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Examination Paediatrics 4e by Wayne Harris
 

Examination Paediatrics 4e by Wayne Harris

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Examination Paediatrics, is designed to assist candidates to pass clinical examinations in paediatrics, particularly at post-graduate level....

Examination Paediatrics, is designed to assist candidates to pass clinical examinations in paediatrics, particularly at post-graduate level.

This book is written for candidates preparing for the Fellowship Examination of the Royal Australasian College of Physicians (Australian & New Zealand candidates and candidates taking the Australian Examination in other countries), and candidates preparing for the Membership Examination for the Royal College of Paediatrics and Child Health (UK) (Part II) (MRCPCH). All undergraduates and postgraduates preparing for any paediatric examination with a clinical component, including those with an OSCE format, should, however, find this book useful as a study aid and reference. The expanded text and new sections will also be useful to GPs, doctors sitting the AMC examination, paediatric residents/house officers, and registrars.

The book contains much useful information including history-taking, examination procedure, relevant investigations, aides mémoire, lists and mnemonics, and management for the majority of chronic paediatric clinical problems seen in hospital-based practice. This edition retains a popular feature of this book – the detailed explanation of the attitudinal skills, body language, and motivation necessary to complete clinical examinations successfully.

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    Examination Paediatrics 4e by Wayne Harris Examination Paediatrics 4e by Wayne Harris Document Transcript

    • ExaminationPaediatrics 4th edition
    • Examination Paediatrics 4th edition Wayne Harris MBBS, MRCP (UK), FRACP Senior Staff Specialist in Paediatrics, West Moreton South Burnett Health Service District Senior Lecturer, Department of Paediatrics and Child Health, University of Queensland, BrisbaneSydney Edinburgh London New York Philadelphia St Louis Toronto
    • Churchill Livingstone is an imprint of Elsevier Elsevier Australia. ACN 001 002 357 (a division of Reed International Books Australia Pty Ltd) Tower 1, 475 Victoria Avenue, Chatswood, NSW 2067This edition © 2011 Elsevier AustraliaThis publication is copyright. Except as expressly provided in the Copyright Act 1968and the Copyright Amendment (Digital Agenda) Act 2000, no part of this publicationmay be reproduced, stored in any retrieval system or transmitted by any means (includingelectronic, mechanical, microcopying, photocopying, recording or otherwise) without priorwritten permission from the publisher.Every attempt has been made to trace and acknowledge copyright, but in some cases thismay not have been possible. The publisher apologises for any accidental infringementand would welcome any information to redress the situation.This publication has been carefully reviewed and checked to ensure that the content is asaccurate and current as possible at time of publication. We would recommend, however, thatthe reader verify any procedures, treatments, drug dosages or legal content described in thisbook. Neither the author, the contributors, nor the publisher assume any liability for injuryand/or damage to persons or property arising from any error in or omission from this publication.National Library of Australia Cataloguing-in-Publication Data___________________________________________________________________Author: Harris, WayneTitle: Examination paediatrics / Wayne Harris.Edition: 4th ed.ISBN: 9780729539401 (pbk.)Notes: Includes index.Subjects: Pediatrics. Pediatrics--Examinations, questions, etc.Dewey Number: 618.92___________________________________________________________________Publisher: Sophie KalinieckiDevelopmental Editor: Neli BryantPublishing Services Manager: Helena KlijnProject Coordinators: Geraldine Minto & Mohanambal NatarajanEdited by Geoffrey PalmerProofread by Ian RossIndex by Annette MuskerCover design by Stan Lamond from Lamond Art & DesignInternal design adapted by Stan Lamond from Lamond Art & DesignTypeset by TNQ Books and Journals Pvt LtdPrinted by China Translation & Printing Services Ltd
    • DedicationTo the late Steve Irwin, the ‘CrocodileHunter’(22.2.1962–4.9.2006),The original Wildlife Warrior,a remarkable larger-than-life father,husband and superheroSteve Irwin was world famous for his remarkably enthusiastic efforts to educate ourplanet about the importance of wildlife and conservation. His honours included:1997, discovery of new species of turtle, Elseya irwini; 2000, Honorary SeniorFellowship (University of the Sunshine Coast); 2001, Centenary Medal (for globalconservation); 2002, Queensland Museum Medal; 2003, Queenslander of the Year;and—posthumously—2007, baby gorilla named after Steve (Rwandan government);2007, Adjunct Professorship (University of Queensland School of Integrative Biology);2009, Australian Land Snail named Crikey steveirwini. I will always remember Steveas my friend. I was privileged to get to know him, initially as a fellow parent, whereour daughters attended the same preschool and became best friends. I  was alwaysimpressed with Steve: his unparalleled devotion to his family; giving freely of histime to launch a fund-raising charity month supporting the local children’s ward,appearing on local radio and giving free entry to over 150 chronic paediatric patientsfrom my hospital to his Australia Zoo; and opening the magnificent wildlife hospital.I respected his intelligence, his passion for his cause and his genuine compassion forsick children, but most of all I was touched by what a fantastic father and husband hewas. His enthusiasm touched the lives of, and inspired, everyone he met. He is greatlymissed. v
    • ContentsDedication v Obesity 157Foreword ix Normal puberty 163Preface xi Precocious puberty 164Preface to the first edition xiii Delayed puberty 169Acknowledgements xv Disorders of Sexual Develop-Abbreviation xvii ment (virilisation in the post-Introduction xxxiii neonatal period) 177 Thyroid disorders 1821 Approach to the examination 1 Thyroid disorders in infants 1862 The long case 73 The short case 14 8 Gastroenterology 1904 Achievement psychology 17 Long Cases5 Behavioural and developmental Inflammatory bowel disease 190 paediatrics 25 Chronic liver disease (CLD) 200 Long Cases Malabsorption/maldigestion 212 Anorexia nervosa 25 Short Cases Attention deficit hyperactivity Gastrointestinal system 217 disorder (ADHD) 35 The abdomen 222 Autistic disorder (autism) 49 Jaundice 229 Short Cases Nutritional assessment 236 Child suspected of having Failure to thrive 244 ADHD 61 Poor feeding 246 Child with possible autism/autistic Weight loss—older child/ spectrum disorder (ASD) 64 adolescent 2466 Cardiology 67 9 Genetics and dysmorphology 253 Long Case Long Cases Cardiac disease 67 Down syndrome 253 Short Case Turner syndrome 265 The cardiovascular system 91 Short Case The dysmorphic child 2777 Endocrinology 107 Long Cases 10 Haematology 287 Congenital adrenal Long Cases hyperplasia 107 Haemophilia 287 Diabetes mellitus 116 Sickle cell disease (SCD) 297 Short Cases Thalassaemia: β-thalassaemia Disorders of Sexual Development major 311 (ambiguous genitalia) 132 Short Cases Diabetes 135 The haematological system 321 Short stature 136 Thalassaemia 329 Tall stature 150 vii
    • viii Contents11 Neonatology 332 Neuromuscular assessment 479 Short Cases Scoliosis 480 The neonatal examination 332 Spina bifida 482 The 6-week check 337 14 Oncology 48712 Nephrology 338 Long Case Long Cases Oncology 487 Chronic kidney disease Short Case (CKD) 338 Late effects of oncology Nephrotic syndrome 356 treatment 498 Short Cases Renal examination 365 15 The respiratory system 503 Hypertension 369 Long Cases Oedema 374 Asthma 503 Neonatal intensive care unit13 Neurology 377 graduate: chronic lung disease/ Long Cases bronchopulmonary dysplasia Cerebral palsy 377 (CLD/BPD) 513 Dystrophinopathies: Duchenne Cystic fibrosis (CF) 521 muscular dystrophy Obstructive sleep apnoea (DMD) 391 (OSA) 546 Seizures and epileptic Short Cases syndromes 403 The respiratory system 552 Spina bifida 420 The chest 556 Short Cases Stridor 556 Developmental assessment 430 Chest X-rays 558 Eye examination 432 Motor cranial nerves 438 16 Rheumatology 561 Neurological assessment of the Long Cases upper limbs 439 Juvenile idiopathic arthritis Gait: a short-case approach 443 (JIA) 561 Neurological assessment of the Juvenile idiopathic inflammatory lower limbs 444 myopathies (JIIMs): juvenile Cerebellar function 447 dermatomyositis (JDM) 577 Large head 447 Systemic lupus erythematosus Small head 450 (SLE) 587 Seizures 459 Short Case Facial weakness 462 Joints 598 Floppy infant 464 Hemiplegia 469 Suggested reading 604 Intellectual impairment 472 Quick Reference Mnemonics 607 Involuntary movements 475 Picture Credits 623 Neurofibromatosis, type 1 Index 625 (NF-1) 477
