RIS ANU es Nc Questions for the MRCPCH Part 2 Written Examination Nick Barnes Wr erel te! Julian FortonQuestions for the MRCPCH Part 2 Written Examination Nick Barnes BSc MBBS MRCPCH Specialist Registrar Paediatrics Department of Paediatrics John Radcliffe Hospital Oxford Julian Forton MA MB BChir MRCPCH Specialist Registrar and Clinical Research Fellow Department of Paediatrics John Radcliffe Hospital Oxford© 2004 PasTest Ltd Egerton Court Parkgate Estate Knutsford Cheshire, WA16 8DX Telephone: 01565 752000 All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise without the prior permission of the copyright owner. First edition 2004 ISBN: 1 904627 16 1 A catalogue record for this book is available from the British Library. The information contained within this book was obtained by the authors from reliable sources. However, while every effort has been made to ensure its accuracy, no responsibility for loss, damage or injury occasioned to any person acting or refraining from action as a result of information contained herein can be accepted by the publisher or the authors. PasTest Revision Books and Intensive Courses PasTest has been established in the field of postgraduate medical education since 1972, providing revision books and intensive study courses for doctors preparing for their professional examinations. Books and courses are available for the following specialties: MRCP Part | and Part 2, MRCPCH Part | and Part 2, MRCOG, DRCOG, MRCGP, MRCPsych, DCH, FRCA, MRCS and PLAB. For further details contact: PasTest Ltd, Freepost, Knutsford, Cheshire, WA16 7BR Tel: 01565 752000 Fax: 01565 650264 Email: enquiries@pastest.co.uk Web site: www. pastest.co.uk ‘Typeset by Saxon Graphics Ltd, Derby Printed by Cambrian Printers, AberystwythCONTENTS Introduction Glossary of abbreviations Question 1 Question 2 Question 3 Question 4 Question 5 Question 6 Question 7 Question 8 Question 9 Question 10 Question 11 Question 12 Question 13 Question 14 Question 15 Question 16 Question 17 Question 18 Question 19 Question 20 Question 21 Question 22 Question 23 Question 24 Question 25, Question 26 Question 27 Question 28 Question 29 Question 30 Question 31 Question 32 Question 33 Question 34 Index vi 13 22 28 41 48 63 79 83 92 100 114 125 134 141 148 155, 163 173 179 188, 196 202 208 216 225 230 aa? 244 256 261 266 269 276INTRODUCTION The written component of the Part 2 MRCPCH examination has historically consisted of three sections — long cases (grey cases), short cases (data interpretation) and clinical photographs. The examination has recently changed in format; the same material is now presented as longer cases integrating difficult differential diagnoses, data and photographs together to create a more in-depth clinical scenario. The obvious advantage to this type of question is that the cases presented are more typical of everyday working practice. Understanding the expected progression of a disease, or the potential repercussions of certain interventions or omissions in treatment, is a part of medicine that is gained with experience rather than from straightforward texts. Children can become ill very quickly and their clinical condition often varies enormously with time. Continuous reassessment with expectant management is therefore of paramount importance and should be a daily routine. Using the progression of an ill child with time as the basis for a question, rather than presenting a snapshot in time, tests a more subtle knowledge of clinical paediatrics. The questions in this book incorporate a clinical history with photographs, radiological investigations and data interpretation, and most involve several stages of decision making. Both general paediatrics and neonatology are covered. Each case-based question is accompanied by a comprehensive explanation and broad overview of the subject pertinent to the individual case. Acknowledgements We thank the Meningitis Research Foundation for permission to include the algorithm “Early Management of Meningococcal Disease in Children”. We also thank the Consultant staff at the John Radcliffe Hospital, Oxford and at the Royal Berkshire Hospital, Reading