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Therapy of Renal Diseases and Related Disorders, Second Edition Therapy of Renal Diseases and Related Disorders, Second Edition Edited by Wadi N. Suki The Methodist Hospital Baylor College of Medicine Houston, Texas and Shaul G. Massry Division of Nephrology University of Southern California Los Angeles, California ..... " KLUWER ACADEMIC PUBLISHERS BOSTON I DORDRECHT I LONDON Distributors for North America: Kluwer Academic Publishers 101 Philip Drive Assinippi Park Norwell, Massachusetts 02061 USA Distributors for all other countries: Kluwer Academic Publishers Group Distribution Centre Post Office Box 322 3300 AH Dordrecht, THE NETHERLANDS Library of Congress Cataloging-in-Publication Data Therapy of renal diseases and related disorders/edited by Wadi N. Suki and Shaul G. Massry.-2nd ed. p. em. Includes bibliographical references. ISBN-13: 978-1-4612-8027-9 e-ISBN-13: 978-1-4613-0689-4 DOl: 10.1 007/978-1-4613-0689-4 1. Kidneys-Diseases-Treatment. 2. Urinary organs Diseases-Treatment. I. Suki, Wadi N., 1934- II. Massry, Shaul G. [DNLM: 1. Kidney Diseases-therapy. WJ 300 T398j RC902.T49 1990 616.6' 106-dc20 DNLMIDLC for Library of Congress 90-4136 CIP Copyright © 1991 by Kluwer Academic Publishers Softcover reprint of the hardcover 2nd edition 1991 Third Printing 1993 Printed on acid-free paper. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system or transmitted in any form or by any means, mechanical, photocopying, recording, or otherwise, without the prior written permission of the publisher, Kluwer Academic Publishers, 101 Philip Drive. Assinippi Park, Norwell. Massachusetts 02061. Contents Foreword. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix Preface to the First Edition. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xi Preface to the Second Edition ....................................................................... . xiii Contributors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. xv PART ONE: DISORDERS OF FLUID, ELECTROLYTE, AND ACID-BASE BALANCE 1. Treatment of hypoo smolar and hyperosmolar states STEPHEN BRENNAN AND J. CARLOS A YUS 2. Polyuric syndromes WILLIAM P. MULDOWNEY AND MICHAEL H. HUMPHREYS. . . . . . . . . . . . . . . .. 17 3. Edematous states ARTHUR GREENBERG AND JULES B. PUSCHETT . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 27 4. Disorders of potassium metabolism JACQUES J. BOURGOIGNIE, JAMES R. OSTER, GUIDO O. PEREZ, AND DOLLIE F. GREEN. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 45 5. Disorders of calcium metabolism AARON HALABE AND ROGER A.L. SUTTON. . . . . . . . . . . . . . . . . . . .. 91 6. Disorders of magnesium metabolism THOMAS DYCKNER . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 111 7. Disorders of phosphate metabolism MOSHE LEVI AND JAMES P. KNOCHEL. . . . . . . . . . . . . . . . 121 8. Nephrolithiasis and nephrocalcinosis JOAN H. PARKS AND FREDRIC L. COE. . . . . . . . . . . . . . . . 139 9. Metabolic alkalosis SANDRA SABATINI AND NEIL A. KURTZMAN . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 159 10. Metabolic acidosis ROBERT M.A. RICHARDSON AND MITCHELL L. HALPERIN. . . . . . . . . . . . . . . .. 177 11. Diabetic ketoacidosis HORACIO J. ADROGUE, JORGE BARRERO, AND GEORGE M. DOLSON... 193 12. Renal tubular acidosis FERNANDO SANTOS, GAD KAINER, AND JAMES C.M. CHAN ............ 207 13. Respiratory acid-base disturbances GREGORIO 1. CASAR AND R. KEITH WILSON. . . . . . . . . . . . 223 14. Mixed acid-base disorders JOHN T. HARRINGTON AND NICOLAOS E. MADIAS .................. 233 15. Fluid and electrolyte abnormalities in children ADRIAN SPITZER AND RICHARD NEIBERGER . . . . .. 245 16. Fluid and electrolyte disorders in the surgical patient HERVY H. HINER, JR. AND WADI N. SUKI . . . . .. 263 17. Fluid and electrolyte disorders in the thermally injured CHARLES BAXTER .......................... 277 18. Acute renal failure DAVID M. GILLUM, JOHN D. CONGER, AND ROBERT J. ANDERSON ........ 285 PART TWO: INTRINSIC PARENCHYMAL DISEASE A. Glomerular 19. Acute glomerulonephritis and glomerulonephritis in bacterial endocarditis DAVID S. BALDWIN AND JOEL NEUGARTEN . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 305 v vi Contents 20. Nephrotic syndrome GERALD C. GROGGEL AND WAYNE A. BORDER. . . . . . . . . . . . . . . . . . . . . . . . .. 317 2l. Goodpasture's syndrome CURTIS B. WILSON . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 333 22. Hematuria and IgA nephropathy JOn OHNO ..................................................... 