the text or for the misuse or misapplication of material in this work. Except where ot herwise stated, drug dosages and recommendations are for the non-pregnant adult who is not breastfeeding. 00_McClure_Prelims.indd iv 11/14/2012 11:16:23 PM v Foreword to the \201 rst edition Textbooks have been the mainstay of medical education for centuries. Clearly, the developme nt of the information superhighway via the Internet has changed how we learn, \201 nd inf ormation, and communicate. What does yet another pediatric textbook add to the current long list of titles? Drs Tasker, McClure, and Acerini have conceived of and edited a new book. It is a handbook of pediatrics that joins a stable of similar publica- tions from Oxford University Press. T here are 23 contributing editors. Using a well-tested format for presentation, the handbook consists of 31 chapters, ranging from sections on epidemiology, evidence, and practice, th rough the more traditional topics, such as nephrology and neurology, and concluding with in ternational health and travel, and pediatrics, eth- ics, and the law. Each chapter follows the same format, 5 \200\22340 sections, fol- lowed by bulleted points. Both signs and sympt oms of illness, as well as speci \201 c diseases are covered. Virtually all topics are lim ited to 1 \200\2232 pages of important information. Tables are carefully inserted, and comp lement the text. Doses of important drugs are included in the text and/or the tables. There are a limited number of \201 gures, but like the tables, they supplement the text and ha ve been carefully chosen to add clarity. The Oxford Handbook of Pediatrics is a worthy addition to your library. It will be particul arly appealing to medical students and younger physicians, who have learned to digest a gre at deal of information quickly and in an abbreviated format. Its availability on a CD-ROM i s an added and neces- sary bene \201 t. Drs Tasker, McClure, and Acerini have done a wonde rful job in ensuring consistency, clarity, and completeness. Professor Howard Bauchner, Boston University School of Medicine/Boston Medical Center, Vise -Chair, Academic Affairs, Editor in Chief, Archives of Disease in Childhood, January 2008 00_McClure_Prelims.indd v 11/14/2012 11:16:23 PM vi Preface The \201 rst \200\230boke \200\231 of pediatrics printed in English was written by Thoma s Phaire (1510 \200\2231560), a man from East Anglia who studied medicine at Oxford Univers ity. The book had 56 pages, measured 3 7/8 inches (9.8cm) by 2 5/8 inches (6.7cm), and cove red \200\230 innumerable passions & diseases, wherunto the bodye of man is subiecte, and as well moste commonly the tender age of chyldren is chefely vexed and greued with thes e dis- eases folowyng. Apostume of the brayne, swellyng of the head \200\231.1 In 155 3, the \200\230innumerable passions & diseases \200\231 came to 39 presenting clini- cal p roblems. As clinicians, we \201 rst met and worked in the heart of East Anglia (Cambridge University) and have now collaborated with Oxford University Press in this venture, a new handbook of pediatrics. Our simi- larity with Thomas Phaire has not escaped us, particularl y as we see the importance of basing a text on common presenting clinical problems. Our principal aim is to provide a compact source of information and clinical thinking that can be used in the clinic or hospital ward, at a time when the child is being seen. The cha llenge, therefore, was to distill the content of information found in several textbooks int o a conveniently sized handbook without the loss of important information. We easily reache d the limit in pages given to us, and so we have had to be strict in sifting out key facts crucial to clinical practice. Our intention is that the handbook be used from the start of one \200\231s education in pediatrics all the way through to higher general training in the \201 eld. We have kept with the tradition of providing content and text that often exceeds that requi red by the generalist \200\224we believe it important for learners and readers to see the f ull landscape. There are spaces where more notes can be added from lectures, other reading, and personal expe- rience. This is intended. It means that the handbook can be made person al, develop with you, and be used in whatever your chosen practice \200\224 hospitalist, ge neralist, or community and family practice. Above all, we hope that the handbook will give you con \201 dence to manage pediatric clinical problems effectively and safely. RCT RJM CLA April 2012 Reference 1 Phaire T (1553). The boke of chyldren. [Reprint edited by Neale AV, Wallis HRE (1965). Edinburgh: E&S Livingstone Ltd, Edinburgh. 