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Clinical Decisions in Pediatric Nephrology Farahnak Assadi, M.D Clinical Decisions in Pediatric Nephrology Farahnak Assadi, M.D. Clinical Decisions in Pediatric Nephrology A Problem-solving Approach to Clinical Cases Farahnak Assadi, M.D. Professor of Pediatrics Director, Section of Pediatric Nephrology Rush University Medical Center Chicago, Illinois ISBN-13: 978-0-387-74601-2 e-ISBN-13: 978-0-387-74602-9 Library of Congress Control Number: 2007933400 c 2008 Springer Science+Business Media, LLC All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, LLC, 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. Printed on acid-free paper. 987654321 springer.com This book is dedicated to the individuals who have given meaning to my life: • To the memory of my mother whose hon- esty and fairness served as a model that I have tried to emulate. Her continued love has allowed me to maintain a frame of ref- erence which has assured my happiness • To my father who remains, in his later years, a source of inspiration to three gen- erations of loving progeny • To my wife, Nassrin, for her support, patience, understanding and great sacri- fices in order for me to pursue my career • To my children, Ladan and Ramin–and to my grandchildren, Emily, Mathew, Caro- line, and Christian–who provide my hope for the future • To all medical students, residents, and fel- lows who have enriched my life • To all the children for whom I have cared, who always taught me so much Preface Over the last twenty-five years of teaching, I have found the evidence-based medicine approach to be very effective in teaching clinical nephrology to students of health professions at all stages of their training. For this reason, I believe that the time has come to undertake the task of publishing a comprehensive book dealing with common renal disorders as they present in clinical practice. This book is designed to expand the clinical knowledge and experience of resi- dents in training and the practicing clinician. The format of case reports will illumi- nate the basic principles and pathophysiology of diseases of the kidney and define diagnosis and treatment. The selected case reports focus on the essential aspects of the patient’s presentation findings and managements needed to assist in the differen- tial diagnosis. They develop a process of logical questioning from the presentation of the signs and symptoms and laboratory data, and they are presented in the way in which our patients come to us with their signs and symptoms or are referred to us by our colleagues. Each question is followed by a detailed discussion that reviews recent publications and translates emerging areas of science into data that is useful at the bedside. The content is an evidence-based medicine approach, resulting in improved quality, safety, and cost-effectiveness of patient care. An update bibliog- raphy will conclude each set of clinical cases. This format will help readers stay abreast of developing areas of clinical nephrology. I am appreciative of the work of the medical editors of Springer Publishers, Inc., for their contributions to this endeavor and all those persons who have dedicated their skills, intelligence, and work to help make this a book of outstanding editorial quality. Farahnak Assadi, M.D. Professor of Pediatrics Director, Section of Nephrology Rush University Medical Center Chicago, Illinois vii Acknowledgements Many of the individuals to whom I am most indebted are only indirect contributors to this book. They are the people who saw some glimmer of hope in the author early in his career and nurtured him in what has been the most rewarding life in pedi- atrics and nephrology. From my student and residency years, Professors Moham- mad Gharib and Arthur Hervada were great teachers and always highly supportive when my knowledge had profound limitations. During my fellowship training at the University of Pennsylvania and Children’s Hospital of Philadelphia, Michael E. Norman established the groundwork for my subsequent work in nephrology. He provided an intellectual environment and I have always been grateful for his efforts on my behalf. In the beginning of my career at the University of Illinois, one could hardly have had a better mentor than Professor Ira Rosenthal. After moving to Thomas Jefferson University, I received extraordinary help from Michael Norman. He supported my efforts to establish the core of an outstanding nephrology program at Dupont Hospital for Children. Leading the Division of Nephrology at Rush University Medical Center has been one of the greatest fortunes of my life. Samuel Gotoff and Kenneth Boyer have made it enjoyable to come to work each and every day for the past several years. Our residents at Rush have enriched the clinical experience immensely. Farahnak Assadi ix Contents 1 Fluid and Electrolyte Disorders .................................. 