343 B. Tubulointerstitial 23. Urinary tract infections MARVIN FORLAND. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 349 24. Vesicoureteral reflux and reflux nephropathy PRISCILLA KINCAID-SMITH . . . . . . . . . . . . . . . . . . . . . . . . .. 363 25. Genitourinary tuberculosis JAMES E. GOW ...................................................... 387 PART THREE: RENAL INVOLVEMENT IN SYSTEMIC DISEASE 26. Systemic lupus erythematosus SUSAN L. ANDREW AND DAVID P. HUSTON. . . . . . . . . . . . . . . . . . . . . .. 395 27. Vasculiticdiseases of the kidney JAMESE. BALOW AND HOWARD A. AUSTIN III ................. 413 28. Noninflammatory vascular diseases of the kidney GARABED EKNOYAN ............................ 425 29. Thrombotic microangiopathy ELLIN LIEBERMAN. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 443 30. Renal involvement in dysproteinemias DOMINIQUE GANEVAL AND JEAN-PIERRE GRUNFELD ... 453 3l. Hyperuricemic nephropathy EDWARD R. AHRENS AND THOMAS H. STEELE . . . . . . . . . . . . . . . . . . .. 469 32. Renal disorders in liver disease MURRA Y EPSTEIN ............................................... 477 33. Renal complications of pregnancy JOHN M. DAVISON, ADRIAN I. KATZ, AND MARSHALL D. LINDHEIMER. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 495 34. Diabetic nephropathy ELI A. FRIEDMAN. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 533 PART FOUR: HEREDITARY AND CONGENITAL DISEASES 35. Renal cystic disorders JARED J. GRANTHAM, JOANN B. RECKLING, AND SHARON L. SLUSHER. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 543 36. Renal disorders in sickle hemoglobinemia STEPHANIE LEAR AND ROBERT M. ROSA .............. 573 37. Inherited renal tubular disorders RUSSELL W. CHESNEY ......................................... . 581 PART FIVE: NEOPLASIA 38. Cancers of the kidney and urinary tract PETER T. SCARDINO AND MADELINE CANTINI ........... 593 PART SIX: CHEMICAL AND PHYSICAL INJURIES 39. Toxic nephropathies JOHN F. MAHER. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 613 40. Acute drug intoxications JAMES F. WINCHESTER. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 639 PART SEVEN: CHRONIC RENAL FAILURE A. Medical therapy 41. Prevention of progression of renal insufficiency GIUSEPPE MASCHIO, LAMBERTO OLDRIZZI, AND CARLO RUGIU . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 649 42. Renal insufficiency EBERHARD F. RITZ ........................................................ 659 43. Anesthesia and surgery in the patient with renal failure DAVID R. BEVAN. . . . . . . . . . . . . . . . . . . . . . . . . . .. 669 44. Nutritional management of the uremic patient MARKUS TESCHNER AND AUGUST HEIDLAND . . . .. 675 45. Cardiovascular complications of uremia and dialysis J. CARLOS A YUS AND R.K. KROTHAP ALLI . . . .. 697 46. Renal osteodystrophy SHAUL G. MASSRY. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 711 47. Neurologic and psychiatric disorders in renal failure SUHAIL AHMAD AND CHRISTOPHER R. BLAGG. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 719 48. Hematologic disorders in renal failure K.M. KOCH. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 733 Contents VII B. Peritoneal dialysis 49. Acute, intermittent, and cycled peritoneal dialysis JOSE A. DIAZ-BUXO .. . . . . . . . . . . . . . . . . . . . . . . . . . .. 739 50. Continuous ambulatory peritoneal dialysis ROBERT A. MACTIER AND KARL D. NOLPH . . . . . . . . . . .. 755 C. Hemodialysis 51. Dialysis access surgery GEORGE P. NOON AND H. DAVID SHORT ............................... 775 52. Dialyzers, dialysates, and water treatment N.K. MAN AND J.L. FUNCK-BRENTANO . . . . . . . . . . . . . . . .. 791 53. Membrane biocompatibility ALFRED K. CHEUNG. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 813 54. Dialysis, ultrafiltration, and hemofiltration RAYMOND C. VANHOLDER, NICOLAS H. HOENICH, AND SEVERIN M. RINGOIR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 841 55. Use of drugs in uremia and dialysis D. CRAIG BRATER ........................................... . 853 D. Transplantation 56. Donor and recipient selection STUART M. FLECHNER. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 867 57. Immunosuppression and treatment of rejection YVES F. CH. VA NRENTERGHEM. . . . . . . . . . . . . . . 887 58. Tubular and metabolic dysfunction following kidney transplantation J. WINAVER, J. GREEN, AND O. S. BEITER. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 905 59. Renal transplantation in systemic inherited and metabolic disease ELEANOR D. LEDERER AND WADIN.SUKT ............................................................................... 921 60. Complications of renal transplantation JOHN A. MURIE AND PETER J. MORRIS. . . . . . . . . . . . . . . . . . .. 943 PART EIGHT: MISCELLANEOUS 61. The catheter GRANNUM R. SANT AND EDWIN M. MEARES, JR. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 955 62. Nonsurgical management of vesicourethral dysfunction J. KEITH LIGHT. . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 969 Index............................................................................................. 975 Forewordl "Where are all these kidney patients coming from? A few perfection the study of the urinary sediment, clinically years ago we had never heard of kidney disease and now practical kidney function tests, and the natural history of a you are speaking of patients in the hundreds of thousands number of kidney diseases including glomerulonephritis. and indeed potentially millions." My reply, not meant to William Goldring, Herbert Chasis, Dana Atchley, and be grim, was "From the cemetery, Sir." This is a summary others studied the effects of hypertension, endocarditis, of some Congressional testimony lance gave on behalf of and circulatory diseases on the kidney and spawned suc extending kidney disease under Medicare. Where indeed cessive generations of alert clinical investigators, who be were all the patients with kidney disease in the United gan to chronicle the natural histories of a wide variety of States before World War II? They were certainly not kidney diseases. Quantitative studies of renal function under the care of nephrologists! Nephrology was not listed flourished under a school headed by Homer Smith, and in the questionnaires for any state or the American Medi surprisingly precise techniques were developed for study cal Association as a subspecialty or even as a special ing a whole range of explicit nephron functions. Imagine interest. Indeed, even in the late 1960s, when I wrote the the joy with the advent of vascular catheterization to be American Medical Association editor and asked why able to apply extraction ratios and the Fick principle in a nephrology had not been included on a questionnaire to precise way to an organ such as the kidney by sampling American physicians about their specialty interests, I re arterial blood, venous blood, and the output of the urine! ceived a "tongue in check" answer, "What's nephrology?" One had a quantitative handle on the entire function of a Indeed, for those of us who bridge back, it is often hard to vital organ - perhaps for the first time in biologic history. realize the rapid evolution of our specialty. For uremia, One no longer looked only at the street side of the we gave low-protein diets, adequate hydration, attention revolving door; one could find out, for example, that if to fluid and electrolytes, comfort, and prayer. In my first ammonia did not go into the acid trap of the urine, it two years at Georgetown, where every death in the hos indeed might go back into the circulation via the renal pital was reviewed, my nephrology division made death vein. conference all but a few weeks out of the first two years. The same story unfolded for a broad range of physio In a 1961 book on uremia2 I wrote: logic substances. In the metabolic school of nephrology, The reversibility of uremic coma has received some attention represented perhaps most brilliantly by Professor John but could use more. In a further effort to discourage pessimism Peters at Yale, a host of pioneer investigators applied the we have therefore placed a capital 'R' following each of the methods of quantitative clinical biochemistry to the ele potentially reversible types of renal disease. It is our sincere ments of the blood whose homeostasis was so carefully hope that the number of 'R's' will provide a pleasant surprise regulated by the kidney. His deep interest in endocrino for the many physicians and medical students who want to logy and metabolism pointed our way to appreciate the think of the uremic syndrome as a terminal state during which endocrine role of the kidney in making or releasing a whole little treatment can be instituted except that designed for the array of potent hormones affecting bodily function (e.g., comfort of the patient. This is not to say that the science