Manual of Pediatric Nephrology
Manual of Pediatric Nephrology Kishore Phadke • Paul Goodyer Martin Bitzan Editors Manual of Pediatric Nephrology Editors Kishore Phadke Department of Pediatric Nephrology Children’s Kidney Care Center St. John’s Medical College Hospital Bangalore, KA India Martin Bitzan Division of Pediatric Nephrology Montreal Children’s Hospital McGill University Montreal, QC Canada Paul Goodyer Division of Pediatric Nephrology Montreal Children’s Hospital McGill University Montreal, QC Canada ISBN 978-3-642-12482-2 ISBN 978-3-642-12483-9 DOI 10.1007/978-3-642-12483-9 Springer Heidelberg New York Dordrecht London (eBook) Library of Congress Control Number: 2013948392 © Springer-Verlag Berlin Heidelberg 2014 This work is subject to copyright. 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Violations are liable to prosecution under the respective Copyright Law. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. While the advice and information in this book are believed to be true and accurate at the date of publication, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper Springer is part of Springer Science+Business Media (www.springer.com) Foreword I The International Society of Nephrology strives to improve access to the best possible kidney care worldwide. An important step is the training of nephrologists and physicians treating patients with renal diseases. These goals form the basis of the ISN “Global Outreach” (GO) programs – to build health equality worldwide and improve kidney care and prevention strategies around the globe by making the knowledge and experience of the developed world accessible to kidney doctors and other specialists in emerging countries. Children are a particularly vulnerable population. Unacceptable delays in the recognition and treatment of (severe) renal disease due to lack of access to basic and comprehensive care often leads to preventable, acute and chronic adverse effects caused by malfunctioning kidneys. Kidney disease during infancy to adolescence due to (intrauterine) malnutrition, genetic inheritance, nephrotoxic agents or poorly treated primary diseases has devastating, often irreversible, effects on bone health, growth and intellectual development. Disparities in preventive and curative or supportive care arise from the lack of knowledgeable health care professionals, lack of (public) health care resources and lack of individual financial means to initiate and support therapies that are taken for granted in more affluent countries. To come to grips with the global challenge of rising numbers of patients with renal diseases, the ISN created the Sister Renal Center program pairing emerging and supporting (established) centers across all continents. As the chair and ardent supporter of our GO programs, I am proud to witness the involvement of dedicated pediatric nephrologists in this endeavor. This Manual is the collaborative product of one of the few and successful pediatric sister center pairs, the Children’s Kidney Care Center at the St. John’s Medical College Hospital in Bangalore, India, and the Nephrology Division at the Montreal Children’s Hospital in Montreal, Canada. Written in a collaborative spirit, it emphasizes a global perspective. May it serve health care professionals, trainees, and physicians to improve the diagnosis and treatment of children with renal diseases worldwide. Paul N. Harden Professor of Nephrology Chair, International Society of Nephrology Sister Renal Program Oxford, UK v Foreword II Today, kidney issues dramatically impact global health, especially the health of children. In developing countries, millions of people – most of them children – die each year from diseases that are preventable and treatable. For many of these children, kidney problems represent a serious threat to their survival. Important examples include – but are not limited to – the delayed recognition and treatment for congenital abnormalities of the kidney and urinary tract, which are a major cause of chronic kidney disease and secondary health problems; severe fluid and electrolyte disturbances and acute kidney injury due to diarrhea, the #2 cause of death in children worldwide; infants with low birth weight in low income groups due to malnutrition and lack of prenatal care, with an increased risk of small kidneys, hypertension in adolescence and chronic kidney disease; HIV associated nephropathy, particularly in areas where highly active antiretroviral therapy is not accessible. A major goal of the International Pediatric Nephrology Association (IPNA) is to reach physicians throughout the world through educational and fellowship programs and enable them to care for children with kidney diseases. This Manual of Pediatric Nephrology is designed to give pediatrician and general physicians as well as trainees and other health care professionals a quick and practical approach to the diagnosis and treatment of children with different types of kidney diseases. The well-structured text is easy to read and covers all important areas in the field. On behalf of IPNA, I welcome this new