(BQ) Part 1 book Harrison''s nephrology and acid-base disorders presents the following contents: Introduction to the renal system, alterations of renal function and electrolytes, acute kidney injury and chronic renal failure.
2nd Edition HARRISON’S TM Nephrology and Acid-Base Disorders Derived from Harrison’s Principles of Internal Medicine, 18th Edition Editors Dan L Longo, md Professor of Medicine, Harvard Medical School; Senior Physician, Brigham and Women’s Hospital; Deputy Editor, New England Journal of Medicine, Boston, Massachusetts Dennis L Kasper, md William Ellery Channing Professor of Medicine, Professor of Microbiology and Molecular Genetics, Harvard Medical School; Director, Channing Laboratory, Department of Medicine, Brigham and Women’s Hospital, Boston, Massachusetts J Larry Jameson, md, phd Robert G Dunlop Professor of Medicine; Dean, University of Pennsylvania School of Medicine; Executive Vice-President of the University of Pennsylvania for the Health System, Philadelphia, Pennsylvania Anthony S Fauci, md Chief, Laboratory of Immunoregulation; Director, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland Stephen L Hauser, md Robert A Fishman Distinguished Professor and Chairman, Department of Neurology, University of California, San Francisco, San Francisco, California Joseph Loscalzo, md, phd Hersey Professor of the Theory and Practice of Medicine, Harvard Medical School; Chairman, Department of Medicine; Physician-in-Chief, Brigham and Women’s Hospital, Boston, Massachusetts 2nd Edition HARRISON’S TM Nephrology and Acid-Base Disorders EditorS J Larry Jameson, MD, PhD Robert G Dunlop Professor of Medicine; Dean, University of Pennsylvania School of Medicine; Executive Vice-President of the University of Pennsylvania for the Health System Philadelphia, Pennsylvania Joseph Loscalzo, MD, PhD Hersey Professor of the Theory and Practice of Medicine, Harvard Medical School; Chairman, Department of Medicine; Physician-in-Chief, Brigham and Women’s Hospital Boston, Massachusetts New York Chicago San Francisco Lisbon London Madrid Mexico City Milan New Delhi San Juan Seoul Singapore Sydney Toronto Copyright © 2013 by McGraw-Hill Education, LLC All rights reserved Except as permitted under the United States Copyright Act of 1976, no part of this publication may be reproduced or distributed in any form or by any means, or stored in a database or retrieval system, without the prior written permission of the publisher ISBN: 978-0-07-181497-3 MHID: 0-07-181497-3 The material in this eBook also appears in the print version of this title: ISBN: 978-0-07-181496-6, MHID: 0-07-181496-5 All trademarks are trademarks of their respective owners Rather than put a trademark symbol after every occurrence of a trademarked name, we use names in an editorial fashion only, and to the benefit of the trademark owner, with no intention of infringement of the trademark Where such designations appear in this book, they have been printed with initial caps McGraw-Hill Education eBooks are available at special quantity discounts to use as premiums and sales promotions, or for use in corporate training programs To contact a representative please e-mail us at bulksales@mcgraw-hill.com Dr Fauci’s work as an editor and author was performed outside the scope of his employment as a U.S government employee This work represents his personal and professional views and not necessarily those of the U.S government TERMS OF USE This is a copyrighted work and McGraw-Hill Education, LLC and its licensors reserve all rights in and to the work Use of this work is subject to these terms Except as permitted under the Copyright Act of 1976 and the right to store and retrieve one copy of the work, you may not decompile, disassemble, reverse engineer, reproduce, modify, create derivative works based upon, transmit, distribute, disseminate, sell, publish or sublicense the work or any part of it without McGraw-Hill Education’s prior consent You may use the work for your own noncommercial and personal use; any other use of the work is strictly prohibited Your right to use the work may be terminated if you fail to comply with these terms THE WORK IS PROVIDED “AS IS.” McGRAW-HILL EDUCATION AND ITS LICENSORS MAKE NO GUARANTEES OR WARRANTIES AS TO THE ACCURACY, ADEQUACY OR COMPLETENESS OF OR RESULTS TO BE OBTAINED FROM USING THE WORK, INCLUDING ANY INFORMATION