    • ForewordThe fourth edition of Examination Paediatrics by Dr Wayne Harris updates this widelyused text to assist paediatric trainees undertaking the current clinical examinationsas part of their vocational training and qualifications. Examination candidates inAustralia, New Zealand, South-East Asia, the United Kingdom and North Americahave benefited from the previous editions over the last 18 years. Next to extensiveexperience and practice in assessing children in clinical situations, this book equipscandidates with a systematic approach to both the long- and short-case elements usedin examinations. The first four chapters outline the general approach to examination procedures,including interaction with examiners and practical preparation for the differingobjectives of short- and long-case examinations. The chapter on achievement psy-chology is of particular relevance to those who find examination settings and experi-ence extremely difficult. Subsequent chapters highlight each of the different areas of paediatrics by sub-speciality organ system. As in previous editions, there is extensive use of mnemonicsto assist candidates with recalling the significant issues in examining patients ordiscussing conditions. Although the book is primarily intended for paediatric trainees, it will also beof use for others undertaking clinical examination of children, including medicalstudents or general practice trainees. The comprehensive coverage of conditions givenin the text assists examination candidates to review the scope of their experience andknowledge, and identify areas where more experience or practice is needed. Dr Harris (and his two co-authors of the previous first and second editions ofExamination Paediatrics) has an extensive involvement in general paediatrics andexamination experience, on which this book is based. In this new edition, he hasprovided updates in important areas where understanding of causation of diseaseand/or treatment have changed in the last 4 years. This latest edition will prove to be an invaluable asset to those preparing for andundertaking paediatric clinic examinations, especially those in paediatric generalistor specialist vocational training. Professor Allan Carmichael, OAM, MBBS (Monash), M.D. (Melb), FRACP Professor of Paediatrics and Child Health Dean, Faculty of Health Science University of Tasmania ix
    • Preface‘Geez, mate, that’s pretty good. You could be a vet!’ Steve Irwin, giving the author the greatest compliment a paediatrician can receive, for cannulating his child.The first edition of this book was written in 1992 to assist candidates preparing forthe Fellowship of the Royal Australasian College of Physicians (Part 1) Examinationin Paediatrics, or for the Membership of the Royal College of Physicians (Part 2)Examination in Paediatrics, inspired by Talley and O’Connor’s book ExaminationMedicine. The second edition came out in 2002, and the third in 2006. Much to myamazement, it has continued to sell well enough to justify ongoing editions. Morechanges are needed with each edition, as the progress of medical knowledge has beenstaggering. I shall try to continue in Talley and O’Connor’s adult-sized footsteps,and hope that this edition is yet more useful than the previous one. I believe thereis much of relevance to senior medical students and general practitioners, as wellas to paediatric trainees. All previous long cases have been extensively updated andrewritten, and a new one (obstructive sleep apnoea) added; previous short cases havebeen updated, and a new one (virilisation) added; there are new expanded sectionsof previous chapters, new mnemonics and broader background information, and anew section covering William’s syndrome. As noted in previous editions, this is notthe ‘Einstein Encyclopaedia of Paediatrics’; its purpose is to make the hurdle of anypaediatric examination easier to clear. Don’t panic! Good luck! Wayne Harris July 2010 xi
    • Preface to the first edition‘Don’t panic.’ The Hitchhiker’s Guide to the Galaxy, Douglas AdamsThis book has been written to assist candidates preparing for the Fellowship of theRoyal Australasian College of Physicians (Part 1) Examination in Paediatrics. It isalso intended to assist candidates preparing for the Membership of the Royal Collegeof Physicians (Part 2) Examination in Paediatrics. It was inspired by Talley andO’Connor’s book Examination Medicine, written to assist in the preparation for theRoyal Australasian College of Physicians’ Internal Medicine (Part I) Examination. We have tried to present a structured and comprehensive approach to the clinicalexamination of the paediatric patient in a way that is particularly relevant for thepostgraduate degrees of the FRACP (Part 1) and the MRCP (Part 2). Approaches arepresented for most of the common-examination long and short cases. This book is not designed to be the ‘Einstein Encyclopaedia of Paediatrics’. As asupplement to the major texts and journal articles, it aims to demonstrate approachesthat the authors have found successful. Our combined experience of the FRACP examination comprises six writtenexaminations, and six clinical examinations (including two ‘extra time’ cases), andfor the MRCP, one clinical examination. This broad coverage of most conceivablecontingencies ensures that many of the approaches have been tested (successfully) inthe actual examination settings by the authors, while others have been tested by ourpeers in their examinations. We hope that this book will be useful for examination candidates, and alsohelpful to paediatric residents/house officers, senior medical students and generalpractitioners who deal with children. While every effort has been made to ensure the accuracy of the information herein,especially with regard to drug selection and dosage, appropriate information sourcesshould be consulted, particularly for new or uncommon drugs. It is the clinician’sresponsibility to check the appropriateness of an opinion in relation to acute clinicalsituations and new developments. Any comments or suggestions will be gratefullyand humbly received, so that future editions of this book may prove to be more useful. Good luck! Wayne Harris Brian Timms Robin Choong 1992 xiii
    • AcknowledgementsSpecial thanks…To my family for supporting me throughout the months spent producing this edition. To my friends and colleagues, Dr Brian Timms and Dr Robin Choong, two of myfavourite people, for supporting me as co-authors over the first and second editionsof this book, and for encouraging me to complete the third edition, and this edition. To the following wonderful people, for your unwavering support, andencouragement: Dr John Arranga, Dr Benjamin Cheung, Dr Lisa Gotley, Mrs TerriIrwin, Dr Herminia Narvaez, Dr Joseph Pasion, Mr Rob Penfold, Dr Alberto Pinzon,Dr Sonia Reichert, Ms Junko Tanaka and, Mr Charles Waterstreet.ReviewersI would like to thank the following specialists who were kind enough to reviewdifferent sections of the book. Their comments were invaluable. However, the bookdoes not necessarily reflect the opinions of these specialists.Navid Adib, FRACP, Paediatric Rheumatologist, The Royal Children’s Hospital, Brisbane.Chris Barnes, FRACP, Paediatric Haematologist, The Royal Children’s Hospital, Parkville.Phillip Britton, MBBS, DCH, Advanced Trainee in Paediatrics, Deputy Chief Resident Medical Officer, The Children’s Hospital at Westmead, New South Wales (The Royal Alexandria Hospital for Children), General Paediatrics.Anita Cairns, FRACP, Paediatric Neurologist, The Royal Children’s Hospital, Brisbane.Sophie Calvert, FRACP, Paediatric Neurologist, Mater Children’s Hospital, Brisbane.A Carmichael, MD, FRACP, Dean, Faculty of Health Science, University of Tasmania, Hobart.Anne Chang, FRACP, Paediatric Respiratory Consultant, The Royal Children’s Hospital, Brisbane.R Choong, FRACP, AIMM, Senior Staff Specialist, Paediatric Intensive Care and Paediatric Emergency Medicine, The Children’s Hospital at Westmead, New South Wales.Andrew Cotterill, FRACP, Director, Department of Endocrinology, Mater Children’s Hospital, BrisbaneLuciano Dalla Pozza, FRACP, Paediatric Oncologist, The Children’s Hospital at Westmead, New South Wales.Alexa Dierig, Clinical Research Fellow NCIRS (National Centre for Immunisation, Research and Surveillance), The Children’s Hospital at Westmead, New South WalesElisabeth Hodson, FRACP, Paediatric Nephrologist, The Children’s Hospital at Westmead, New South Wales.Rick Jarman, FRACP, Consultant Paediatrician, The Royal Children’s Hospital, Parkville. xv
    • xvi AcknowledgementsMaina Kava, MBBS, MD, DCH, DNB, FCPS, Paediatric Neurology Fellow, Princess Margaret Hospital, Western AustraliaGulam Khandaker, MBBS, MPH, DCH, PhD candidate, The Children’s Hospital at Westmead, New South Wales; Clinical Research Fellow, NCIRS (National Centre for Immunisation, Research and Surveillance)Johnny Kwei, MBBS, Plastic Surgical Advance Trainee, The Children’s Hospital at Westmead, New South WalesI B Masters, FRACP, Director, Department of Respiratory Medicine, The Royal Children’s Hospital, BrisbaneJim McGill, FRACP, Director, Department of Metabolic Medicine, The Royal Children’s Hospital, Brisbane.Martha F Mherekumombe, MBCHB, M Med, DCH, FRACP, Paediatric Trainee, Senior Registrar, The Children’s Hospital at Westmead, New South WalesAhmed Moustafa, MBChB, AMC, DCH, Paediatric Registrar, The Children’s Hospital at Westmead, New South WalesEdward OLoughlin, MD, FRACP, Paediatric Gastroenterologist, The Children’s Hospital at Westmead, New South Wales.Robert Ouvrier, FRACP, Paediatric Neurologist, The Children’s Hospital at Westmead, New South Wales.Marilyn Paull, MBBS, B Med Sci (Hons), Hons in Neurophysiology Advanced Trainee, DCRMO Advanced Paediatric Trainee and Deputy Chief Resident at Children’s Hospital at Westmead, New South Wales.James Pelekanos, FRACP, Paediatric Neurologist, Brisbane; Senior Lecturer, University of Queensland.Susan Sawyer, MD, FRACP, Director, Centre for Adolescent Health, The Royal Children’s Hospital, ParkvilleBrian Timms, FRACP, Consultant Paediatrician, Consultant Neonatologist, Melbourne.Peter van Asperen, FRACP, Paediatric Thoracic Physician, The Children’s Hospital at Westmead, New South Wales.Garry Warne, FRACP, Paediatric Endocrinologist, The Royal Children’s Hospital, Parkville.Chris Whight, FRACP, Paediatric Cardiologist, The Royal Children’s Hospital, Brisbane.
