Oxford Specialist Handbooks in Paediatrics Paediatric Nephrology Second edition Lesley Rees MD, FRCPCH Consultant Paediatric Nephrologist Gt Ormond St Hospital for Children NHS Trust London, UK Paul A. Brogan MSc, PhD, MRCP, FRCPCH Senior Lecturer and Honorary Consultant Paediatric Rheumatologist Gt Ormond St Hospital for Children NHS Trust London, UK Detlef Bockenhauer PhD, FRCPCH Consultant Paediatric Nephrologist Gt Ormond St Hospital for Children NHS Trust London, UK Nicholas J.A. Webb DM, FRCP, FRCPCH Consultant Paediatric Nephrologist Royal Manchester Children’s Hospital Manchester, UK 1 1 Great Clarendon Street, Oxford OX2 6DP United Kingdom Oxford University Press is a department of the University of Oxford. It furthers the University’s objective of excellence in research, scholarship, and education by publishing worldwide. Oxford is a registered trade mark of Oxford University Press in the UK and in certain other countries © Oxford University Press 2012 The moral rights of the authors have been asserted First edition published 2007 Second edition published 2012 Impression: 1 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, without the prior permission in writing of Oxford University Press, or as expressly permitted by law, by licence or under terms agreed with the appropriate reprographics rights organization. Enquiries concerning reproduction outside the scope of the above should be sent to the Rights Department, Oxford University Press, at the address above You must not circulate this work in any other form and you must impose this same condition on any acquirer British Library Cataloguing in Publication Data Data available Library of Congress Cataloging-in-Publication Data Paediatric nephrology / Lesley Rees . [et al.].—2nd ed. p. ; cm.—(Oxford specialist handbooks in paediatrics) Rev. ed. of: Paediatric nephrology / Lesley Rees, Nicolas J.A. Webb, Paul A. Brogan. 2007. Includes bibliographical references and index. ISBN 978–0–19–960137–0 (alk. paper) I. Rees, Lesley, 1951-II. Rees, Lesley, 1951-Paediatric nephrology. III. Series: Oxford specialist handbooks in paediatrics. [DNLM: 1. Kidney Diseases—Handbooks. 2. Child. 3. Infant. WS 39] LC classifi cation not assigned 618.92’61–dc23 2011033028 ISBN 978–0–19–960137–0 Printed in China on acid-free paper by Asia Pacifi c Offset 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 clinical procedures with the most up-to-date published product information and data sheets provided by the manufacturers and the most recent codes of conduct and safety regulations. The authors and the publishers do not accept responsibility or legal liability for any errors in the text or for the misuse or misapplication of material in this work. Except where otherwise stated, drug dosages and recommendations are for the non-pregnant adult who is not breast-feeding Links to third party websites are provided by Oxford in good faith and for information only. Oxford disclaims any responsibility for the materials contained in any third party website referenced in this work. iii Preface to second edition Following on from the success of the fi rst edition of our handbook, we have produced a second edition which has been totally updated. In par- ticular, we have brought in our colleague, Dr Bockenhauer. We have used his expertise to ensure that this second edition is fully up-to-date in genetics. In addition, he has developed and simplifi ed the concepts of electrolyte balance by going back to basic renal physiology. Our original aim in producing a handbook that is a comprehensive and clinically-orientated guide to the management of children with all forms of renal disease remains. We have continued to try to make the text as practical as possible in the expectation that this will become the fi rst text that the paediatrician refers to when presented with a child with an acute or chronic kidney disorder. The book provides up-to-date information for the general paediatrician and the paediatric nephrologist. It is pocket sized so that it is easily referred to in the clinical setting and uses a system of bullet points to facilitate this. It covers both common conditions seen by all paediatricians during the day-to-day care of patients on a general paediatric ward or in the outpatient clinic, and also those seen rarely and usually in specialized paediatric nephrology centres in collaboration with local units. The focus is heavily on investigation and management and there is intentionally little emphasis on basic pathogenesis and disease mechanisms. Where possible we have made evidence-based recommendations, though in the many instances where high quality evidence is lacking, we make