THAT CAN BE ACCESSED THROUGH THE WORK VIA HYPERLINK OR OTHERWISE, AND EXPRESSLY DISCLAIM ANY WARRANTY, EXPRESS OR IMPLIED, INCLUDING BUT NOT LIMITED TO IMPLIED WARRANTIES OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE McGraw-Hill Education and its licensors not warrant or guarantee that the functions contained in the work will meet your requirements or that its operation will be uninterrupted or error free Neither McGraw-Hill Education nor its licensors shall be liable to you or anyone else for any inaccuracy, error or omission, regardless of cause, in the work or for any damages resulting therefrom McGraw-Hill Education has no responsibility for the content of any information accessed through the work Under no circumstances shall McGraw-Hill Education and/or its licensors be liable for any indirect, incidental, special, punitive, consequential or similar damages that result from the use of or inability to use the work, even if any of them has been advised of the possibility of such damages This limitation of liability shall apply to any claim or cause whatsoever whether such claim or cause arises in contract, tort or otherwise Contents Contributors vii 11 Chronic Kidney Disease 123 Joanne M Bargman, Karl Skorecki Preface ix 12 Dialysis in the Treatment of Renal Failure 141 Kathleen D Liu, Glenn M Chertow Section I Introduction to the Renal System 13 Transplantation in the Treatment of Renal Failure 148 Anil Chandraker, Edgar L Milford, Mohamed H Sayegh Cellular and Molecular Biology of the Kidney Alfred L George, Jr., Eric G Neilson 14 Infections in Kidney Transplant Recipients 158 Robert Finberg, Joyce Fingeroth Adaption of the Kidney to Renal Injury 14 Raymond C Harris, Eric G Neilson Section IV Section II Glomerular and Tubular Disorders Alterations of Renal Function and Electrolytes 15 Glomerular Diseases 162 Julia B Lewis, Eric G Neilson Azotemia and Urinary Abnormalities 22 Julie Lin, Bradley M Denker 16 Polycystic Kidney Disease and Other Inherited Tubular Disorders 189 David J Salant, Craig E Gordon Atlas of Urinary Sediments and Renal Biopsies 32 Agnes B Fogo, Eric G Neilson 17 Tubulointerstitial Diseases of the Kidney 205 Laurence H Beck, David J Salant Acidosis and Alkalosis 43 Thomas D DuBose, Jr Fluid and Electrolyte Disturbances 56 David B Mount Section V Renal Vascular Disease Hypercalcemia and Hypocalcemia 81 Sundeep Khosla 18 Vascular Injury to the Kidney 218 Stephen C Textor, Nelson Leung Hyperuricemia and Gout 85 Christopher M Burns, Robert L Wortmann, H Ralph Schumacher, Lan X Chen 19 Hypertensive Vascular Disease 228 Theodore A Kotchen Nephrolithiasis 95 John R Asplin, Fredric L Coe, Murray J Favus Section VI Urinary Tract Infections and Obstruction Section III 20 Urinary Tract Infections, Pyelonephritis, and Prostatitis 254 Kalpana Gupta, Barbara W Trautner Acute Kidney Injury and Chronic Renal Failure 10 Acute Kidney Injury 104 Sushrut S Waikar, Joseph V Bonventre v Contents vi 21 Urinary Tract Obstruction 265 Julian L Seifter Section VII Appendix Laboratory Values of Clinical Importance 281 Alexander Kratz, Michael A Pesce, Robert C Basner, Andrew J Einstein Cancer of the Kidney and Urinary Tract Review and Self-Assessment 299 Charles Wiener, Cynthia D Brown, Anna R Hemnes 22 Bladder and Renal Cell Carcinomas 272 Howard I Scher, Robert J Motzer Index 313 CONTRIBUTORS Numbers in brackets refer to the chapter(s) written or co-written by the contributor Andrew J Einstein, MD, PhD Assistant Professor of Clinical Medicine, Columbia University College of Physicians and Surgeons; Department of Medicine, Division of Cardiology, Department of Radiology, Columbia University Medical Center and New YorkPresbyterian Hospital, New York, New York [Appendix] John R Asplin, MD Medical Director, Litholink Corporation, Chicago, Illinois [9] Joanne M Bargman, MD, FRCPC Professor of Medicine, University of Toronto; Staff Nephrologist, University Health Network; Director, Home Peritoneal Dialysis Unit, and Co-Director, Renal Rheumatology Lupus Clinic, University Health Network, Toronto, Ontario, Canada [11] Murray J Favus, MD Professor, Department of Medicine, Section of Endocrinology, Diabetes, and Metabolism; Director, Bone Program, University of Chicago Pritzker School of Medicine, Chicago, Illinois [9] Robert C Basner, MD Professor of Clinical Medicine, Division of Pulmonary, Allergy, and Critical Care