    • Abbreviations1,25(OH)2 D3 calcitriol3β-HSD 3 beta-hydroxysteroid dehydrogenase3D three-dimensional3DE three-dimensional echocardiography5-ASA 5-aminosalicylate6-MP 6-mercaptopurine6-MMP 6-methylmercaptopurine6-TG 6-thioguanine7vPCV heptavalent conjugated pneumococcal vaccine21OHD-CAH congenital adrenal hyperplasia due to 21 hydroxylase deficiency11OHD-CAH congenital adrenal hyperplasia due to 11 beta hydroxylase deficiency17OHD-CAH congenital adrenal hyperplasia due to 17 hydroxylase deficiency17OHP 17 hydroxyprogesteroneAAI atlanto-axial instabilityAAN American Academy of NeurologyAAP American Academy of PediatricsAAVC accessory atrioventricular connectionABC airway, breathing, circulationABPA allergic bronchopulmonary aspergillosisACA anterior cerebral arteryACE angiotensin-converting enzymeAChR acetylcholine receptorACM Arnold-Chiari malformationACR acute cellular rejection and/or American College of RheumatologyACS acute chest syndrome (in sickle cell disease)ACTH adrenocorticotropic hormoneAD autosomal dominantADD attention deficit disordera-DGP anti-deamidated gliadin-related peptideADH antidiuretic hormoneADHD attention deficit hyperactivity disorderADI-R Autism Diagnostic Interview—RevisedADL activities of daily livingADLTLE autosomal-dominant lateral temporal lobe epilepsy (aka ADPEAF)ADNFLE autosomal-dominant nocturnal frontal lobe epilepsyADOS Autism Diagnostic Observation ScheduleADPEAF autosomal-dominant partial epilepsy + auditory features (aka ADLTLE)ADR adriamycinAED anti-epileptic drugAFO ankle-foot orthosisAGC aspartate glutamate carrierAGS Alagille syndromeAHA American Heart AssociationAHI apnoea hypoxic indexAIDs anti-inflammatory drugs xvii
    • xviii Examination paediatricsAIDS acquired immune deficiency syndromeAIR anaesthesia-induced rhabdomyolysisaka also known asALL acute lymphoblastic leukaemiaALP alkaline phosphataseALT alanine aminotransferaseALTE apparent life threatening eventAMH/MIS anti-Müllerian hormone/Müllerian inhibiting substanceAML acute myeloid leukaemiaAN anorexia nervosaANA antinuclear antibodyANC absolute neutrophil countANLL acute non-lymphoblastic leukaemiaAP anteroposteriorAPD automated peritoneal dialysisaPTT activated partial thromboplastin timeAR autosomal recessiveARB angiotensin II receptor blockerAS aortic stenosisASCA anti-Saccharomyces cerevisiae antibodiesASD atrial septal defect and/or autistic spectrum disorderASQ Ages and Stages QuestionnaireAST aspartate aminotransferaseAT alpha-1-antitrypsinATG anti-thymocyte globulinATN acute tubular necrosisATNR asymmetric tonic neck reflexATZ alpha-1-antitrypsin ZAV atrioventricularAVM arteriovenous malformationAVN atrioventricular nodeAVNRT atrioventricular node re-entry tachycardiaAZA azathioprineBAV bicuspid aortic valveB–B Bardet–Biedl syndromeBDP-HFA beclomethasone diproprionateBDZ benzodiazepineBECTS benign epilepsy with centrotemporal spikesBFNS benign familial neonatal seizuresBiPAP bilevel positive airways pressureBMD bone mineral density and/or Becker muscular dystrophyBMI body mass indexBMTx bone marrow transplantationBN bulimia nervosaBPD bronchopulmonary dysplasiaBSL blood sugar level (commonly used term for plasma glucose level)BSS Bernard-Soulier syndromeBTX-A botulinum toxin ABUD budesonideB-W Beckwith-Wiedemann syndromeC1 atlas; first cervical vertebraC2 axis; second cervical vertebra
    • • Abbreviations xixCAB chlorambucilCACN calcium channelCAE childhood absence epilepsyCAH congenital adrenal hyperplasia and/or chronic active hepatitisCAL café-au-laitCAPD continuous ambulatory peritoneal dialysisCAR Central Africa RepublicCARS Childhood Autism Rating ScaleCAS Child Assessment ScheduleCATCH-22 cardiac defects, abnormal facies, thymic hypoplasia [and T cell deficiency], cleft palate, hypoparathyroidism [and hypocalcaemia] [chromosome 22: band 11 of long arm, microdeletions (22q11)]CBAVD congenital bilateral absence of the vas deferensCBCL Child Behaviour ChecklistCB-SCT cord-blood-derived stem cell transplantationCBZ carbamazepineCCF congestive cardiac failureCCP citrullinated cyclic peptideCCPD continuous cycling peritoneal dialysisCCPT Connors continuous performance testCD Crohn’s disease and/or cadaveric donorCDA Child Disability AllowanceCDH congenital dislocation of the hipCDI Child Development InventoriesCDP constitutional delayed puberty (and growth)CF cystic fibrosisCFC cardio-facial-cutaneous syndrome and/or chlorinated fluorocarbonsCFLD cystic fibrosis-associated liver diseaseCFR-BD cystic fibrosis-related bone diseaseCFRD cystic fibrosis-related diabetesCFTR cystic fibrosis transmembrane conductance regulatorCGH comparative genomic hybridization (array same as CMA)CGMS continuous glucose monitoring systemCH congenital hemihypertrophyCHAQ Childhood Health Assessment QuestionnaireCHARGE colobomatous malformation of eye, heart, atresia choanae, retardation (cognitive & somatic), genital anomalies, ear anomalies +/– deafnessCHAT Checklist for Autism in ToddlersCHD congenital heart diseaseCHILD congenital hemidysplasia, ichthyosiform [erythroderma], limb defectsCHQ Child Health QuestionnaireCHR cholinergic receptorCI cochlear implantationCIC ciclesonideCKD chronic kidney diseaseCKD-MBD chronic kidney disease mineral bone diseaseCLCN chloride channelCLD chronic liver disease and/or chronic lung disease
    • xx Examination paediatricsCLZ clonazepamCMA chromosomal microarray analysis (same as CGH)CML chronic myeloid leukaemiaCMP cow’s milk proteinCMT Charcot-Marie-Tooth diseaseCMV cytomegalovirusCNF1 congenital nephotic syndrome of the Finnish typeCNI calcineurin inhibitorCNS central nervous systemCNV copy-number variantCO2 carbon dioxideCOFS cerebro-oculo-facial-skeletal syndromeCOG children’s oncology groupCOX-2 cyclo-oxygenase 2CP cerebral palsyCPA cyclophosphamideCPAP continuous positive airways pressureCPR cardiopulmonary resuscitationCRF chronic renal failureCRH corticotrophin-releasing hormoneCRINS corticosteroid-resistant idiopathic nephrotic syndromeCRP C-reactive proteinCS corticosteroidCSA cyclosporine ACSF cerebrospinal fluidCSINS corticosteroid-sensitive idiopathic nephrotic syndromeCSWS epileptic encephalopathy with continuous spike-and-wave during sleepCT computed tomographyCTAF conotruncal anomaly face syndromeCTE CT enterographyCTS centrotemporal spikesCVA cerebrovascular accidentCVAD central venous access deviceCVS cardiovascular system and/or chorionic villus samplingCXR chest X-rayCYP17A1 cytochrome P450 enzyme 17-hydroxylaseCYP21A2 cytochrome P450 enzyme 21-hydroxylaseDAP dystrophin-associated proteinDAT dopamine transporterdB decibelDBCL Developmental Behaviour ChecklistDCCT Diabetes Control and Complications TrialDCD developmental coordination disorder (clumsiness)DCM dilated cardiomyopathyDDAVP desmopressin (1-deamino 8-D arginine vasopressin)DDH developmental dysplasia of the hipDDST Denver developmental screening testDDST-II Denver developmental screening test—II (revised)DEXA dual X-ray absorptiometryDFO desferrioxamineDFP deferiprone