recommendations based on the authors’ personal experience and current best practice. All of the chapters have been written by the four authors, who are experienced consultants at two large UK Children’s Hospitals, although we have received signifi cant input from a number of our colleagues (listed on pages vi and vii) to whom we are most grateful. We would also like to thank Helen Liepman at Oxford University Press for seeing the handbook through from conception to delivery. LR, PB, DB, and NW 2011 This page intentionally left blank v Contents Contributors vi Symbols and abbreviations viii 1 Patient assessment 1 2 The neonate 43 3 Congenital abnormalities 49 4 Urinary tract infection 75 5 Enuresis 91 6 Homeostasis 99 7 Tubular disease 139 8 Renal calculi and nephrocalcinosis 173 9 Glomerular disease 181 10 Systemic disease affecting the kidney 225 11 Vasculitis 275 12 Infections and the kidney 315 13 Renal cystic diseases and ciliopathies 319 14 Syndromes associated with renal diseases 335 15 Renal tumours 347 16 Hypertension 353 17 Acute kidney injury 377 18 Chronic kidney disease 409 19 Chronic peritoneal dialysis 465 20 Extracorporeal treatment 483 21 Transplantation 511 22 Drug prescribing 573 23 Psychosocial issues 581 Appendix: Supplementary information 599 Index 625 vi Contributors Staff at Gt Ormond St Hospital for Children NHS Trust: Dr William van’t Hoff Ms Liz Wright Consultant Paediatric Clinical Nurse Specialist in Nephrologist Extracorporeal Treatments Dr Kjell Tullus Mr David Fisher Consultant Paediatric Nurse Counsellor Nephrologist Dr Vic Larcher Dr Steven Marks Consultant Paediatrician Consultant Paediatric Nephrologist Professor Robert Kleta Professor of Paediatric Professor Neil Sebire Nephrology Consultant Paediatric Pathologist Professor Michael Dillon Ms Vanessa Shaw Emeritus Professor of Paediatric Chief Paediatric Renal Dietician Nephrology Ms Sue Patey Dr Despina Eleftheriou Senior Paediatric Renal Pharmacist Honorary Consultant Paediatric Rheumatologist Ms Eileen Brennan Nurse Consultant in Paediatric Dr Despoina Maritsi Nephrology Clinical Fellow in Paediatric Rheumatology Ms Michelle Cantwell Clinical Nurse Specialist in Peritoneal Dialysis Staff at Royal Manchester Children’s Hospital: Dr Mohan Shenoy Dr Malcolm Lewis Consultant Paediatric Nephrologist Consultant Paediatric Nephrologist Ms Hong Thoong Lead Pharmacist—Paediatric Dr Nick Plant Medicine Consultant Paediatric Nephrologist Dr Bernadette Brennan Consultant Paediatric Oncologist Dr Neville Wright Consultant Paediatric Radiologist Mr Alan Dickson Consultant Paediatric Urologist CONTRIBUTORS vii Elsewhere: Dr Declan de Freitas Dr Jonathan Evans Consultant Nephrologist Consultant Paediatric Nephrologist Manchester Royal Infi rmary, Queens Medical Centre, Manchester, UK Nottingham, UK Dr Judith E Worthington Dr Ian Roberts Principal Clinical Scientist Consultant Renal Histopathologist Manchester Royal Infi rmary, John Radcliffe Infi rmary, Manchester, UK Oxford, UK Dr Bronwyn Kerr Dr Annett Birgitta Kranz Consultant Clinical Geneticist, Consultant Paediatric St Mary’s Hospital, Nephrologist Manchester, UK Universitätsklinikum Münster Klinik u.Poliklinik Dr Kay Metcalfe f.Kinderheilkunde, Germany Consultant Clinical Geneticist, St Mary’s Hospital, Manchester, UK viii Symbols and abbreviations G -GT gamma-glutamyltransferase AA amyloid-A AAV ANCA-associated vasculitis Ab antibody ABMR antibody-mediated rejection ABPM ambulatory blood pressure monitoring ACEI angiotensin converting enzyme inhibitor aCGH array comparative genomic hybridization (assay for chromosomal microdeletions/insertions) ACL anticardiolipin antibody ACR American College of Rheumatology AD autosomal dominant ADH anti-diuretic hormone ADPKD autosomal dominant polycystic kidney disease AFP alpha fetoprotein AG anion gap AKI acute kidney injury AL amyloid-L ALG anti-lymphocyte globulin ALMS Asprevas Lupus Management Study ALP alkaline phosphatase ALT alanine transaminase ANA antinuclear antibodies ANCA anti-neutrophil cytoplasmic antibody anti-ds-DNA anti double stranded DNA AP alternative pathway APA antiphospholipid antibodies APC antigen-presenting cells APD automated peritoneal dialysis APS antiphospholipid syndrome APTT activated prothrombin time AQ2 aquaporin 2 AR autosomal recessive ARB angiotensin II receptor blocker ARPKD autosomal recessive polycystic kidney disease AS Alport syndrome ASOT anti-streptolysin O titre SYMBOLS AND ABBREVIATIONS ix AST aspartate aminotransferase ATG anti-thymocyte globulin ATN acute tubular necrosis AV arteriovenous AVP arginine vasopressin AXR abdominal X-ray BCG Bacille Calmette-Guérin bd twice daily BHS British Hypertension