00_McClure_Prelims.indd vi 11/14/2012 11:16:23 PM vii Authors \200\231 disclaimer All reasonable efforts have been undertaken in order to ensure the accu- racy of drug doses in this book. UK readers are advised to also consult the British National Formulary for ch ildren (2012; see http://www.bnf. org/bnf/index.htm)). Other readers should refer to their own regional or national guidelines. The authors cannot be held responsible for any errors here in. 00_McClure_Prelims.indd vii 11/14/2012 11:16:23 PM viii Acknowledgements We would like to extend our thanks and gratitude to all the contributors. We would also lik e to thank our colleagues who reviewed and advised on the content of our chapters, in parti cular Dr Robert Ross-Russell, Dr Roddy O \200\231Donnell, Jenny Pool, Amy Stewart, Clare Br adley Stevenson, and Liesje Cornwell for their helpful comments. We are also indebted to Dr Stephan Sanders for his comments and criticisms of our draft manu- script. We would also l ike to thank Drs Kim Jones and Tony Jaffa, Profs Deirdre Kelly and Brett McDermott, and Ms Julia Smith, Kelly Lamour, and Lynne Radbone for their contribution to the last edition. We are especially grateful to Beth Womack and Elizabeth Reeve at OUP for their help and assis tance, and for their patience with us. Finally, but not least, a special thanks goes to our respective families for their encouragement, support, and understanding throughout the pre paration of this book. Robert C. Tasker Robert J. McClure Carlo L. Acerini RJM is indebted to Marge, Hannah, and Thomas, for their endless love, support, and sacri \201 ce. 00_McClure_Prelims.indd viii 11/14/2012 11:16:23 PM ix Contributors Mr David Albert Consultant Otolaryngologist, Great Ormond Street Hospital for Children NHS Foundation Trust, London Dr David Coghill Ninewells Hospital and Medical School, Dundee, UK Mr David Crabbe Consultant Pediatric Surgeon Leeds Teaching Hospitals NHS Trust and Bradfor d Teaching Hospitals NHS Foundation Trust, UK Miss Louise Allen Consultant Pediatric Ophthalmologist Cambridge University Hospitals NHS F oundation Trust, Cambridge, and Associate Lecturer, University of Cambridge, UK Dr Saul N Faust Reader in Pediatric Immunology and Infectious Diseases and Director, NIHR W ellcome Trust Clinical Research Facility, University of Southampton, UK Dr R Mark Beattie Consultant Pediatric Gastroenterologist University Hospital Southampton S outhampton, UK Dr Rob Freeman Consultant Orthopedic Surgeon, Robert Jones and Agnes Hunt Orthopedic Hospit al NHS Trust, Shropshire, UK Mr Yogesh Bajaj Consultant Pediatric Otolaryngologist Barts, and the London Children \200 \231s Hospital, London Dr Georgina Hall Consultant, Pediatric Hematology/Oncology Unit, John Radcliffe Hospital, O xford, UK Dr Ian Balfour-Lynn Consultant in Respiratory Pediatrics Royal Brompton Hospital, London Dr Peter Heinz Consultant Pediatrician Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK Dr Tony Caccetta Dermatology Registrar Princess Margaret Hospital, Perth, Western Australia Dr Ewen D Johnston Consultant Neonatologist Simpson Center for Reproductive Health, Royal I n \201 rmary of Edinburgh, UK Professor Imti Choonara Professor in Child Health, Academic Division of Child Health, Unive rsity of Nottingham, Derbyshire Children \200\231s Hospital Derby, UK 00_McClure_Prelims.indd ix 11/14/2012 11:16:24 PM x CONTRIBUTORS Dr Samir Lati \201 Consultant in Pediatric Intensive Care, Akron Children \200\231s Hospit al Akron, Ohio, USA Dr Willie Reardon Consultant Clinical Geneticist, Our Lady \200\231s Hospital for Sick Chil dren, Dublin, Ireland Dr Elaine Lewis Consultant Community Pediatrician, Cambridge University Hospitals NHS Found ation Trust, Cambridge, UK Dr Lesley Rees Consultant Pediatric Nephrologist Great Ormond Street Hospital for Children Foundation Trust, London Dr James C Nicholson Consultant Pediatric Oncologist