    • • Abbreviations xxiDFS deferasiroxDFT deferitrinDFZ deflazacortDGGE denaturing gradient gel electrophoresisDGS DiGeorge syndromeDHEA dehydroepiandrosteroneDHEAS dehydroepiandrosterone sulphateDHT dihydrotestosteroneDIC disseminated intravascular coagulationDICA Diagnostic Interview for Children and AdolescentsDIDMOAD diabetes insipidus-diabetes mellitus-optic atrophy-deafness (Wolfram)DIOS distal intestinal obstruction syndromeDISC Diagnostic Interview for ChildrenDISCO Diagnostic Interview for Social and Communications DisordersDISIDA di-iso-propyl-imino-di-acetic acid [disofenin hepatobiliary scan]DKA diabetic ketoacidosisDMARDs disease-modifying antirheumatic drugsDMD Duchenne muscular dystrophyDMSA di-mercapto-succinic-acidDNA deoxyribonucleic acidDQ developmental quotientDS Down syndromeDSD disorder of sexual developmentdsDNA double-stranded DNADSM-IV Diagnostic and Standard Manual of Mental Disorders, 4th editionDTPA Tc99m diethylene triamine penta-acetic acidD-W Dandy-Walker syndromeEACA epsilon-aminocaproic acidEBV Epstein-Barr virusECA epilepsy childhood absenceECG electrocardiogramECMO extracorporeal membrane oxygenationEDIC Epidemiology of Diabetes Interventions and ComplicationsEEG electroencephalogramEEN exclusive enteral nutritionEFD eformoterol fumarate dihydrateEGTCSA epilepsy with generalised tonic-clonic seizures aloneEHBA extrahepatic biliary atresiaEJA epilepsy juvenile absenceEJM epilepsy juvenile myoclonusELBW extremely low birthweightEMA endomysial antibodyEM-AS epilepsy with myoclonic atonic seizures (Doose syndrome)EME early myoclonic encephalopathyEMG electromyogramENT ears, nose and throatEPR early-phase allergic responseEPS electrophysiologic studyER endoplasmic reticulum and/or extended release
    • xxii Examination paediatricsERA enthesitis-related arthritisERCP endoscopic retrograde cholangiographyERG electroretinogramERPT endorectal pull-through procedureESLD end-stage liver diseaseESM ethosuximide and/or ejection systolic murmurESR erythrocyte sedimentation rateESRD end-stage renal diseaseFA1AT faecal alpha-1-antitrypsin excretion testFAS foetal alcohol syndromeFBC full blood countFDA Food and Drug AdministrationFET forced inspiratory timeFEV1. forced expiratory volume in one secondffDNA cell-free foetal DNAFFP fresh frozen plasmaFHM familial hemiplegic migraineFISH fluorescence in situ hybridisationF-IX factor 9FLAIR fluid attenuation inversion recovery sequencesFLE frontal lobe epilepsyfMMC foetal myelomeningocoelefMRI functional MRIFP fluticasone proprionateFS febrile seizuresFS+ febrile seizures plusFSGS focal segmental glomerulosclerosisFSH facio-scapulo-humeral and/or follicle stimulating hormoneFVC forced vital capacityF-VIIa activated factor 7F-VIII factor 8G6PD glucose-6-phosphate dehydrogenaseGABA gamma-amino butyric acidGABR gamma-amino butyric acid receptorGAD glutamic acid decarboxylaseGADA glutamic acid decarboxylase antibodiesGARS Gilliam Autism Rating ScaleGBM glomerular basement membraneGBS Guillain-Barre syndrome and/or group B streptococcusGCDH glutaryl-CoA dehydrogenaseG-CSF granulocyte colony stimulating factorGEFS+ generalised epilepsy with febrile seizures plusGFR glomerular filtration rateGGT gamma glutamyl transferaseGH growth hormoneGI glycaemic indexGIT gastrointestinal tractGLUT1-DS glucose transporter 1 deficiency syndromeGM-CSF granulocyte-macrophage colony stimulating factorGn gonadotrophinGN glomerulonephritisGnRH gonadotrophin-releasing hormone (aka LHRH)
    • • Abbreviations xxiiiGOR gastro-oesophageal refluxGORD gastro-oesophageal reflux diseaseGSD glycogen storage diseaseGSTM1 glutathione-S-transferaseGTCS generalised tonic clonic seizuresGTT glucose tolerance testGVHD graft versus host diseaseHACAs human anti-chimeric antibodiesHAT hepatic artery thrombosisHb haemoglobinHbA1C haemoglobin A1C: glycosylated haemoglobinHb AS haemoglobin AS (sickle cell trait)HBB haemoglobin subunit beta globin geneHBeAg hepatitis B e antigenHb C haemoglobin CHb F haemoglobin F (foetal)Hb S haemoglobin S(sickle cell)Hb SC haemoglobin SC (sickle cell/haemoglobin C disease)HBsAg hepatitis B surface antigenHb SS homozygous sickle cell diseaseHC head circumferenceHCC hepatocellular carcinomahCG human chorionic gonadotrophinHCM hypertrophic cardiomyopathyHCV hepatitis CHD haemodialysisHDL high-density lipoproteinHEADS Home, Education/Employment/Eating/Exercise, Activities, Drugs & alcohol, Sexuality/Sexual health/Suicide/Self-harm & depression/SafetyHFA hydrofluoroalkaneHFI hereditary fructose intoleranceHGPRT hypoxanthine guanine phosphoribosyl-transferaseHHT hereditary haemorrhagic telangiectasiaHIDA hepatobiliary iminodiacetic acidHIV human immunodeficiency virusHKAFO hip-knee-ankle-foot orthosisHLA human leucocyte antigenHLHS hypoplastic left heart syndromeHNF human nephron filterHPA hypothalamic-pituitary-adrenal (axis)HPG hypothalamic-pituitary-gonadal (axis)HPOA hypertrophic pulmonary osteoarthropathyHPFH hereditary persistence of Hb FHPLC high-performance liquid chromatographyHRCT high-resolution CTHRQOL health-related quality of lifeHS hippocampal sclerosisHSCT haematopoietic stem cell transplantationHSE herpes simplex encephalitisHSP Henoch–Schönlein purpuraHSTCL hepatosplenic T-cell lymphoma
    • xxiv Examination paediatricsHSV herpes simplex virusHUS haemolytic uraemic syndromeHVZ herpes varicella-zosterHx historyHz hertzIA-2 tyrosine phosphataseIAA insulin autoantibodiesIBD inflammatory bowel diseaseIBW ideal body weightIC intracranialICA internal carotid arteryICD implantable cardioverter defibrillatorICD-10 international classification of diseasesICE-GTC intractable childhood epilepsy with generalised tonic-clonic seizuresICH intracranial haemorrhageICP intracranial pressureICS inhaled cortico-steroidsICSI intracytoplasmic sperm injectionICU intensive care unitID intellectual disabilityIDDM insulin-dependent diabetes mellitusIEM inborn error of metabolismIGF insulin-like growth factorIGFBP insulin-like growth factor bonding proteinIg immunoglobulinIHH intermittent hypercarbic hypoxia and/or:IHH idiopathic hypogonadotrophic hypogonadismIIMs idiopathic inflammatory myopathiesILAE International League against epilepsyILAR International League against rheumatismIM intramuscular and/or infectious mononucleosisIMP inosine monophosphateINCS intranasal corticosteroidINR international normalised ratioINS idiopathic nephrotic syndromeIPTAS isolated patients’ travel and accommodation schemeIQ intelligence quotientIRT immunoreactive trypsinITT immune tolerance therapyIU international unitsIUGR intrauterine growth retardationIV intravenousIVH intraventricular haemorrhageIVIG intravenous immunoglobulinIVC inferior vena cavaJA juvenile arthritisJAE juvenile absence epilepsyJBS Johanson–Blizzard syndromeJDM juvenile dermatomyositisJIA juvenile idiopathic arthritisJLNS Jervell and Lange-Nielsen syndrome
    • • Abbreviations xxvJME juvenile myoclonic epilepsy (Janz syndrome)JMML juvenile myelomonocytic leukaemiaJPsA juvenile psoriatic arthritisJVP jugular venous pressureKAFO knee-ankle-foot orthosisKCl potassium chlorideKCN potassium channelKF Kayser-FleischerK–T–W Klippel-Trenauney-Weber syndromeLABA long-acting beta-2 adrenoreceptor agonistLCR locus control regionLDL low density lipoproteinLDLT living-donor lobar transplantationLEOPARD lentigines, ECG, ocular, pulmonary [stenosis], abnormal [genitalia], retarded growth, deafnessLEV levetiracetamLFTs liver function testsLGL Lown-Ganong-Levine syndromeLGS Lennox-Gastaut syndromeLH luteinising hormoneLHRH luteinising hormone-releasing hormone (aka GnRH)LJM limited joint mobilityLKS Landau-Kleffner syndromeLMN lower motor neuroneLP lumbar punctureLPR late-phase allergic responseLQTS long QT syndromeLRD living related donorLS lower segmentLTG lamotrigineLTM leukotriene modifierLTRAs leukotriene receptor antagonistsLTx liver transplantationLV left ventricleLVF left ventricular failureLVH left ventricular hypertrophyLVOTO left ventricular outflow tract obstructionLVS levamisoleMACE Malone antegrade continence enemaMAE epilepsy with myoclonic absencesMAG-3 mercapto-acetyl-triglycine (chelated to technetium-99m)MAHA microangiopathic haemolytic anaemiaMAS macrophage activation syndromeMCA middle cerebral arteryMcC-A McCune-AlbrightM-CHAT Modified Checklist for Autism in ToddlersMD myotonic dystrophyMDA muscular dystrophy associationMDI metered dose inhalerMDLS Miller-Dieker lissencephalopathy syndromeMDS myelodysplastic syndromeMEI myoclonic epilepsy in infancy