Medicine, Columbia University College of Physicians and Surgeons, New York, New York [Appendix] Robert Finberg, MD Chair, Department of Medicine, University of Massachusetts Medical School, Worcester, Massachusetts [14] Laurence H Beck, Jr., MD, PhD Assistant Professor of Medicine, Boston University School of Medicine, Boston, Massachusetts [17] Joyce Fingeroth, MD Associate Professor of Medicine, Harvard Medical School, Boston, Massachusetts [14] Joseph V Bonventre, MD, PhD Samuel A Levine Professor of Medicine, Harvard Medical School; Chief, Renal Division; Chief, BWH HST Division of Bioengineering, Brigham and Women’s Hospital, Boston, Massachusetts [10] Agnes B Fogo, MD John L Shapiro Professor of Pathology; Professor of Medicine and Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee [4] Cynthia D Brown, Md Assistant Professor of Medicine, Division of Pulmonary and Critical Care Medicine, University of Virginia, Charlottesville, Virginia [Review and Self-Assessment] Alfred L George, Jr., MD Professor of Medicine and Pharmacology; Chief, Division of Genetic Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee [1] Christopher M Burns, MD Assistant Professor, Department of Medicine, Section of Rheumatology, Dartmouth Medical School; Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire [8] Craig E Gordon, MD, MS Assistant Professor of Medicine, Boston University School of Medicine; Attending, Section of Nephrology, Boston Medical Center, Boston, Massachusetts [16] Anil Chandraker, MD, FASN, FRCP Associate Professor of Medicine, Harvard Medical School; Medical Director of Kidney and Pancreas Transplantation; Assistant Director, Schuster Family Transplantation Research Center, Brigham and Women’s Hospital; Children’s Hospital, Boston, Massachusetts [13] Kalpana Gupta, MD, MPH Associate Professor, Department of Medicine, Boston University School of Medicine; Chief, Section of Infectious Diseases, VA Boston Healthcare System, Boston, Massachusetts [20] Raymond C Harris, MD Ann and Roscoe R Robinson Professor of Medicine; Chief, Division of Nephrology, Vanderbilt University School of Medicine, Nashville, Tennessee [2] Lan X Chen, MD, PhD Penn Presbyterian Medical Center, Philadelphia, Pennsylvania [8] Glenn M Chertow, MD, MPH Norman S Coplon/Satellite Healthcare Professor of Medicine; Chief, Division of Nephrology, Stanford University School of Medicine, Palo Alto, California [12] Anna R Hemnes, Md Assistant Professor, Division of Allergy, Pulmonary, and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, Tennessee [Review and Self-Assessment] Fredric L Coe, MD Professor of Medicine, University of Chicago, Chicago, Illinois [9] Sundeep Khosla, MD Professor of Medicine and Physiology, College of Medicine, Mayo Clinic, Rochester, Minnesota [7] Bradley M Denker, MD Associate Professor, Harvard Medical School; Physician, Department of Medicine, Brigham and Women’s Hospital; Chief of Nephrology, Harvard Vanguard Medical Associates, Boston, Massachusetts [3] Theodore A Kotchen, MD Professor Emeritus, Department of Medicine; Associate Dean for Clinical Research, Medical College of Wisconsin, Milwaukee, Wisconsin [19] Thomas D DuBose, Jr., MD, MACP Tinsley R Harrison Professor and Chair, Internal Medicine; Professor of Physiology and Pharmacology, Department of Internal Medicine, Wake Forest University School of Medicine, Winston-Salem, North Carolina [5] Alexander Kratz, MD, PhD, MPH Associate Professor of Pathology and Cell Biology, Columbia University College of Physicians and Surgeons; Director, Core Laboratory, Columbia University Medical Center, New York, New York [Appendix] vii viii Contributors Nelson Leung, MD Associate Professor of Medicine, Department of Nephrology and Hypertension, Division of Hematology, Mayo Clinic, Rochester, Minnesota [18] Julia B Lewis, MD Professor, Department of Medicine, Division of Nephrology, Vanderbilt University Medical Center, Nashville, Tennessee [15] Julie Lin, MD, MPH Assistant Professor of Medicine, Harvard Medical School, Boston, Massachusetts [3] Kathleen D Liu, MD, PhD, MAS Assistant Professor, Divisions of Nephrology and Critical Care Medicine, Departments of Medicine and Anesthesia, University of California–San Francisco, San Francisco, California [12] Edgar L Milford, MD Associate Professor of Medicine, Harvard Medical School; Director, Tissue Typing Laboratory, Brigham and