Cambridge University Hospitals NHS Fou ndation Trust, Cambridge, UK Professor Benjamin J Stenson Consultant Neonatologist Simpson Center for Reproductive Healt h, Royal In \201 rmary of Edinburgh, UK Dr Roddy O \200\231Donnell Consultant in Pediatric Intensive Care, Cambridge University Hos pitals NHS Foundation Trust, Cambridge, UK Dr Robert M R Tulloh Consultant in Pediatric Cardiology, Bristol Royal Hospital for Childre n and Bristol Royal In \201 rmary, UK Dr Alasdair Parker Consultant Pediatric Neurologist, Cambridge University Hospitals NHS Fou ndation Trust, Cambridge, UK 00_McClure_Prelims.indd x 11/14/2012 11:16:24 PM CONTENTS xi Contents Detailed contents xiii Symbols and abbreviations xxxii 1 Practicing pediatrics 2 Epidemiology, evidence, and practice 3 Clinical assessment 4 Resu scitation 5 Emergency and high dependency care 6 Neonatology 7 Practical procedures 8 Cardi ovascular 9 Respiratory medicine 10 Gastroenterology and nutrition 11 Nephrology 12 Endocri nology and diabetes 13 Growth and puberty 14 Neurology 15 Child development 16 Child and fa mily psychiatry 17 Hematology 18 Oncology 19 Infectious diseases 20 Bones and joints 21 Ado lescent health 22 Dermatology 23 Pediatric surgery 24 Special senses 1 9 19 35 45 107 201 225 253 297 349 399 455 495 555 567 607 651 693 731 793 805 843 893 00_McClure_Prelims.indd xi 11/14/2012 11:16:24 PM xii CONTENTS CONTENTS 25 Genetics 26 Inherited metabolic disease 27 Community child health 28 Child protection 29 Pharmacology and therapeutics 30 International health and travel 31 Pediatrics, ethics, an d the law 925 953 979 997 1011 1021 1029 Index 1047 00_McClure_Prelims.indd xii 11/14/2012 11:16:24 PM xiii Detailed contents 1 Practicing pediatrics 1 Reading and learning pediatrics 2 Professional conduct and attitudes 3 Professional skills 4 Knowledge 6 2 Epidemiology, evidence, and practice 9 Introduction 10 Descriptions in populations 10 Summary of study designs 11 Levels of eviden ce 12 Basics of statistics 14 Training and special knowledge skills 16 Useful websites and resources 18 References 18 3 Clinical assessment 19 Communication skills 20 Taking a pediatric history: introduction 21 The presenting complain t 22 History of present illness 24 Past health history 25 Symptom review 26 Family history 28 Examining a child: introduction 28 General condition 29 Vital signs 30 Respiratory syste m 31 Cardiovascular system 32 Gastrointestinal system 33 Genitourinary system 34 Musculoske letal system 34 00_McClure_Prelims.indd xiii 11/14/2012 11:16:24 PM xiv DETAILED CONTENTS 4 Resuscitation 35 Cardiopulmonary arrest 36 Rapid cardiopulmonary assessment 36 Pediatric basic life support 38 Choking children 39 Pediatric advanced life support 40 Rhythm disturbances 42 Treating s upraventricular tachycardia 43 Following unsuccessful resuscitation 44 5 Emergency and high dependency care 45 The ABC of high dependency 46 Respiratory distress 48 Respiratory distress: management 50 F oreign-body inhalation 51 Drowning 52 Circulation: cardiovascular dif \201 culty 53 Cardio vascular system dif \201 culty: assessment 54 Cardiovascular system dif \201 culty: thera py \200\2241 56 Cardiovascular system dif \201 culty: therapy \200\2242 58 Cyanosis: asses sment 60 Cyanosis: management 62 Anaphylaxis 64 Hypovolaemic shock 65 Burns 66 Burns: treat ment 68 Sepsis 70 Altered level of consciousness 72 Altered level of consciousness: clinica l assessment 74 Altered level of consciousness: Glasgow coma scale 76 Altered level of cons ciousness: management 78 Status epilepticus 80 Poisoning 82 Poisoning: management 84 Poison ing: antidotes and substrates 86 Fluid and electrolytes 89 Fluid and electrolytes: dehydrat ion 90 Fluid and electrolytes: abnormalities 92 Renal insuf \201 ciency 94 00_McClure_Prelims.indd xiv 11/14/2012 11:16:24 PM DETAILED CONTENTS xv Glucose: hypoglycaemia 96 Diabetic ketoacidosis 98 Diabetic ketoacidosis: treatment 100 Inb orn error of metabolism 102 Other acid \200\223base problems 104 Further reading 106 6 Neonatology 107 Newborn life support 108 Perinatal de \201 nitions 110 Small for gestational age 111 Large for gestational age 112 Prematurity 114 Birth at the limit of viability 116 Outcome follow ing prematurity 117 Basic obstetrics 118 Obstetric problems 120 Maternal