underlying nephrology erythropoietin, renin, aldosterone, etc.), and indeed the was inactive, Quite to the contrary, many cases of fruitful very survival of the human organism. The role of the kidney science relating to the kidney area not only existed but in controlling vitamin D metabolism, calcium absorption, flourished and had a profound impact on many young parathyroid function, and the complex inter-relation clinicians. Thomas Addis raised to a state of applied ships comprising calcium/phosphorous homeostasis, bone growth, and bone repair were only mistily appreci I Revised for the second edi tion. ated and became one of the great metabolic success stories of postwar nephrology and metabolism. Postwar nephro 2 Schreiner GE Maher JF: Uremia, Chemistry and Pathogenesis logy rushed to the fore and supplied nephrologists with & Treatment. Charles C. Thomas, Springfield, IL, p 24, 1961. such wonderful tools as the f1amephotometer, electro- ix x Foreword phoresis, microchemistry, immunoassay, sonography, pediatrician, for it is the final common pathway of literally renal biopsy, immunofluorescence, electron microscopy, hundreds of disease processes that lead to scarring and and unclear magnetic resonance, and permitted a total destruction of nephrons. integration of form, histologic structure, and function. We estimate that there are well in excess of 300,000 Clinical nephrology became indeed the real fusion of bio patients in the world living on the varied methodologies chemistry, physiology, immunology, renal endocrinology, represented by the three basic forms of substituted kidney and the focus of newer imaging techniques. function - hemodialysis, peritoneal dialysis, and renal With this precision in diagnosis, one could realistically transplantation: over 100,000 persons in the United States hope for rational therapy, and one could be optimistic that alone, well over 110,000 in the countries compromising some day the correct therapy would be correctly applied the EDTA Registry, and over 100,000 in the Pacific Rim. to the correct patient with the appropriately diagnosed If we add on South America, Africa, and the lesser de disease. veloped nations, the total could well be over 400,000 by With the evolution of such developments, an expert the time this book is printed. These 400,000 plus persons observer could indeed realistically hope that out of the and their families, who have intimate, repetitive personal myriad and mushrooming books of nephrology would experiences with uremia, serve as living withnesses of the come one with a message of constructive hope, focusing on medical progress of nephrology in the past three decades. the treatment of renal disease. Indeed, Dr. Suki and Dr. They are witnesses of the fact that many of today's kidney Massry have fulfilled that hope with this book, which is patients have indeed, literally, "come from the cemetery." appropriately entitled, The Therapy of Renal Disease and But it is not enough to consider only the techniques of Related Disorders. They have systematically taken the substitution therapy. For with living patients come not available scientific information and fused it into a practical only the facets of uremia that are not yet handled by text of therapy for the patient. The first section, entitled therapy, such as cardiovascular complications, renal "Disorders of Fluid, Electrolyte and Acid-Base Balance," osteodystrophy, anemia, disorders of immune surveil covers some of the more challenging general conditions, lance, nutritional problems, etc., but there is also a neces such as hyperosmolar and hypoosmolar states, polyuria, sity to know which particular patients fit which particular edema, and acute renal failure. The book then proceeds therapy best, and to choose the optimum time for applying systematically to disorders of the ions, potassium, calcium, one particular therapy to one particular patient. Indeed, magnesium, phosphate, and the major quartet of acid the management of the uremic patients becomes essenti base balance, embracing alkalosis and acidosis in its ally a life plan for that person, and the ills that kidney clinically presentable forms. The book proceeds to the patients have live on with them, instead of going pre intrinsic parenchymal diseases, covering the major areas maturely with them to the grave. of glomerular and tubular interstitial disorders and what This is a book that is unique among many books avail can be done about them. From there