Women’s Hospital, Boston, Massachusetts [13] Robert J Motzer, MD Professor of Medicine, Weill Cornell Medical College; Attending Physician, Genitourinary Oncology Service, Memorial Sloan-Kettering Cancer Center, New York, New York [22] David B Mount, MD, FRCPC Assistant Professor of Medicine, Harvard Medical School, Renal Division, VA Boston Healthcare System; Brigham and Women’s Hospital, Boston, Massachusetts [6] Eric G Neilson, MD Thomas Fearn Frist Senior Professor of Medicine and Cell and Developmental Biology, Vanderbilt University School of Medicine, Nashville, Tennessee [1, 2, 4, 15] Michael A Pesce, PhD Professor Emeritus of Pathology and Cell Biology, Columbia University College of Physicians and Surgeons; Columbia University Medical Center, New York, New York [Appendix] David J Salant, MD Professor of Medicine, Boston University School of Medicine; Chief, Section of Nephrology, Boston Medical Center, Boston, Massachusetts [16, 17] Mohamed H Sayegh, MD Raja N Khuri Dean, Faculty of Medicine; Professor of Medicine and Immunology; Vice President of Medical Affairs, American University of Beirut, Beirut, Lebanon; Visiting Professor of Medicine and Pediatrics, Harvard Medical School; Director, Schuster Family Transplantation Research Center, Brigham and Women’s Hospital; Children’s Hospital, Boston, Massachusetts [13] Howard I Scher, MD Professor of Medicine, Weill Cornell Medical College; D Wayne Calloway Chair in Urologic Oncology; Chief, Genitourinary Oncology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, New York [22] H Ralph Schumacher, MD Professor of Medicine, Division of Rheumatology, University of Pennsylvania, School of Medicine, Philadelphia, Pennsylvania [8] Julian L Seifter, MD Associate Professor of Medicine, Harvard Medical School; Brigham and Women’s Hospital, Boston, Massachusetts [21] Karl Skorecki, MD, FRCP(C), FASN Annie Chutick Professor in Medicine (Nephrology); Director, Rappaport Research Institute, Technion–Israel Institute of Technology; Director, Medical and Research Development, Rambam Health Care Campus, Haifa, Israel [11] Stephen C Textor, MD Professor of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota [18] Barbara W Trautner, MD, PhD Assistant Professor, Section of Infectious Diseases, Baylor College of Medicine; The Michael E DeBakey Veterans Affairs Medical Center, Houston VA Health Services Research and Development Center of Excellence, Houston, Texas [20] Sushrut S Waikar, MD, MPH Assistant Professor of Medicine, Harvard Medical School; Brigham and Women’s Hospital, Boston, Massachusetts [10] Charles M Wiener, Md Dean/CEO Perdana University Graduate School of Medicine, Selangor, Malaysia; Professor of Medicine and Physiology, Johns Hopkins University School of Medicine, Baltimore, Maryland [Review and Self-Assessment] Robert L Wortmann, MD, FACP, MACR Professor, Department of Medicine, Dartmouth Medical School and Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire [8] PREFACE The first section of the book, “Introduction to the Renal System,” provides a systems overview, beginning with renal development, function, and physiology, as well as providing an overview of how the kidney responds to injury The integration of pathophysiology with clinical management is a hallmark of Harrison’s, and can be found throughout each of the subsequent diseaseoriented chapters The book is divided into seven main sections that reflect the scope of nephrology: (I) Introduction to the Renal System; (II) Alterations of Renal Function and Electrolytes; (III) Acute Kidney Injury and Chronic Renal Failure; (IV) Glomerular and Tubular Disorders; (V) Renal Vascular Disease; (VI) Urinary Tract Infections and Obstruction; and (VII) Cancer of the Kidney and Urinary Tract While Harrison’s Nephrology and Acid-Base Disorders is classic in its organization, readers will sense the impact of the scientific advances as they explore the individual chapters in each section Genetics and molecular biology are transforming the field of nephrology, whether illuminating the genetic basis of a tubular disorder or explaining the regenerative capacity of the kidney Recent clinical studies involving common diseases like chronic kidney disease, hypertensive vascular disease, and urinary tract infections provide powerful evidence for medical decision making and treatment These rapid changes in nephrology are