disorders causing neonatal disease 124 Birth trauma 126 Non-speci \201 cally ill neonate 128 Neonatal jaundi ce 130 Hypoglycaemia 132 Neonatal seizures 134 The \202 oppy infant 136 Hydrops foetalis 138 Routine care of the newborn 140 Milk feeding 142 Routine neonatal examination 144 Norma l variations and minor abnormalities 146 Newborn \202 uid and electrolyte balance 148 Res piratory distress syndrome 150 Acute respiratory diseases 152 Neonatal X-rays 156 Neonatal respiratory support 160 Conventional positive pressure ventilation 162 High frequency oscil latory ventilation 164 Bronchopulmonary dysplasia 166 Circulatory adaptation at birth 168 P atent ductus arteriosus 169 CNS malformations 170 00_McClure_Prelims.indd xv 11/14/2012 11:16:24 PM xvi DETAILED CONTENTS Hypoxic \200\223ischaemic encephalopathy 172 Cerebral hemorrhage and ischaemia 176 Necrotiz ing enterocolitis 178 Neonatal infection 180 Transplacental (congenital infection) 182 Prev ention of neonatal infection 184 Neonatal abstinence syndrome 186 Inborn errors of metaboli sm 187 Retinopathy of prematurity 188 Metabolic bone disease 190 Orofacial clefts 191 Neona tal hematology 192 Rh disease (rhesus haemolytic disease) 194 Bilirubin encephalopathy (ker nicterus) 195 Neonatal dermatology 196 Perinatal death 198 7 Practical procedures 201 Capillary blood sampling 202 Venepuncture 203 Intravenous cannulation 204 Peripheral arteri al blood sampling 205 Peripheral arterial cannulation 205 Umbilical arterial catheter 206 U mbilical venous catheter 208 Central venous catheterization via a peripheral vein 209 Airwa y management 210 Mask ventilation 211 Endotracheal intubation 212 Insertion of a chest drai n 214 Intraosseous infusion 216 Intracardiac injection 216 Pericardiocentesis 217 Abdominal paracentesis 217 Urethral bladder catheterization 218 Suprapubic aspiration of urine 219 L umbar puncture 220 Cerebral ventricular tap 221 Exchange transfusion 222 00_McClure_Prelims.indd xvi 11/14/2012 11:16:24 PM DETAILED CONTENTS xvii 8 Cardiovascular 225 Common presentations 226 Cyanosis 226 Heart failure 226 Heart murmurs 228 Murmurs: clinical features 230 Acyanotic: congenital heart disease 232 Left to right shunt: atrial septal de fect 233 Ventricular septal defect 234 Persistent ductus arteriosus 234 Right to left shunt : tetralogy of Fallot 235 Transposition of the great arteries 236 Common mixing: complete a trioventricular septal defect 236 Tricuspid atresia 237 Aortic stenosis 237 Pulmonary steno sis 238 Coarctation of the aorta 238 Hypoplastic left heart syndrome 239 Total anomalous pu lmonary venous connection 239 Hypertrophic obstructive cardiomyopathy 240 Dextrocardia 240 Infection: infective bacterial endocarditis 242 Rheumatic fever 244 Pericarditis 246 Myocar ditis 248 Cardiomyopathy 249 Cardiac arrhythmias 250 9 Respiratory medicine 253 Introduction 254 Common presentation: wheeze 255 Common presentation: stridor 256 Common pr esentation: cough 257 Common presentation: breathlessness 258 Common presentation: snoring 259 Investigations 260 Asthma 262 Asthma: drug delivery devices 264 Asthma: clinic manageme nt (1) 266 00_McClure_Prelims.indd xvii 11/14/2012 11:16:24 PM xviii DETAILED CONTENTS Asthma: clinic management (2) 268 Cystic \201 brosis 270 Cystic \201 brosis: problems 2 71 Cystic \201 brosis: management (1) 272 Cystic \201 brosis: management (2) 274 Chroni c lung disease of prematurity 276 Congenital respiratory tract disorders 278 Sleep apnoea 2 79 Allergic rhinitis 280 Upper airway infections 282 Laryngeal and tracheal in \202 ammati on 284 Bronchial disease 286 Bronchiolitis 288 Pneumonia 290 Pneumonia: treatment 292 Pneum onia: effusion, empyema 293 Pulmonary tuberculosis 294 Other disorders 295 10 Gastroenterology and nutrition 297 Healthy eating for children 298 Vomiting 300 Acute diarrhea 302 Chronic diarrhea 304 Consti pation 306 Faltering growth (failure to thrive) 308 Recurrent abdominal pain 310 Gastrointe stinal hemorrhage 312 Jaundice 314 Adverse reactions to food 316 Nutritional disorders 320 Nutritional support 322 Parenteral nutrition 324 Oesophageal disorders 326 Pancreatitis 328 Intestinal disorders 330 In \202 ammatory bowel disease 332 Malabsorption 334 Coeliac dis ease 336 00_McClure_Prelims.indd xviii 11/14/2012 11:16:24 PM DETAILED CONTENTS