    • xxvi Examination paediatricsMEN2b multiple endocrine neoplasia type 2bMEP maximum expiratory mouth pressureMERRF mitochondrial encephalopathy with red ragged fibresMesPGN mesangial proliferative glomerulonephritisMG myasthenia gravisMHC major histocompatibility complexMI meconium ileusMIBG radioactive iodine metaidobenzoguanidineMIP maximum inspiratory mouth pressureMIS/AMH müllerian inhibiting substance/anti-müllerian hormoneMLD metachromatic leukodystrophyMLDS myeloid leukaemia of Down syndromeMLPA multiple ligation probe amplificationMMC myelomeningocoeleMMF mycophenolate mofetilMMR measles-mumps-rubellaMOM mometasoneMPGN membranoproliferative glomerulonephritisMPH methylphenidate and/or mid-parental heightMPS mucopolysaccharidosisMRA magnetic resonance angiographyMRD minimal residual diseaseMRE magnetic resonance enterographyMRI magnetic resonance imagingmRNA messenger RNAMRSA methicillin-resistant staphylococcus aureusMSG monosodium glutamateMSU mid-stream urineMTLE with HS mesial temporal lobe epilepsy with hippocampal sclerosisMTX methotrexateMVA motor vehicle accidentMVP mitral valve prolapsedMW molecular weightMx managementNAC National Asthma CouncilnAChR nicotinic acetyl choline receptorNaCl sodium chlorideNAI non-accidental injuryNDSS National Diabetes Syringe SchemeNEC necrotising enterocolitisNEJM New England Journal of MedicineNF-1 neurofibromatosis type 1NF-2 neurofibromatosis type 2NFNS neurofibromatosis Noonan syndromeNH&MRC National Health and Medical Research CouncilNICU neonatal intensive care unitNIV non-invasive ventilationNPPV non-invasive positive pressure ventilationNREM non-rapid eye movementNS Noonan syndrome and/or nephrotic syndromeNSAID non-steroidal anti-inflammatory drugNTBC 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexenedione
    • • Abbreviations xxviiNTD neural tube defectOB bronchiolitis obliteransOCD obsessive compulsive disorderOCPill oral contraceptive pillOCS oral corticosteroidsODD oppositional defiant disorderOI osteogenesis imperfectaOMIM online Mendelian inheritance in manOSA obstructive sleep apnoeaOT occupational therapistOXC oxcarbazepineP2 pulmonary component of the second heart soundP-A posterior anteriorpaCO2 partial pressure carbon dioxide, arterialPADP program of aids to disabled peoplePAIS partial androgen insensitivitypANCAs perinuclear antineutrophil cytoplasmic antibodiesPAPVD partial anomalous pulmonary venous drainagePAPVR partial anomalous pulmonary venous returnPB phenobarbitonePBAC Pharmaceutical Benefits Advisory CommitteePBID paucity of bile interlobular ductsPBM peak bone massPBS Pharmaceutical Benefits SchemePCDAI paediatric Crohn’s disease activity indexPCF peak cough flowPCM protein calorie malnutritionPCO polycystic ovary syndromePCR polymerase chain reactionPDA patent ductus arteriosusPDD-NOS pervasive developmental disorder not otherwise specifiedPDDST Pervasive Developmental Disorders Screening TestPEDS Parents Evaluation of Developmental StatusPedsQL Pediatric Quality of Life Inventory Generic Core ScalesPEEP positive end expiratory pressurePEF peak expiratory flowPEFR peak expiratory flow ratePEG percutaneous endoscopic gastrostomyPELD pediatric end-stage liver diseasePEP positive expiratory pressurePERT pancreatic enzyme replacement therapyPET pre-emptive transplantation and/or positron emission tomographyPFE pulmonary fat embolismPFIC progressive familial intrahepatic cholestasis disordersPFTs pulmonary function testsPGD preimplantation genetic diagnosisPGE primary generalised epilepsyPGH preimplantation genetic haplotypingPHPT pseudohypoparathyroidismPHT phenytoin and/or pulmonary hypertensionPI protease inhibitor and/or pancreatic insufficiency
    • xxviii Examination paediatricsPIH pyridoxal isonicotinoyl hydraxonePIP peak inspiratory pressurePKU phenylketonuriaPLE protein losing enteropathyPLMD periodic limb movement disorderPMA postmenstrual agepMDI pressurized metered-dose inhalerPME progressive myoclonic epilepsiesPML progressive multifocal leukoencephalopathyPND prenatal diagnosisPNF primary non-functionPPS peripheral pulmonary arterial stenosisPPV23 polysaccharide pneumococcal vaccinePR per rectumPRA plasma rennin activityPS pancreatic sufficiency and/or pulmonary (valve) stenosisPSG polysomnographyPT prothrombin time and/or physiotherapistPTC percutaneous transhepatic cholangiographyPTH parathyroid hormonePTLD post-transplant lymphoproliferative disorder/diseasePUVA psoralin plus ultraviolet lightPVH periventricular haemorrhagePVL periventricular leukomalaciaP-W Prader-WilliPWS Prader-Willi syndromeQEEG quantitative EEGQTc corrected QT intervalRACP Royal Australasian College of PhysiciansRAS renin angiotensin systemRAST radioallergosorbent testRBBB right bundle branch blockRBC red blood cellRCDP rhizomelic chondrodysplasia punctataRCPCH Royal College of Paediatrics and Child HealthRCT randomised controlled trialRDA recommended daily allowanceR-DPSDQ revised Denver pre-screening developmental questionnaireREM rapid eye movementRF radiofrequency and/or rheumatoid factorrhDNase 1 dornase alfa (recombinant human deoxyribonuclease 1)rhGH recombinant human growth hormoner-HuEPO recombinant human erythropoietinRLD restrictive lung diseaseRLS restless legs syndromeRNA ribonucleic acidROP retinopathy of prematurityRRT renal replacement therapyRSV respiratory syncytial virusRSV-Ig RSV intravenous immune globulinRTA renal tubular acidosisRTx renal transplantation
    • • Abbreviations xxixRV right ventricleRVF right ventricular failureRVH right ventricular hypertrophyRVOTO right ventricular outflow tract obstructionRWS Romano-Ward syndromeRx treatmentS1 first heart soundS2 second heart soundS3 third heart soundS4 fourth heart soundSABA short acting beta-2 adrenoreceptor agonistSaO2 oxygen saturationSAP serum alkaline phosphataseSBBOG small bowel bacterial overgrowthSBDS Shwachman-Bodian-Diamond syndromeSBE subacute bacterial endocarditis and/or small bowel enteroclysisSBFT small bowel follow throughSBS short bowel syndromeSBVCE small bowel video-capsule endoscopySC subcutaneousSCA sickle cell anaemiaSCAIP single-condition amplification internal primer sequencingSCD sickle cell disease and/or sudden cardiac deathSCFE slipped capital femoral epiphysisSCN sodium channelSCQ social communication questionnaireSCT stem cell transplantationSDB sleep disordered breathingSDR selective dorsal rhizotomySDS Shwachman-Diamond syndrome and/or standard deviation scoreSEMLS single event multilevel surgerySGA small for gestational ageSI sacroiliacSIADH syndrome of inappropriate antidiuretic hormone secretionSIDS sudden infant death syndromeSLD specific learning difficultiesSLE systemic lupus erythematosusSMA spinal muscular atrophySMCP submucosal cleft palateSMEB severe myoclonic epilepsy borderlineSMEI severe myoclonic epilepsy in infancy (Dravet syndrome)SMO supramalleolar orthosisSOD septo-optic dysplasia and/or superoxide dismutaseSOJIA systemic onset juvenile idiopathic arthritisSPECT single photon emission computed tomographysPLA2 secretory phospho-lipase A2SQUID superconducting quantum interference deviceSRY sex-determining region on the Y chromosomeSSPE subacute sclerosing panencephalitisSSRI selective serotonin reuptake inhibitorST speech therapist