manual as a valuable resource for practitioners and trainees in (pediatric) nephrology alike. Its appeal lies in the combined perspective provided by experienced nephrologists from different continents that takes into account the realities in emerging countries. It corresponds well to IPNA’s goals to “work to disseminate knowledge about kidney disease in children in the areas where care is needed most”. Isidro B, Salusky, M.D. Secretary General IPNA Distinguished Professor of Pediatrics David Geffen School of Medicine at UCLA Los Angeles, CA, USA vii Preface This manual does not want to replace standard textbooks of nephrology or pediatric nephrology. Rather, it offers a first, quick and practical approach for the care of children with kidney disease. It is meant to serve practitioners, trainees, pediatricians, general physicians, and other health care professionals. The manual is the product of a long-lasting collaboration between two pediatric nephrology units, the Children’s Kidney Care Center at the Saint John’s Medical College Hospital (St-John’s) in Bangalore, India, and the Division of Nephrology at the Montreal Children’s Hospital (MCH) in Montreal, Canada. The authors approached each chapter with the practical reality in emerging and resource-poor countries in mind. We hope that this concept renders the manual useful and versatile in a variety of settings and diverse medical practices. To this end, we are proud of the manual’s endorsement by the International Society of Nephrology Global Outreach initiative and the support by the International Pediatric Nephrology Association. It is unavoidable in a practically oriented, abbreviated book like ours that topics are missing or only mentioned cursorily. The editors purposefully focused on relatively frequent and clinically important disorders and practical, mainly diagnostic and therapeutic, aspects. Detailed pathological and pathophysiological reviews were omitted to keep the text short and lean. The reader is strongly encouraged to seek in-depth information in more comprehensive textbooks. The editors appreciate specific suggestions that help improve the utility and ameliorate the shortcomings of this manual. Please, email the editors your comments. We hope that the global pediatric nephrology community will adopt the manual as a dynamic, interactive project with input from trainees and practitioners alike. The editors have an enduring commitment to research and education in pediatric renal diseases locally and internationally with the ultimate goal of improving children’s access to renal care – irrespective of monetary resources, societal status, or geography. They are connected through a long-standing collaboration within the ISN Sister Renal Center program. Kishore Phadke, Professor of Pediatrics and Nephrology, Director of the Children’s Kidney Care Center and former Chair of Pediatrics at St-John’s was the driving force behind this manual. He established a successful, internationally recognized Pediatric Nephrology Training Program, whose trainees now work across India, and in Sri Lanka, Bangladesh and the Middle ix x Preface East. Paul R. Goodyer, McGill Professor, former Director of the Division of Nephrology at the MCH, is a successful investigator of kidney development and genetic renal diseases with a number of international collaborations, including St-John’s. Martin Bitzan, Associate Professor of Pediatrics and Pediatric Nephrology and current Director of the Division of Nephrology and of the Pediatric Nephrology Training program at the MCH has a focused research and clinical interests in glomerular and systemic renal diseases and international education in pediatric nephrology. The editors wish to acknowledge their collaborators from the St-John’s and the MCH. Their input and contributions were instrumental in the genesis of this manual. Not all of them are named individually. “Thank you” also to the associate/ publishing editors and “midwives” of this oeuvre, Sandra Lesny, Rosmarie Unger, Marion Krämer, and Dr. Tobias Kemme from Springer, and project manager Steven Muthu Raj Joe Arun from SPi Global, India, for their patient professionalism. We apologize to our families, particularly our spouses, for enduring long absences and distractions associated with this manual and with our clinical work. Last, but not least, we thank our students and trainees for reminding us of our educational mission, and our patients and their families, who taught us so much and who continue inspiring us. Bangalore, India Montreal, Canada Montreal, Canada Kishore Phadke Paul Goodyer Martin Bitzan Contents 1 Evaluation of Renal Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Paul Goodyer and Kishore Phadke 1 2 Fluids, Electrolytes, and Acid–Base Disorders . . . . . . . . . . . . . . . . . . . Anil Vasudevan and Kishore Phadke 65 3 Glomerular Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141 Martin Bitzan 4 Tubular Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231 Paul Goodyer 5 Cystic Renal Diseases. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 249 Indra Gupta and Martin Bitzan 6 Issues Related to Pediatric Urology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 263 Arpana A. Iyengar, John-Paul Capolicchio, Mohamed El-Sherbiny, Roman Jednak, and Konrad M. Szymanski 7 Hypertension. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 315 Martin Bitzan 8 Acute Kidney Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349 Indra Gupta and Martin Bitzan 9 Chronic Kidney Disease (CKD) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 373 Arpana A. Iyengar and Bethany J. Foster 10 Chronic Dialysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 401 Kishore Phadke, Martin Bitzan, and Sudarshan Shetty 11 Renal Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 429 Kishore Phadke, Paul Goodyer, and Arpana A. Iyengar 12 Oncology and Kidney. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 445 Anand Prakash and David Mitchell 13 The Kidney and the Tropics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 461 Anita Shet xi xii Contents 14 HIV and the Kidney. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 479 Anita Shet 15 Newborn and the Kidney. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493 P.N. Suman Rao 16 Nephrotoxicity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 517 Anil Vasudevan and Nivedita Kamath 17 Appendix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 533 Jyoti Sharma and Anil Vasudevan Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 611 Abbreviations mg ABG ABPM ACE ADH ADH rickets ADPKD AG AKI ANA ANCA anti-DsDNA anti-GBM antibodies APLA ARB ARH rickets ARPKD ATN ATN AVP BE BMD BP BUN BUN CAPD CCPD CDI CHD CKD Cl CNI COX-2 Cr Micrograms Arterial blood gases Ambulatory blood pressure monitoring Angiotensin-converting enzyme Anti diuretic hormone Autosomal dominant hypophosphatemic rickets Autosomal dominant polycystic kidney disease Anion gap Acute kidney injury Antinuclear antibodies Anti neutrophilic cytoplasmic antibodies Anti-deoxyribonuclease B Anti glomerular basement antibodies Antiphospholipid antibodies Angiotensin receptor blocker Autosomal recessive hypophosphatemic rickets Autosomal recessive polycystic kidney disease Acute tubular necrosis Acute interstitial nephritis Arginine vasopressin Base excess Bone mineral density Blood pressure Blood urea nitrogen Blood urea nitrogen Continuous ambulatory peritoneal dialysis Continuous cycling peritoneal dialysis Central diabetes insipidus Coronary heart disease Chronic kidney disease Chloride Calcineurin inhibitors Cyclooxygenase type 2 Creatinine xiii xiv Cr CTA CVD D5 DABP DBP DDS DIC DKA DMSA DRCG DXA e GFR e.g. EKG ELBW ESRD ESWL GBM GCD GFR GI gm HAART HBPM HBPM HCO3 HIVAN HTN HUS IRIS kDa kDa KIM-1 LDH LVH mL MA MAG 3 MCKD mEq mg MH MNE mosm Abbreviations Creatinine clearance CT angiography Cerebrovascular disease 5 % dextrose Diastolic ambulatory blood pressure Diastolic blood pressure Dialysis equilibrium syndrome Disseminated intravascular coagulation Diabetic ketoacidosis Dimercaptosuccinic acid Direct nuclide cystography Dual-energy X-ray absorptiometry Estimated GFR For example Electrocardiogram Eextremely low birth weight End-stage renal disease Extracorporeal lithotripsy Glomerular basement membrane Glomerulocystic disease Glomerular filtration rate Gastrointestinal Gram Highly active antiretroviral therapy Hemoglobin Home blood pressure monitoring Bicarbonate HIV-associated nephropathy Hypertension Hemolytic uremic syndrome Immune reconstitution inflammatory syndrome Kilodaltons Potassium Kidney injury molecule −1 Lactic dehydrogenase Left ventricular hypertrophy Milliliters Metabolic acidosis Mercaptoacetyltriglycine 3 Multicystic kidney disease Milliequivalent Milligrams Masked hypertension Monosymptomatic enuresis Milliosmoles Abbreviations xv MRA MRV Na NDI NGAL NHBPEP NPHP NRTI NS ORS PCNL PCO2 PET PH PI PO2 PRA PRA RBC rhGH RPGN RTA RTA SABP SBP SIAD TIN TmP TRP TTKG UAG UAG UOG UTI VACTERL VATER VCUG VUR WBC XLH rickets MR angiography MR venography Sodium Nephrogenic diabetes insipidus Neutrophil gelatinase-associated lipocalin National high blood pressure education program Nephronophthisis Nucleoside reverse transcriptase inhibitor Normal saline Oral rehydration solution Percutaneous nephrolithotomy Partial pressure of carbon dioxide Peritoneal equilibration test Primary hyperoxaluria Protease inhibitors Partial pressure of oxygen Panel reactive antibodies Plasma renin activity Red blood cell Human recombinant growth hormone Rapidly progressive glomerulonephritis Renal tubular acidosis Renal tubular acidosis Systolic ambulatory blood pressure Systolic blood pressure Syndrome of inappropriate antidiuresis Tubulointerstitial nephritis Renal tubular phosphate threshold maximum Tubular reabsorption of phosphate Transtubular potassium gradient Urine anion gap Urinary anion gap Urine osmolal gap Urinary tract infection Vertebral, anorectal, cardiac, tracheoesophageal, renal, limb defects Vertebral, anorectal, tracheal, esophageal, renal Voiding cystourethrography Vesicoureteral reflux White blood cell X-linked hypophosphatemic rickets
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