xix Gastrointestinal infections 338 Intestinal parasites 340 Acute hepatitis 342 Chronic liver failure 344 Alpha1-antitrypsin de \201 ciency 346 Wilson \200\231s disease 346 Liver trans plantation 347 11 Nephrology 349 Polyuria and frequency 350 Abdominal/renal mass 351 Haematuria 352 Proteinuria 354 Urinary tract infection 356 Vesicoureteric re \202 ux 360 Acute kidney injury 362 Acute kidney inj ury: diagnosis and treatment 364 Chronic kidney disease 366 Chronic kidney disease: treatme nt 368 Congenital urinary tract anomalies 370 Inherited renal disease 372 Glomerulonephriti s 374 Haemolytic \200\223uraemic syndrome 376 Nephrotic syndrome 378 Nephrotic syndrome: co mplications and follow-up 380 Renal tubular disorders 382 Proximal renal tubular acidosis 3 84 Bartter \200\231s syndrome 386 Renal calculi 388 Hypertension: de \201 nition 390 Hyper tension: causes and features 394 Hypertension: management 396 12 Endocrinology and diabetes 399 Obesity 400 Obesity: management 404 Type 1 diabetes mellitus 406 Type 1 diabetes mellitus: management 408 00_McClure_Prelims.indd xix 11/14/2012 11:16:24 PM xx DETAILED CONTENTS Type 1 diabetes mellitus: insulin therapy 410 Acute complications of Type 1 diabetes mellit us 412 Type 1 diabetes mellitus: long-term complications 414 Type 1 diabetes mellitus: asso ciated illnesses 415 Type 2 diabetes mellitus 416 Other forms of diabetes mellitus 418 Goit er 420 Solitary thyroid nodule 421 Thyroid carcinoma 421 Medullary thyroid cancer 421 Conge nital hypothyroidism 422 Acquired hypothyroidism 424 Hyperthyroidism (thyrotoxicosis) 425 G raves \200\231s disease 426 Thyroiditis 428 Adrenal insuf \201 ciency 430 Adrenal insuf \201 ciency: treatment 432 Adrenal excess 434 Congenital adrenal hyperplasia 436 Mineraloco rticoid excess 438 Mineralocorticoid de \201 ciency 439 Inherited endocrine syndromes 440 Hypocalcaemia 442 Rickets 444 Hypercalcaemia 446 Posterior pituitary: syndrome of inappropr iate antidiuretic hormone secretion 448 Hypopituitarism 449 Posterior pituitary: diabetes insipidus 450 Polycystic ovarian syndrome 452 13 Growth and puberty 455 Normal growth 456 Normal puberty 458 Assessment of growth 460 Assessment of puberty 462 Sho rt stature 466 Constitutional delay in growth and puberty 468 Intrauterine growth retardati on 468 Turner \200\231s syndrome 469 00_McClure_Prelims.indd xx 11/14/2012 11:16:24 PM DETAILED CONTENTS xxi Coeliac disease 469 Chronic in \202 ammatory disorders 469 Skeletal dysplasias 469 Growth hormone de \201 ciency 470 Growth hormone de \201 ciency: management 472 Tall stature 474 Delayed puberty: assessment 476 Delayed puberty: management 478 Precocious puberty 480 Pre cocious puberty: management 482 Variants of normal puberty 484 Disorders of sex development 486 Disorders of sex development: management 488 Androgen insensitivity syndrome 490 Micro penis 491 Gynaecomastia 492 14 Neurology 495 Examination 496 Examination: children aged <5 years who can \200\231t walk 498 Congenital a bnormalities 500 Paroxysmal episodes: not epilepsy 502 Paroxysmal episodes: general managem ent 504 Seizures and childhood epilepsies 505 Seizures: management 506 Epilepsies: neonatal 508 Epilepsies: infantile 510 Epilepsies: mid to late childhood (1) 512 Epilepsies: mid to late childhood (2) 514 Headache 516 Headache: migraine 518 Bell \200\231s palsy 519 Acute disseminated encephalomyelitis 520 Stroke in childhood 522 Acute abnormal movements 524 Sub dural hemorrhage in a child under 2 years 526 Neurocutaneous disorders 530 Macrocephaly and microcephaly 534 Degenerative disorders 536 Neuromuscular disorders 538 00_McClure_Prelims.indd xxi 11/14/2012 11:16:24 PM xxii DETAILED CONTENTS Peripheral neuropathies 541 Guillain \200\223Barr syndrome 542 Neuromuscular junction 544 Muscular disorders 546 Management of neuromuscular junction and muscular disorders 548 Cerebral palsy 550 Acute encephalopathy 552 15 Child development 555 Managing and living with disability 556 Normal development 557 Gross motor development 558 Fine motor development 559 Speech and language development 560 Social, emotional, and behav ioral development 561 Developmental assessment 562 Neurodevelopmental delay 564 Learning di f \201 culties/disabilities 566 Developmental co-ordination disorder 566 Communication dif \201 culties 