    • xxx Examination paediatricsSTAR steroidogenic acute regulatory proteinSTE speckle tracking echocardiographySUNDS sudden unexpected nocturnal death syndromeSVAS supravalvular aortic stenosisSVC superior vena cavaSVT supraventricular tachycardiaS-W Sturge–Weber syndromeSX salmeterol xinofoateT1DM type 1 diabetes mellitusTAB tiagabineTAC tacrolimus (alternate abbreviation TRL)TAM transient abnormal myelopoiesisTAPVD total anomalous pulmonary venous drainageTAR thrombocytopenia absent radius syndromeTB tuberculosisTBI total body irradiationTc-99m technetium-99mTCA tricyclic antidepressantTCD transcranial DopplerTDI tissue Doppler imagingTdP torsades de pointes (twisting of points)TEV talipes equinovarusTGA transposition of the great arteriesTIBC total iron-binding capacityTIPSS transjugular intrahepatic portosystemic stent shuntTL transient leukaemiaTLE temporal lobe epilepsyTMD transient myeloproliferative diseaseTMJ temporomandibular jointTMP-SMX trimethoprim with sulfamethoxazoleTNF tumour necrosis factorTNF-α tumour necrosis factor alphaTOF tetralogy of Fallot and/or tracheo-oesophageal fistulaTORCH toxoplasmosis, other [e.g. syphilis], rubella, CMV, HSV/ HVZTRF teacher report formTRL tacrolimus (alternate abbreviation TAC)TPM topiramateTPMT thiopurinemethyltransferaseTPN total parenteral nutritionTS Turner syndrome and/or tuberous sclerosisTSC tuberous sclerosis complexTSH thyroid stimulating hormonetTG tissue transglutaminaseUAC umbilical arterial catheterUC ulcerative colitisUCB umbilical cord bloodUCBT umbilical cord blood transplantationUMN upper motor neuroneUMOD uromodulin (gene)UPPP uvulopharyngopalatoplastyURSO ursodeoxycholic acidURTI upper respiratory tract infection
    • • Abbreviations xxxiUS upper segmentUTI urinary tract infectionVA ventriculoatrialVACTERL vertebral, anal, cardiac, tracheo-esophageal, renal, limb (anomalies)VBT vigabatrin (one of two widely used abbreviations; the other is VGB)VCFS velocardiofacial syndromeVCR vincristineVER visual evoked responseVF ventricular fibrillationVGB vigabatrin (preferred abbreviation)VNS vagal nerve stimulationVNTR variable number of tandem nucleotide repeatsVOC vaso-occlusive crisesVOE vaso-occlusive pain eventsVP ventriculoperitonealVPA valproateVPI velopharyngeal incompetenceVSD ventricular septal defectVT ventricular tachycardiaVUR vesicoureteric refluxvWF von Willebrand’s factorWAGR Wilms, aniridia, genital [anomalies], retardation syndromeWBSCR Williams-Beuren syndrome critical regionWD Wilson diseaseWFH World Federation of HemophiliaWGA whole gene amplificationWHO World Health OrganizationWHtR waist-to-height ratioWISC-R Wechsler Intelligence Scale for Children-revisedWPPSI Wechsler preschool and primary scale of intelligenceWPW Wolff-Parkinson-White syndromeWS Williams syndromeWt weightXRT radiotherapyYSR youth self-report
    • IntroductionThis book is designed primarily to assist candidates in passing clinical examinationsin paediatrics, particularly at the postgraduate level. The first edition was foundhelpful in many countries with many different clinical examination scenarios,although it was specifically designed to tackle the clinical section of the FRACP Part 1Examination in Paediatrics and the MRCP (now the MRCPCH) Part 2 examination.Written examinations, which must be passed in most countries before any clinicalexamination can be sat, are not covered other than in the brief outline given below.Basic training requirementsMost potential candidates should be familiar with these. Comprehensive informationon training can be obtained and downloaded from the websites of the various learnedcolleges. For Australia, New Zealand, the United Kingdom and Canada, the relevantaddresses are as follows: • Fellowship in the Royal Australasian College of Physicians (FRACP): http://www. racp.edu.au/paed/index.htm • Membership of the Royal College of Paediatrics and Child Health (MRCPCH): http://www.rcpch.ac.uk/rcpch/index.htm • Fellowship of the Royal College of Physicians of Canada (FRCPC): http://rcpsc. medical.org/english/index (click on ‘Residency Education’ for this one)The written examinationIn Australia, the written component of the FRACP examination comprises two paperstaken on the same day. Paper 1 contains 70 A-type questions (best single responseof five alternatives) testing knowledge of basic sciences and lasts two hours, whilepaper 2 consists of 100 A-type questions assessing investigational material, clinicalpaediatrics and therapeutics, and lasts three hours. It is held annually. In the UK, the MRCPCH Part I examination lasts two and a half hours, andcomprises 60 multiple-choice questions (MCQs), 15 of which are common to theMRCPCH and the MRCP(UK). The written component of the MRCPCH PartII examination comprises three short-answer papers: the first is the case historypaper, containing four or more compulsory questions and lasting 55 minutes; thesecond is the data interpretation paper, containing 10 compulsory questions andlasting 45 minutes; and the third is the photographic material paper, containing 20compulsory questions and lasting 50 minutes. The best preparation for any written examination involves doing as many pastexamination questions as possible, and extensive reading of major texts and journals.The most useful textbooks and journals are listed under Suggested Reading at the endof the book. There are no set curricula specified for most paediatric examinations. The college regularly releases previous examination papers. These are essentialreading. There are also a number of papers that are composites of rememberedquestions that previous candidates have written down after their examination, whichhave been ‘handed down’ over the years. The main problem with ‘remembered’ papersis their inaccuracy. However, most candidates seem to find these helpful. xxxiii
    • xxxiv Introduction The RACP produces self-assessment questions for paediatricians, the AustralianSelf-Assessment Programme (ASAP), which are strongly recommended. Generally, books of multiple-choice questions available in any medical bookshopare of much less value. Remember, repeated practising of multiple-choice questions is the most valuablepreparation.The clinical examinationThis book is aimed at assisting in preparing for clinical examinations. Chapter 1details a general approach. It will be noted that certain cases are emphasized, notbecause they are more important than others but because they are good examples ofthe complicated material required for long and short cases.The Mini-Clinical Evaluation Exercise (mini-CEX)This is a recent addition to the trainee’s preparation for paediatric practice. It hasbeen introduced by the RACP as a formative assessment tool to evaluate traineesin real-life medical settings, in their normal working environment, as part of thePhysician Readiness for Expert Practice (PREP) Program. A variety of environmentsshould be evaluated during the trainee’s basic training, not just one favoured hospitaldepartment. Skills assessed include history taking, communication skills, physicalexamination and management strategies. A mini-CEX is not unlike a hybrid longcase/short case, so becoming proficient at these will assist trainees in handlingshort and long cases. Trainees receive valuable feedback to assist their learning,although the mini-CEX does not contribute to decisions regarding eligibility forprogression through the PREP programme. Trainees are rated in the following areasas listed on the mini-CEX evaluation sheet: medical interviewing skills; physicalexamination skills; professional qualities/communication; counselling skills; clinicaljudgement; and organisation/efficiency. The trainee is responsible for organisingtheir own mini-CEX encounters. Each of these assessments should focus on a fewspecified competencies. Each case should take around 15–20 minutes, immediatelyfollowed by feedback from the assessor, this feedback being the most usefulcomponent of the mini-CEX process. For more information, trainees should go tohttp://www.racp.edu.au or http://www.racp.org.nz.Achievement psychology: the psychology of passingChapter 4 discusses the psychological aspects of the preparation for, and performancein, the examination. This is a very important area that should not be overlooked.