exciting for new students of medicine and underscore the need for practicing physicians to continuously update their knowledge base and clinical skills Our access to information through web-based journals and databases is remarkably efficient Although these sources of information are invaluable, the daunting body of data creates an even greater need for synthesis by experts in the field Thus, the preparation of these chapters is a special craft that requires the ability to distill core information from the ever-expanding knowledge base The editors are therefore indebted to our authors, a group of internationally recognized authorities who are masters at providing a comprehensive overview while being able to distill a topic into a concise and interesting chapter We are indebted to our colleagues at McGraw-Hill Jim Shanahan is a champion for Harrison’s and these books were impeccably produced by Kim Davis We hope you find this book useful in your effort to achieve continuous learning on behalf of your patients Harrison’s Principles of Internal Medicine has been a respected information source for more than 60 years Over time, the traditional textbook has evolved to meet the needs of internists, family physicians, nurses, and other health care providers The growing list of Harrison’s products now includes Harrison’s for the iPad, Harrison’s Manual of Medicine, and Harrison’s Online This book, Harrison’s Nephrology and Acid-Base Disorders, now in its second edition, is a compilation of chapters related to kidney function Our readers consistently note the sophistication of the material in the specialty sections of Harrison’s Our goal was to bring this information to our audience in a more compact and usable form Because the topic is more focused, it is possible to enhance the presentation of the material by enlarging the text and the tables We have also included a review and self-assessment section that includes questions and answers to provoke reflection and to provide additional teaching points Renal dysfunction, electrolyte, and acid-base disorders are among the most common problems faced by the clinician The evaluation of renal function relies heavily on laboratory tests, urinalyses, and characteristics of urinary sediments Evaluation and management of renal disease also requires a broad knowledge of physiology and pathology since the kidney is involved in many systemic disorders Thus, this book considers a broad spectrum of topics including acid-base and electrolyte disorders, vascular injury to the kidney, as well as specific diseases of the kidney Kidney disorders, such as glomerulonephritis, can be a primary basis for clinical presentation More commonly, however, the kidney is affected secondary to other medical problems such as diabetes, shock, or complications from dye administration or medications As such, renal dysfunction may be manifest by azotemia, hypertension, proteinuria, or an abnormal urinary sediment, and it may herald the presence of an underlying medical disorder Renal insufficiency may also appear late in the course of chronic conditions such as diabetes, lupus, or scleroderma and significantly alter a patient’s quality of life Fortunately, intervention can often reverse or delay renal insufficiency And, when this is not possible, dialysis and renal transplant provide lifesaving therapies Understanding normal and abnormal renal function provides a strong foundation for diagnosis and clinical management Therefore, topics such as acidosis and alkalosis, fluid and electrolyte disorders, and hypercalcemia are covered here These basic topics are useful in all fields of medicine and represent a frequent source of renal consultation J Larry Jameson, MD, PhD Joseph Loscalzo, MD, PhD ix ... (SCNN1A, 12 p13; SCNN1B, SCNN1G, 16 pp12 .1) Kinases WNK -1, WNK-4 (WNK1, 12 p13; WNK4, 17 q 21. 31) Vasopressin V2 receptor (AVPR2, Xq28) Water channel, aquaporin-2 (AQP2, 12 q13) 264350 Anion exchanger -1. .. receptor (CASR, 3q13.33) Claudin -16 or paracellin -1 (CLDN16 or PCLN1, 3q27) Sodium potassium ATPase, 1- subunit (ATP1G1, 11 q23) 2 412 00 6 016 78 602023 602522 6 011 99 14 5980 248250 15 4020 Disorders Involving... Anion exchanger -1 (SLC4A1, 17 q 21. 31) Anion exchanger -1 (SLC4A1, 17 q 21. 31) Proton ATPase, 1 subunit (ATP6V1B1, 2p13.3) Proton ATPase, 11 6-kD subunit (ATP6V0A4, 7q34) 17 9800 Pseudohypoaldosteronism