566 16 Child and family psychiatry 567 Prevalence 568 Classi \201 cation, categories, and dimensions 569 Comorbidity and causatio n 570 Developmental perspective 571 Systemic thinking 572 Assessment 573 History taking 574 Communicating 575 Asking the dif \201 cult questions 576 Depression 578 Suicide and non-f atal deliberate self-harm 580 Bipolar disorder 582 Anxiety disorders 584 Post-traumatic str ess disorder 586 Obsessive compulsive disorder 588 Attachment disorder 589 00_McClure_Prelims.indd xxii 11/14/2012 11:16:25 PM DETAILED CONTENTS xxiii Schizophrenia 590 Somatoform disorders and typical consultation \200\223liaison presentations 592 Anorexia nervosa 594 Bulimia nervosa 596 Oppositional de \201 ant and co nduct disorders 598 Attention de \201 cit hyperactivity disorder 600 Autism spectrum disor ders 602 Individual psychotherapy 604 Family therapy 605 Psychopharmacotherapy 606 17 Hematology 607 Peripheral blood \201 lm 608 Anemia 610 Haemolytic anaemias 612 De \201 ciency anaemias 614 Red blood count membrane defect anaemias 616 Red blood count enzyme defect anaemias 618 Sickle cell disease 620 Thalassaemia 622 Immune haemolytic anemia 624 Red blood cell fragm entation 625 Aplastic anemia 626 Failure of red cell production (pure red cell aplasia) 628 Polycythaemia 630 Abnormal bleeding or bruising 632 Coagulation studies 634 Di sseminated intravascular coagulation 635 Hemophilia A 636 Hemophilia B 637 von Willebrand d isease 638 Platelet function disorders 640 Thrombocytosis 641 Thrombocytopenia 642 Acute im mune thrombocytopenia 643 Thrombophilia 644 Blood transfusion 646 Blood transfusion reactio ns 648 00_McClure_Prelims.indd xxiii 11/14/2012 11:16:25 PM xxiv DETAILED CONTENTS 18 Oncology 651 Epidemiology of childhood cancer 652 Clinical assessment: history 653 Clinical examination 654 Key investigations 655 Acute lymphoblastic leukemia 656 Acute myeloid leukemia 658 Chro nic myeloid leukemia (adult type) 659 Juvenile myelomonocytic leukemia 659 Lymphoma 660 Cen tral nervous system tumors (1) 662 Central nervous system tumors (2) 664 Neuroblastoma 666 Wilms \200\231 tumor (nephroblastoma) 668 Other renal tumors in childhood 669 Bone tumors 6 70 Rhabdomyosarcoma 672 Germ cell tumors 673 Primary liver tumors 674 Other rare tumors 675 Langerhans cell histiocytosis 676 Haemophagocytic lymphohistiocytosis 677 Chemotherapy 678 Stem cell transplant 680 Radiotherapy 681 Surgery 681 Acute care 682 Acute care: biochemis try 684 Acute care: other 685 Urgent care 687 Principles of follow-up 688 Palliative care 6 90 19 Infectious diseases 693 Introduction 694 The child with fever 696 Fever: examination and assessment 698 Fever: mana gement (green features) 700 Fever: management (red or amber features) 701 Prolonged fever o f unknown cause 702 00_McClure_Prelims.indd xxiv 11/14/2012 11:16:25 PM DETAILED CONTENTS xxv Common infections characterized by rash 704 Exanthem 1: measles 706 Exanthem 2: group A str eptococcus 707 Exanthem 3: rubella 708 Exanthem 4: enteroviruses 708 Exanthem 5: parvovirus 709 Exanthem 6: human herpes virus 6 709 Rash: chickenpox and zoster 710 Rash: infectious mononucleosis 711 Lyme disease 712 Mumps 713 Bacteraemia and shock 714 Kawasaki disease 716 Skin and soft tissues 718 Meningitis 720 Mycobacteria 722 Tropical infections 723 Immunode \201 ciency disorders 724 Human immunode \201 ciency virus 726 Immunizations 728 20 Bones and joints 731 Clinical assessment 732 The limping child 734 The limping child: differential diagnosis 736 Infections: septic arthritis 738 Infections: osteomyelitis 740 Spinal disorders 742 Spine: kyphosis 744 Spine: scoliosis 746 Hip disorders: developmental dysplasia of the hip 748 Hi p disorders: Perthes \200\231 disease 750 Hip disorders: slipped upper femoral epiphysis 75 2 Knee disorders 754 Orthopedic trauma 758 Osteochondroses 760 Osteogenesis imperfecta 762 Osteopetrosis 764 Cleidocranial dysplasia 765 Skeletal dysplasias 766 00_McClure_Prelims.indd xxv 11/14/2012 11:16:25 PM xxvi DETAILED CONTENTS Juvenile idiopathic arthritis 768 Juvenile idiopathic arthritis: clinical principles and management 770 Systemic arthritis 772 Oligoarticular juvenile idiopathic arthritis 773 Rheu matoid factor-positive polyarthritis 774 Rheumatoid factor-negative polyarthritis 776 Psori atic