1 2 Acid-base Disturbances ......................................... 69 3 Disorders of Divalent Ion Metabolism ............................ 97 4 Nephrolithiasis .................................................125 5 Hypertension ..................................................145 6 Acute Renal Failure ............................................167 7 Hereditary Nephritis and Genetic Disorders .......................201 8 Glomerular, Vascular, and Tubulo-Interstitial Diseases .............237 9 Chronic Kidney Disease .........................................287 10 Renal Osteodystrophy ..........................................313 11 End-Stage Renal Disease and Dialysis ............................337 12 Transplantation ................................................353 Index .............................................................377 xi Chapter 1 Fluid and Electrolyte Disorders CASE 1 A 16-year old female is brought into the hospital in a stuporous state. No history is initially obtainable. The physical examination is unremarkable except for the abnor- mal mental status. There are no obvious signs of volume depletion or expansion. The BP is 100/59 mmHg. Laboratory data reveal serum sodium 102 mEq/l, potassium 2.5 mEq/l, chloride 66 mEq/l, bicarbonate 32mEq/l, BUN 9 mg/dl, and creatinine 0.4 mg/dl. Urine sodium is 130 mEq/l, potassium 61 mEq/l, chloride 107 mEq/l and osmolality 467 mOsml/kg. What is the most likely diagnosis? A. Primary hyperaldosteronism B. Diuretic abuse C. Bartter’s syndrome D. SIADH E. Excessive emesis The correct answer is B. The differential diagnoses of hyponatremia, hypokalemia, and metabolic alkalosis with a high urine sodium and chloride concentrations is highly suggestive of diuretic abuse. Hypokalemia essentially excludes SIADH. Neither hyperaldosteronism nor Bartter’s syndrome causes marked hyponatremia. The high urine chloride excludes vomiting, suggesting the patient has surreptitious diuretic abuse. The urine sodium plus potassium is well above that in the plasma; thus, solute is being lost in excess of water, which will directly lower the plasma sodium concentration. References Chung HM, Kluge R, Schrier RW, Anderson RJ (1987) Clinical assessment of extracellular fluid volume in hyponatremia 83:905–908 Assadi F (1993) Hyponatremia. Pediatr Nephrol 7:503–505 Fichman MP, Vorherr H, Kleeman CR, et al. (1971) Diuretic-induced hyponatremia. Ann Intern Med 75:853–563 F. Assadi, Clinical Decisions in Pediatric Nephrology. 1 C Springer 2008 2 Clinical Decisions in Pediatric Nephrology Can you estimate the urine pH in this patient? A. Acid pH B. Alkaline pH The correct answer is B. The urine pH should be alkaline; the anion gap in the urine is positively charged [(Na+ + K+) > Cl−]. This is probably due to bicarbonate excretion in an attempt to correct the metabolic alkalosis. Reference Rose BD, Post TW (2001) Clinical pathology of acid-base and electrolyte disorders, 5th ed, McGraw-Hill, New York, pp 699–710 What would your initial therapy be? A. Administration of hypertonic saline alone B. Combination therapy with hypertonic saline and potassium chloride C. Administration of potassium chloride alone D. Combination therapy with hpertonic saline, potassium chloride and potassium sparing diuretic The correct answer is C. Potassium chloride alone will correct the hypokalemia and metabolic alkalosis and raise the plasma sodium concentration toward normal; as potassium enters cells, sodium will leave to maintain electroneutrality, thereby correcting the hyponatremia. The administration of large amounts of potassium (4 mEq/kg) over the first 24 hours) can raise the plasma sodium concentration by 10 mEq/l over the first day, which is the maximum desired rate of correction of the hyponatremaia. If this were ignored and hypertonic saline also given, overly rapid correction would ensue. References Berl T, Linas SL, Aisenbrey GA, Anderson RJ (1977) On the mechanism of polyuria in potassium depletion: The role of polydipsia. J Clin Invest 60:620–625 Robertson GL, Shelton RL, Athar S (1976) The osmoregulation of vasopressin. Kidney Int 10:25–37 CASE 2
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