Free ebooks ==> www.Ebook777.com www.Ebook777.com Free ebooks ==> www.Ebook777.com Core Concepts in the Disorders of Fluid, Electrolytes and Acid-Base Balance www.Ebook777.com David B Mount • Mohamed H Sayegh Ajay K Singh Editors Core Concepts in the Disorders of Fluid, Electrolytes and Acid-Base Balance Free ebooks ==> www.Ebook777.com Editors David B Mount, MD Renal Division VA Boston Healthcare System Brigham and Women’s Hospital Harvard Medical School Boston, MA, USA Mohamed H Sayegh, MD Renal Division Brigham and Women’s Hospital Harvard Medical School Boston, MA, USA Ajay K Singh, MB, FRCP (UK) Renal Division Brigham and Women’s Hospital Harvard Medical School Boston, MA, USA ISBN 978-1-4614-3769-7 ISBN 978-1-4614-3770-3 (eBook) DOI 10.1007/978-1-4614-3770-3 Springer New York Heidelberg Dordrecht London Library of Congress Control Number: 2012941302 © Springer Science+Business Media New York 2013 This work is subject to copyright All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed Exempted from this legal reservation are brief excerpts in connection with reviews or scholarly analysis or material supplied specifically for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work Duplication of this publication or parts thereof is permitted only under the provisions of the Copyright Law of the Publisher’s location, in its current version, and permission for use must always be obtained from Springer Permissions for use may be obtained through RightsLink at the Copyright Clearance Center 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) www.Ebook777.com To my wife and children; Erika, Julia, and Nicholas –DBM Preface Fluid, electrolyte, and acid–base disorders are central to the day-to-day practice of almost all areas of patient-centered medicine, both medical and surgical Despite the steep learning curve for trainees, the underlying pathophysiology and/or management is often viewed as “settled,” with the perception that there is little in this field that is new However, there have been significant recent developments in all aspects of these important disorders This book encompasses these new findings in comprehensive reviews of both pathophysiology and clinical management, meant for both the nephrologist and the nonspecialist physician or medical trainee Virtually every subject in this textbook has witnessed major developments in the last decade New pathophysiology includes the molecular identification of “pendrin” (SLC26A4) as the apical Cl−/HCO3− exchanger in b[beta]-intercalated cells [1, 2]; this transporter functions in distal chloride and bicarbonate transport, with evolving roles in the pathophysiology of hypertension and metabolic alkalosis A host of previously uncharacterized genetic tubular disorders have recently yielded to molecular genetics, with major impact of this gene identification on the understanding of renal physiology and pathophysiology In particular, the identification in 2001 [3] of causative mutations in the WNK1 (With No K/Lysine) and WNK4 kinases in familial hypertension with hyperkalemia (Gordon’s syndrome) led to a still-evolving cascade of insight into the role of these and associated signaling proteins in the coordination of aldosterone-dependent and aldosterone-independent regulation of distal potassium, sodium, and chloride transport [4] Characterization of multiple genes for familial hypomagnesemia led to the identification of novel magnesium transport pathways [5] and to the identification of cell-associated epidermal growth factor as a major paracrine regulator of distal tubular magnesium transport [6] Finally, characterization of FGF23 (fibroblast growth factor-23) as the disease gene for autosomal dominant hypophosphatemic rickets [7] uncovered a major new regulatory hormone in calcium and phosphate balance [8, 9] At the clinical level, the spectrum of the acquired causes of electrolyte disorders continues to expand Examples include hypokalemia due to the activation of colonic potassium secretion in Ogilvie’s syndrome [10], and hypomagnesemia, with or without associated hypokalemia, after treatment with the EGF antagonist cetuximab [6, 11, 12] The management of electrolyte disorders has also evolved considerably in the last decade Nowhere is this more vii Preface viii evident than in hyponatremia, with the recent availability of vasopressin antagonists [13, 14] and the increasing familiarity with relowering of serum sodium concentration in patients who have