    • Chapter 1Approach tothe examinationPositive mindsetAll paediatric clinical examinations test the following aspects: 1. Clinical skills—history taking, physical examination, interpretation of findings, construction of a diagnosis or differential diagnoses, method of investigation, overall management of the patient. 2. Attitudes. 3. Interpersonal relationships.Candidates invited to postgraduate clinical examinations have usually satisfied theirrelevant learned college regarding their factual knowledge. Consequently, their factualknowledge should be at a standard appropriate for making management decisions inthe case being examined. Clinical skills are usually taught adequately to most candidates at the hospitalswhere they were trained. However, little if any attention is paid to developing properattitudes and interpersonal relationship skills. Advertisers and sales representativesknow the importance of personal contact. They realise that appearance, personalityand speech are crucial in successful negotiations. The ‘viva’ is very similar in thatcandidates have to ‘sell’ themselves and their knowledge to the examiners. Successfulcandidates usually possess certain characteristics, namely: 1. A positive, confident response to personal confrontation. People with strong personalities have little trouble, but those who are easily embarrassed and shy away from confrontation between equals may do poorly. This response can be changed by methods described in this book. 2. Ability to sort out relevant from irrelevant information. Those who ‘think’ ask , questions, seek explanations and try to understand rather than learn by rote do well. 3. Familiarity with the method of examination. 4. Endurance. Candidates who are ‘street-wise’ and naturally confident tend to have little difficulty, whereas their less confident colleagues are left to learn the hard way and eventually succeed or fail.Preparation for the ‘viva’ requires effective communication skills during physicalconfrontation. Attitudes, interpersonal skills and projection of a confident,professional consultant image can be learned and developed. Techniques include: 1. Mental rehearsal, visualisation, affirmations (see Chapter 4). 2. Body language. 3. Eye contact. 4. Breath control. 5. Dress sense. 6. Speech training. 7. Development of equanimity. 1
    • 2 Examination paediatrics Ability to summarise. 8. Reasoning skills. 9.10. Examiner assessment.Body languageNon-verbal communication in the form of a person’s gestures is a very accurateindicator of his or her attitudes, emotions, thoughts and desires. In order to learn body language, set aside a couple of minutes a day to study and readother people’s gestures. Examine your own body language. Copy the body languageof people you admire and respect, such as a consultant who you feel would have nodifficulty passing the clinical examination. The model you choose does not necessarilyhave to be a real person: he or she may be a composite of ideal body language. There is an old saying: ‘If you would be powerful, pretend to be powerful’. Oneway to adopt an attitude that helps you achieve any objective is to act ‘as if ’ you werealready there. If you change your posture, your breathing patterns, your muscletension, your tone of voice, you instantly change the way you feel. For example, ifyou feel depressed, consciously stand up straight, throw your shoulders back, breathedeeply and look upward. See if you can feel depressed in that posture. You’ll findyourself feeling alert, vital and confident. An important component of body language is consistency. If you are giving what youthink is a positive message, but your voice is weak, high-pitched and tentative and yourgestures reveal poor self-confidence, you will be unconvincing and ineffective. Individualswho consistently succeed are those who can commit all of their resources, mental andphysical, towards reaching a goal. One way to develop consistency is to model yourselfon individuals who are consistent. Copy the way they stand, sit and move, their key facialexpressions and gestures, their tone of voice, their vocabulary, their breathing patterns andso on. You will begin to generate the same attitudes that they experience, and experiencethe same successful results. Effectiveness comes from delivering one unified message. When you next attend a place where people meet and interact, study the individualswho have adopted the gestures and postures of the individuals with whom they aretalking. This mimicry is how one individual tells another that he or she is in agreementwith their thoughts and attitudes. You can use this unconscious mimicry to youradvantage. One of the best ways of establishing effective personal communication isthrough mimicking the breathing patterns, posture, tone of voice, gestures, words andphrases of the person or people with whom you are interacting. Once you establishcontact with someone, you create a bond and reach a stage where you begin to initiatechange rather than just mimicking the other person, a stage where you have establishedso much mutual contact that when you change, the other person unconsciously followsyou. If, when you try to lead someone, he or she does not follow, it simply means thereis insufficient rapport. Mimic, strengthen the mutual contact and try again.Eye contactWhen answering questions or making a point, look the examiner straight in the eye.Powerful individuals have always been characterised by exceptional eyes. If you havedifficulty maintaining eye contact, there are a few techniques you can use to developa more effective gaze. Work on not blinking. Practise unblinking eye contact, especially when underpressure. If you are intimidated and unable to look directly into the examiner’s eyes, alittle trick is to concentrate your gaze on a point midway between the examiner’s eyes.
    • 1 • Approach to the examination 3Another helpful hint is to imagine a triangle on the examiner’s forehead, with the apexat the highest point and the base of the triangle formed by an imaginary line betweenboth eyes. Keep your gaze directed at this area. In the mirror, practise narrowing yourlids a bit but do not squint. When you move your eyes from one point to another(as from one examiner to another), do it without blinking. When you look from oneperson to another without blinking, it is unnerving for anyone watching you. Tofurther emphasise this powerful gaze, move your eyes first without blinking and thenfollow with your head movement just behind the eye movement. Slightly loweringyour head forwards while maintaining the gaze also adds power to the eye movement.Breath controlOften, especially in a viva voce or an important interview, you find yourself struck bya sudden panic attack. Breath control can be particularly useful to regain composure,prevent fear, reduce stress or fatigue, and generate energy. It is a technique developedby the samurai to regain control during life and death struggles. Take a deep breath,then exhale slowly and imperceptibly. As you are exhaling, contract your abdominalmuscles so that you feel as though you are tightening a corset. Relax the muscles at theend of exhalation. Do not expel all the air. Leave about 20% in the lungs. Then inhalegently. Your lips should be slightly parted, expelling your breath over your lower teethwith your tongue gently touching your hard palate. You may repeat breath controlas often as required. Practise breath control until it is second nature and then use itwhenever you are under any physical or mental stress.Dress and groomingYou have to ‘sell’ yourself and your knowledge. Your appearance is your most important‘equipment’ for the clinical examination. It must reflect the public expectations ofthe professional person. Look around at what is worn by successful individuals inthe respected professions (such as your examiners). Ask yourself: ‘Do I look likea mature, careful, conservative and respected junior consultant?’ Male candidatesshould avoid colourful suits, jackets and trousers, and unusual ties. Dark suits (greys,navy blue, pin-stripes, non-committal ties, red ties) are more suitable (this is termed‘power dressing’ by image consultants). Female candidates should preferably avoidrevealing or tight dresses as well as showy jewellery. Footwear should be clean andappropriate. Long hair and beards should probably be avoided, but if you cannot bear to shaveyour beard, at least keep it neat and trimmed. It is safer to be clean shaven and havetidy hair. Visit the hairdresser a couple of days before (and not on the day of) theclinical examination so that it does not appear as though you have had it done just forthe examinations. If you have difficulties with perspiration when under pressure, use an unscentedantiperspirant on the day of the examination. Apply the antiperspirant to yourforehead, hairline and neck to prevent beads of sweat appearing during the ‘viva’ (oneof the previous co-authors used this method successfully).Speech trainingPoor speech will definitely adversely affect your ‘viva’ performance and is surprisinglycommon. By poor speech we mean poor diction, inaudible voice and bad vocabulary.