arthritis 777 Enthesitis-related arthritis 778 Systemic lupus erythematosus 780 Juveni le dermatomyositis 782 Mixed connective tissue disease \200\224overlap syndromes 784 Sclero derma 786 Henoch \200\223Sch nlein purpura 788 Polyarteritis nodosa 789 Wegener \200\231s granulomatosis 790 Takayasu \200\231s arteritis (pulseless disease) 791 21 Adolescent health 793 Communication 794 Adolescence: overview 796 Adolescent health problems 798 Substance misuse 800 Sexual health problems 801 Adolescence and chronic illness 802 22 Dermatology 805 Assessment of a rash 806 Atopic eczema/dermatitis 808 Red scaly rashes 810 Papular rashes ( 1) 812 Papular rashes (2) 814 Vesiculobullous rashes 816 Red blanching (erythematous) rashe s 818 Pruritus 820 Pustular rashes 822 Purpuric rashes 823 Lymphoedema 823 Blood vessel dis orders 824 00_McClure_Prelims.indd xxvi 11/14/2012 11:16:25 PM DETAILED CONTENTS xxvii Skin infection: viral and bacterial 826 Fungal skin infections 828 Parasitic skin infection s 829 Protozoal skin infections 829 Hair disorders 830 Nail disorders 832 Photosensitivity and light eruptions 834 Pigmentation disorders 836 Collagen and elastin disorders 838 Conne ctive tissue disorders 839 Miscellaneous skin conditions 840 23 Pediatric surgery 843 Symptoms and signs that should cause concern 844 Congenital abnormalities: upper airway 846 Congenital abnormalities: tracheo-oesophageal 848 Congenital abnormalities: esophagus 850 Congenital abnormalities: lung 851 Congenital abnormalities: chest 852 Idiopathic hypertrop hic pyloric stenosis 854 Ingested foreign bodies 856 Bezoars 857 Midgut malrotation and vol vulus 858 Intussusception 860 Duodenal atresia 862 Small bowel atresias 862 Meconium ileus 862 Acute appendicitis 864 Mesenteric adenitis 865 Meckel \200\231s diverticulum 865 Gastro schisis 866 Exomphalos (omphalocele) 866 Inguinal hernias 868 Hydroceles 868 Hirschsprung \200\231s disease 870 Rectal prolapse 871 Anorectal malformations 872 Umbilical anomalies 8 74 Testicular torsion 876 Orchitis and epididymitis 877 00_McClure_Prelims.indd xxvii 11/14/2012 11:16:25 PM xxviii DETAILED CONTENTS Testicular trauma 877 Undescended testes (cryptorchidism) 878 Retractile testes 879 Hypospa dias 879 Phimosis and paraphimosis 880 Balanitis/balanoposthitis 880 Priapism 880 Penile tr auma 881 Imperforate hymen 881 Labial adhesions in infants 881 Miscellaneous conditions 882 Perioperative care 884 Consent for surgery 886 Post-operative care: \202 uids 888 Post-o perative care: analgesia 890 Post-operative care: drains and wounds 891 24 Special senses 893 Common presentations 894 Hearing assessment 896 Childhood deafness 898 Disorders of the ear 900 Common disorders of the nose 902 Disorders of mouth and tongue 904 Visual development and examination 905 Vision assessment 906 Vision screening in the UK 908 Squints (strabismu s) 910 Ametropia (refractive disorders) 911 Amblyopia 911 Visual impairment 912 Nystagmus 9 13 Disorders of the eye: infection and in \202 ammatory 914 Eye foreign body 916 Cataract 916 Glaucoma 917 Orbit and eyelids 918 Back of the eye problems 920 Trauma 922 00_McClure_Prelims.indd xxviii 11/14/2012 11:16:25 PM DETAILED CONTENTS CONTENTS xxix 25 Genetics 925 Useful resources 926 Clinical genetics and genetic counseling 927 When to refer to clinical genetics 928 Taking a family history 930 Genetic testing 932 Chromosome tests 933 Molecula r genetic analysis 934 Practical issues relating to genetic testing 935 Down syndrome 936 C ommon chromosomal disorders 938 Genetic disorders with cardiac features 940 Genetic testing in cognitive impairment 942 Genetic disorders with neuromuscular features 944 Genetic diso rders with dermatological features 946 Genetic disorders of growth 948 Miscellaneous geneti c conditions 950 Miscellaneous congenital malformations 951 26 Inherited metabolic disease 953 General principles 954 Metabolic syndromes: neurological 956 Metabolic syndromes: metabolic acidosis 958 Metabolic syndromes: storage dysmorphism 959 Metabolic syndromes: hepatic syn dromes 960 Metabolic syndromes: cardiac syndromes 962 Urea cycle disorders 964 Disorders of amino acid metabolism 965 Disorders of organic acid metabolism 966 Disorders of carbohydra te metabolism 967 Disorders of lipoprotein metabolism 967 Lysosomal storage diseases 