for their advice in the preparation of this book.GLOSSARY OF ABBREVIATIONS ABPA Allergic bronchopulmonary aspergillosis ACE Angiotensin converting enzyme ACTH Adrenocorticotrophic hormone AER Auditory evoked response AIS Androgen insensitivity syndrome ALP Alkaline phosphatase ALT Alanine transaminase ANA Anti-nuclear antibody APTT Activated partial thromboplastin time ASB Assisted support of breathing ASD Atrial septal defect ASOT Anti streptolysin-O-titre AV Atrioventricular AVSD. Atrioventricular septal defect BE Base excess BECTS _ Benign epilepsy with centrotemporal spikes CF Cystic fibrosis CK Creatine kinase CLD Chronic lung disease CMV Cytomegalovirus CMV Continuous mandatory ventilation CPAP. Continuous positive airway pressure cRP C-reactive protein CSF Cerebrospinal fluid cT Computed tomography CTG Cardiotocogram DcT Distal convoluted tubuleDHT DKA DMSA DTPA EBM EBV EMLSCS ESR Err FISH GAL-1-PUT. GORD HCG HDU HSV IVC IVU LDH LFT LHRH LRTI MAG3 MCNS. MCUG MIBG MIH MRI NEC NE NG NPA NVD Ol PA PAPVD PCP PCR PDA PEEP Ces Dihydrotestosterone Diabetic ketoacidosis Dimercaptosuccinic acid Diethylenetriaminepentaacetic acid Expressed breast milk Epstein-Barr virus Emergency lower segment caesarean section Erythrocyte sedimentation rate Endotracheal tube Fluorescent in-situ hybridisation Galactose-1-phosphate uridyl transferase Gastro-oesophageal reflux disease Human chorionic gonadotrophin High dependency unit Herpes simplex virus Inferior vena cava Intravenous urogram Lactate dehydrogenase Liver function test Luteinising hormone releasing hormone Lower respiratory tract infection Mercaptoacety! triglycine Minimal change nephrotic syndrome Micturating cystourethrogram 123-m-iodobenzylguanidine Mullerian inhibitory hormone Magnetic resonance imaging Necrotising enterocolitis Neurofibromatosis Nasogastric Nasopharyngeal aspirate Normal vaginal delivery Oxygenation index Pulmonary artery Partial anomalous pulmonary venous drainage Pneumocystis carinii pneumonia Polymerase chain reaction Patent ductus arterious Positive end expiratory pressureSIADH SIPPV SLE SSNS SVC SVT TGA UAC USS UTI UVC VSD VUR VZIG CT Usm nanan) Pulmonary interstitial emphysema Prothrombin time Pelvico-ureteric junction obstruction Psoralen with ultraviolet A light Renin-angiotensin-aldosterone Radioallergosorbent test Respiratory distress syndrome Respiratory rate Respiratory syncytial virus Severe acute respiratory syndrome Severe combined immunodeficiency disease Serum glutamate oxaloacetate transaminase (aka aspartate aminotransferase, AST) Syndrome of inappropriate antidiuretic hormone secretion Synchronised intermittent mandatory ventilation Synchronised intermittent positive pressure Systemic lupus erythematosus Steroid sensitive nephrotic syndrome Superior vena cava Supraventricular tachycardia Transposition of the great arteries Umbilical artery catheter Ultrasound scan Urinary tract infection Umbilical venous catheter Ventricular septal defect Vesicoureteric reflux Varicella zoster immune globulinAn (1-month-old girl, born at term with no perinatal complications, was admitted to hospital with high swinging fever, tachycardia and pyurias Height and weight were on the 3rd centile. Urine from suprapubic aspirate revealed > 100, 000 organisms and Escherichia‘coli was grown on urine culture. She was treated successfully with iv cefirroxime for 5 days. She was commenced on prophylactic trimethoprim prior to discharge and was extensively investigated over the next 3 months.Wee as 1.1. What investigation is this? A Micturating cystourethrogram B Indirect cystogram C Intravenous urogram D_ DMSA scan EB F DTPA scan MAG3 scan (late views) 2. What is demonstrated? Horseshoe kidney Right-sided vesicoureteric reflux (VUR) led ectopic kidney ided renal agenesis Right-sided pyelonephritis O7M™™ OOD DS pg: & = 2. = 8 a = Z & 3 § a s = g 3 What is the likely pathology? Right-sided grade 2 VUR Right-sided grade 3 VUR Right-sided grade 4 VUR Right-sided pelvicoureteric junction obstruction Left-sided pelvicoureteric junction obstruction Posterior urethral valves Right-sided renal scarring Tamm IAwe> Left-sided renal scarring