corrected too quickly [15] The integrated analysis and management of fluid, electrolyte, and acid– base disorders can be a daunting challenge, especially for trainees With this in mind, the last chapter includes ten real-life clinical vignettes that provide a step-by-step analysis of the pathophysiology, differential diagnosis, and management of selected clinical problems Boston, MA, USA David B Mount Mohamed H Sayegh Ajay K Singh References Royaux IE, Wall SM, Karniski LP, et al Pendrin, encoded by the Pendred syndrome gene, resides in the apical region of renal intercalated cells and mediates bicarbonate secretion Proc Natl Acad Sci U S A 2001;98:4221–6 Verlander JW, Hassell KA, Royaux IE, et al Deoxycorticosterone upregulates PDS (Slc26a4) in mouse kidney: role of pendrin in mineralocorticoid-induced hypertension Hypertension 2003;42:356–62 Wilson FH, Disse-Nicodeme S, Choate KA, et al Human hypertension caused by mutations in WNK kinases Science 2001;293:1107–12 Welling PA, Chang YP, Delpire E, Wade JB Multigene kinase network, kidney transport, and salt in essential hypertension Kidney Int 2010;77:1063–9 Schlingmann KP, Weber S, Peters M, et al Hypomagnesemia with secondary hypocalcemia is caused by mutations in TRPM6, a new member of the TRPM gene family Nat Genet 2002;31:166–70 Groenestege WM, Thebault S, van der Wijst J, et al Impaired basolateral sorting of pro-EGF causes isolated recessive renal hypomagnesemia J Clin Invest 2007; 117:2260–7 Consortium A Autosomal dominant hypophosphataemic rickets is associated with mutations in FGF23 The ADHR Consortium Nat Genet 2000;26:345–8 Wolf M Forging forward with 10 burning questions on FGF23 in kidney disease J Am Soc Nephrol 2010;21:1427–35 Alon US Clinical practice Fibroblast growth factor (FGF)23: a new hormone Eur J Pediatr 2011;170:545–54 10 Blondon H, Bechade D, Desrame J, Algayres JP Secretory diarrhoea with high faecal potassium concentrations: a new mechanism of diarrhoea associated with colonic pseudo-obstruction? Report of five patients Gastroenterol Clin Biol 2008;32:401–4 11 Cao Y, Liao C, Tan A, Liu L, Gao F Meta-analysis of incidence and risk of hypomagnesemia with cetuximab for advanced cancer Chemotherapy 2010;56:459–65 12 Cao Y, Liu L, Liao C, Tan A, Gao F Meta-analysis of incidence and risk of hypokalemia with cetuximab-based therapy for advanced cancer Cancer Chemother Pharmacol 2010;66:37–42 13 Schrier RW, Gross P, Gheorghiade M, et al Tolvaptan, a selective oral vasopressin V2-receptor antagonist, for hyponatremia N Engl J Med 2006;355:2099–112 14 Zeltser D, Rosansky S, van Rensburg H, Verbalis JG, Smith N Assessment of the efficacy and safety of intravenous conivaptan in euvolemic and hypervolemic hyponatremia Am J Nephrol 2007;27:447–57 15 Perianayagam A, Sterns RH, Silver SM, et al DDAVP is effective in preventing and reversing inadvertent overcorrection of hyponatremia Clin J Am Soc Nephrol 2008; 3:331–6 Free ebooks ==> www.Ebook777.com Contents The Physiology of Water Homeostasis Jeff M Sands, David B Mount, and Harold E Layton Disorders of Water Metabolism Joshua M Thurman and Tomas Berl 29 Potassium and the Dyskalemias Alan Segal 49 Disorders of Calcium, Phosphate, and Magnesium Metabolism Ali Hariri, David B Mount, and Ashghar Rastegar 103 Management of Fluid and Electrolyte Abnormalities in Children John T Herrin 147 Diuretic Therapy Arohan R Subramanya and David H Ellison 171 Renal Acidification Mechanisms I David Weiner, Jill W Verlander, and Charles S Wingo 203 Core Concepts and Treatment of Metabolic Acidosis Michael R Wiederkehr and Orson W Moe 235 Metabolic Alkalosis F John Gennari 275 10 Respiratory Acid–Base Disorders Biff F Palmer 297 11 Mixed Acid–Base Disorders Jeffrey A Kraut and Ira Kurtz 307 12 Case Studies in Electrolyte and Acid–Base Disorders David B Mount 327 Index 363 ix www.Ebook777.com ... www.Ebook777.com Core Concepts in the Disorders of Fluid, Electrolytes and Acid -Base Balance www.Ebook777.com David B Mount • Mohamed H Sayegh Ajay K Singh Editors Core Concepts in the Disorders of Fluid, Electrolytes. .. a still-evolving cascade of insight into the role of these and associated signaling proteins in the coordination of aldosterone-dependent and aldosterone-independent regulation of distal potassium,... identification on the understanding of renal physiology and pathophysiology In particular, the identification in 2001 [3] of causative mutations in the WNK1 (With No K/Lysine) and WNK4 kinases in familial