Table Of ContentOxford 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
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The moral rights of the authors have been asserted
First edition published 2007
Second edition published 2012
Impression: 1
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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
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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
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v
Contents
C ontributors vi
S ymbols and abbreviations v iii
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
A ppendix: Supplementary information 599
I ndex 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
Professor Robert Kleta
Nephrologist
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
A SOT 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 Society
BKV BK virus
BM basement membrane
BMI body mass index
BMT bone marrow transplant
BNF-C British National Formulary for Children
BOR branchio-oto-renal
BP blood pressure
BSA body surface area
BTS British Transplantation Society
BVAS Birmingham vasculitis activity score
CAA coronary artery abnormality
CAKUT congenital abnormalities of the kidney and urinary tract
cANCA cytoplasmic anti neutrophil cytoplasmic antibody
CAPD continuous ambulatory peritoneal dialysis
CAPS catastrophic antiphospholipid syndrome
CaSR calcium sensing receptor
CAVH continuous arteriovenous haemofi ltration
CBD chronic beryllium disease
CCF congestive cardiac failure
CCPD continuous cycling peritoneal dialysis
CD collecting duct
CD20 cluster differentiation
CF cystic fi brosis
CFB complement factor B
CFH complement factor H
CFI complement factor I
CFTR cystic fi brosis transmembrane regulator
CFU colony-forming units
CGH comparative gene hybridization
CIC clean intermittent catheterization
CIMT carotid intima media thickness
CINCA chronic infantile neurological, cutaneous, and articular
(syndrome)