    • 4 Examination paediatricsTape-record yourself speaking or have someone sit about 2 metres from you andlisten to you speak. Then note: 1. Whether or not every word was heard clearly. 2. Whether or not what you said was understood. 3. Whether or not you used jargon, slang or abbreviations.Addressing these three points will help you assess whether or not your speech isa problem. Take note of the pitch and rapidity of your speech. If you do speak tooquickly, make a conscious effort to slow down by reading aloud at a pace that allowsa listener to make a note of the content of your speech. Another useful exerciseis to study the speech of newsreaders. Note that they speak clearly, concisely andslowly so that every word is understood. They also use very little jargon, slangor abbreviations. Try mimicking a newsreader’s speaking style the next time youpresent a case. Other methods of improving your speech are: 1. Enrolment with a professional teacher. 2. Using a video camera to film your efforts and then playing back the result.EquanimitySir William Osler suggested that physicians should possess equanimity: composureunder pressure, and when faced with the adversities of life. This is particularly trueduring the clinical examination, where your long case may be an uncooperative,crying child, for example. Candidates may experience stress as a result of physicalconfrontation, inadequate knowledge, low self-confidence and poor physicalhealth. Equanimity, although coming more easily to phlegmatic personalities, maybe cultivated. Knowledge and experience create confidence, which in turn leads tocalmness. The above causes of stress can all be overcome by more study (to improveknowledge base), more practice, mental reprogramming and then actively seekingout stressful situations to increase experience. Adequate exercise, a well-balanced dietand enough sleep should go a long way towards maintaining physical health.Ability to summariseThe ability to summarise, encapsulate the essence and emphasise the major issueswithout losing too much detail requires understanding and experience. However, itis a necessary skill for the physician, and therefore needs to be developed throughpractice. Remember: practice makes the impossible possible. Note that the limitof effective retention of verbal information is usually less than 15 seconds, so thebetter you can convey the essential details to the examiners in the ‘viva’, the greaterthe effect.Reasoning skillsReasoning skills involve the ability to analyse a problem rapidly, break it down intomanageable parts and then formulate a solution. An adequate knowledge base,experience and rational thinking are needed. To assist in developing these skills, make a habit of meticulously examiningevery detail of a child’s clinical records. Analyse the data, learn to pick out any vitalinformation that is missing and ignore irrelevant information. Deduce from availabledata what other information you need to justify your conclusions.
    • 1 • Approach to the examination 5Assessment of examinerAn ancient Chinese general, Sun Tze, once stated, ‘Know the enemy and knowyourself, and you can fight a hundred battles with no danger of defeat’. Althoughexaminers are not exactly the enemy, you still need to assess: 1. Whether or not the examiner is friendly or unhelpful. 2. The quality of communication between the examiner and yourself. 3. The strength of the examiner’s personality compared to yours.On occasions, it may be worthwhile doing some reconnaissance work and finding outsome information about your potential examiners (likes, dislikes, areas of interest).The clinical examinationPreparationThe road to success in any ‘viva’ usually entails doing a large number of long andshort cases. You need to begin seeing cases at least several months before the clinicalexamination. Your service commitment should provide you with all the material yourequire for training and preparation. Treat each patient you see during the course ofyour daily work as a practice long or short case. Endeavour to do at least one (butpreferably two or three) long case(s) per week. Try to expose yourself to as manydifferent examiners as possible. Preparation for the short case requires much practice, especially when morecandidates fail their short cases than their long cases. If possible, visit other hospitals,especially if these have a reputation for teaching. Experience as a ‘bulldog’ (i.e.observing an actual examination and assisting the candidate) is also invaluable ingaining insight about the conduct of the examination and the expectations of theexaminers. Taking turns as an examiner during practice sessions with your colleaguesis worthwhile because it allows you to experience first-hand the annoying habits ofcandidates. Mental rehearsal of short cases (and long cases) will accelerate learning(see Chapter 4, Achievement psychology). Most major teaching hospitals hold trial and mock examinations a few weeksbefore the actual ‘viva’. These simulated examinations provide invaluable feedbackabout your progress. Part of your preparation involves obtaining all the relevant information from theappropriate learned college about the requirements for paediatric training; this isinvariably available in booklet form or is downloadable from the college’s website.Familiarise yourself well with the contents and study the regulations. About 6 monthsbefore the examination, write to the college for an application form. Fill in yourapplication form a few months before the examination and ensure that the form andyour examination fee reach the college before the closing date. Remember to apply forexamination leave from your employer. If possible, in the last few weeks before the ‘viva’, do some reconnaissancework and locate the examination venue as far as you can. Check it out in relationto where you will be staying, ascertain the suitable modes of transport, publictransport timetables and the length of time it will take to travel to the venue at thetimes scheduled for the examinations. Remember to check your accommodationarrangements in advance. In the week before the examination, check the clothing you intend to wear (makesure it fits!), check that you have the right equipment and remember the appointmentwith the hairdresser. In the few days before the examination, try to get some mentalrest and leave study aside. Avoid any major changes in your daily routine and lifestyle.
    • 6 Examination paediatrics A checklist (prepared in advance) of what you need to do on the day may behelpful. Make sure you arrive at least 30 minutes before the examination is due tostart, so that you can recover from the trip, relax, go to the bathroom and so on.The longer the travelling distance, the more provision you need to make to cover fordelays during travel (one of the previous co-authors experienced car trouble on theday of the ‘viva’). There are certain ‘rules’ that you need to obey at the ‘viva’. Do not enter theexaminer’s room until asked. Do not sit down until invited. Do not slouch whenseated but sit four-square and upright (it creates the impression that you mean whatyou say). Minimise nervous hand movements. If you tend to fidget, turn your handmovements into gestures. Bags should be placed under the chair (or given to the‘bulldog’) and removed when leaving. Do not stare, smile politely and always answercourteously. Remember to speak up and be as brief and factual as possible. Avoidjargon, slang, clichés, abbreviations, brand names (of medications), meaninglessexpressions, and rising inflections at the end of sentences. Most importantly, donot antagonise or argue with the examiners. You will always lose! Some examples ofantagonising behaviour include patronising answers, appearing to show little or nointerest in the subject matter and a negative response to criticism. If you decide to use beta-blockers during the examination, it would be wise touse the drug during at least one practice session, particularly if you have asthma. Itwould be inconvenient to have an asthma attack at the examination, as happened to acolleague who took a cardioselective beta-blocker. After 12 puffs or so of salbutamol,he passed.EquipmentEquipment is always available at the examination. However, it would be wise to takethe following with you: 1. Pens (more than one and make sure they work), pencils and paper. 2. Your own stethoscope. 3. A hand-held eye chart for testing visual acuity (with a piece of string attached to the chart, the length of which is the recommended distance from eye to chart; this allows you to quickly position the chart at the correct distance from eye). 4. Cotton wool (fine sensation), new blunt pins. 5. Watch, preferably with a sweeping second hand. 6. Pocket tape measure. 7. Torch with new batteries and bulb. 8. A red-topped hat pin for visual field testing in the older child. 9. Toys, red woollen ball on a thread to test visual fields in infants, small container of hundreds and thousands (or equivalent) to test hearing, raisins (pincer grip), coloured cubes for developmental assessment. 10. Other: pocket ophthalmoscope, pocket tendon hammer, spatulas, tuning forks, Denver II developmental chart, percentile charts for height, weight and head circumference.