968 Di sorders of fatty acid oxidation 970 Mitochondrial disorders 971 Peroxisomal disorders 972 D isorders of nucleotide metabolism 973 Disorders of porphyrin metabolism 974 Disorders of me tal metabolism and transportation 976 00_McClure_Prelims.indd xxix 11/14/2012 11:16:25 PM xxx DETAILED CONTENTS CONTENTS 27 Community child health 979 Introduction 980 Voluntary and charitable organizations 980 Organizations and structures 98 1 Health surveillance and promotion 982 Special educational needs 986 Children with disabil ities 987 Speci \201 c learning dif \201 culties 988 Chronic fatigue syndrome 990 Absence from school 991 Constipation and soiling 992 Enuresis 994 28 Child protection 997 De \201 nitions 998 Illness fabricated or induced by carers 999 Physical abuse 1000 Sexual abuse 1002 Emotional abuse 1003 Medical involvement in child protection 1004 Referrals to other agencies 1005 Medical assessment 1006 Assessment by social care 1008 Prevention strat egies 1010 Further reading 1010 29 Pharmacology and therapeutics 1011 Prescribing for children 1012 Adverse drug reactions 1014 Pharmacokinetics 1016 Drug metabo lism 1017 Pain management 1018 Sedation 1019 Fever 1019 30 International health and travel 1021 Child survival: world health 1022 World health: childhood illness 1023 00_McClure_Prelims.indd xxx 11/14/2012 11:16:25 PM DETAILED CONTENTS xxxi Taking children on holiday 1024 Illness after international travel 1026 Further reading 102 8 31 Pediatrics, ethics, and the law 1029 Ethics 1030 Common law 1031 Recognition of ethical issues in everyday clinical practice 103 2 Clinical case study 1033 The doctor \200\223child relationship 1034 Parental responsibili ty 1035 The doctor \200\223parent relationship 1035 Assent and consent 1036 Con \201 denti ality and disclosure 1038 Withholding or withdrawing treatment in children 1040 Good ethica l and legal practice in suspected child abuse 1042 Serious case reviews 1043 Legal aspects of international adoption 1044 00_McClure_Prelims.indd xxxi 11/14/2012 11:16:25 PM xxxii Symbols and abbreviations X i d n l p s 4 5 +/ \200\223 +ve \200\223ve AABR ABC ABCD ABPM ACE ACh ACL ACTH AD ADEM A DH ADHD ADP ADPKD ADR A&E AFP AG AIDS AIS AKI ALA ALCL ALD controversial topic increased decreased normal leading to primary secondary male female wit h or without positive negative Automatic auditory brainstem response airway, breathing, cir culation airway, breathing, circulation, disability ambulatory blood pressure monitoring an giotensin converting enzyme acetylcholine anterior cruciate ligament adrenocorticotrophin a utosomal dominant acute disseminated encephalomyelitis antidiuretic hormone attention de \201 cit hyperactivity disorder adenosine 5-diphosphate Autosomal dominant polycystic kidne y disease adverse drug reaction accident and emergency (department) alpha-foetoprotein anio n gap acquired immune de \201 ciency syndrome androgen insensitivity syndrome acute kidney injury aminolevulinic acid anaplastic large cell lymphoma adrenoleucodystrophy 00_McClure_Prelims.indd xxxii 11/14/2012 11:16:25 PM SYMBOLS AND ABBREVIATIONS xxxiii ALL ALP ALS ALT AML AN ANA ANC ANCA ANLL AP APC APD APH APRT APTT AR ARA ARDS ARF ARM ARPKD ARR AS ASA ASCA ASD ASIS ASOT AST AT ATP AV AVP AVSD AXR AZA BA BAL acute lymphoblastic leukemia alkaline phosphatase advanced life support alanine transferase acute myeloid leukemia anorexia nervosa anti-nuclear antigen antenatal care anti-neutrophi l cytoplasmic antibodies acute non-lymphoblastic leukemia antero-posterior activated protei n C automated peritoneal dialysis antepartum hemorrhage adenine phosphoribosyltransferase a ctivated partial thromboplastin time autosomal recessive acute rheumatoid arthritis acute r espiratory distress syndrome acute renal failure arti \201 cial rupture of membranes autos omal recessive polycystic kidney disease absolute risk reduction Angelman syndrome 5-aminos alicylic acid anti-Saccharomyces cerevisiae antibodies atrial septal defect anterior superi or iliac spine anti-streptolysin O titre aspartate transaminase ataxia telangiectasia adeno sine triphosphate arteriovenous arginine vasopressin atrioventricular septal defect abdomin al X-ray azathioprine bone age British anti-Lewisite 00_McClure_Prelims.indd xxxiii 11/14/2012 11:16:25 PM