it launches into the able today. This is a book that presents material positively. vast sea of relationships with systemic diseases such as This is a book that blends the analytical aspects of diagnosis SLE, vasculitis, hyperuricemia, dysproteinemia, liver dis with the hard realities of scientific and appropriate ther ease, pregnancy, and diabetes, among others. Adequate apy. This is a book that will be enjoyed by young nephro attention is paid to genetic and congenital disorders, logists and by physicians with a wide diversity of interests. including the genetic counseling of families beset by gene Most of all, it is a book that will be deeply appreciated by tically determined disorders. Neoplasia, chemical and their patients. physical injuries, and a number of other unusual events are considered with practical insights. Then the book George E. Schreiner, M.D. tackles the vast problem of uremia and the newer experi Distinguished Professor of Medicine ence with diet, dialysis, and transplantation. Former Director, Division of Nephrology Uremia is to the nephrologist what the baby is to the Georgetown University School of Medicine Preface to the First Edition In the last fifteen years, many books and monographs have The major strides forward in renal therapy shall be clear been published which deal with different aspects of renal to the reader of this volume. Areas where advances or structure and function, and the various renal diseases. The breakthroughs are still needed or where solid, objective number of published works reflects the explosion of scien proof of efficacy is still lacking shall be equally clear. The tific knowledge about the kidney and its diseases. Parallel rapid pace of new research on renal therapy continued with this increased knowledge have come major advances during the period that this text was in preparation, and this in the handling and management of patients suffering from rapid pace attests to the vitality of nephrology as a disci disorders of the kidney. These advances, many of which pline. We look forward to the preparation of new editions are life-saving, in large measure have been responsible for of this volume reflecting substantive advances which will the emergence of nephrology as a full-fledged medical continue to be made. specialty. It is fitting, in closing, to acknowledge the generosity of In spite of the progress made in the therapy of renal each of the contributing authors who have given selflessly diseases and related disorders, there has not been a text of their precious time to prepare their respective chapters, devoted fully to this subject. The present text attempts to and the forbearance of our publisher, who has waited bring together in one ready reference what is known about patiently as the process of assembling and editing this renal therapeutics today thereby focusing attention on this volume proceeded. vital aspect of nephrology and recording the present state of-the-art. Wadi N. Suki Shaul G. Massry Preface to the Second Edition It is said that a static science is a dead science, and to any above all for our patients, advances have been made and observer of nephrology it is quite clear that there has been continue to be made as we write these words. We have nothing static about this discipline. Even while the first invited many of the past authors to update their chapters, edition was under preparation, newer treatments were while several new authors were invited to rewrite chapters being developed, the efficacy of new treatments was being on topics previously covered or to write new chapters on tested, and the results of such trials were being published. topics not previously covered. The task of writing a chap It is impossible to capture in a book all the progress that ter is an onerous task and, having ourselves written many is being made in a particular discipline that is changing chapters for many texts, we are keenly aware of the time rapidly, for to do so would be akin to capturing motion in a and effort that goes into the preparation of a chapter. In still picture. One can convey the impression of motion in a addition, it is all for very little reward, whether it is in still picture, but it takes a video or a movie to capture terms of monetarv returns to the author or in terms of the motion. And so it is in nephrology, a field in which it academic recognition that the author derives from writing should be clear to anyone who takes more than a cursory a chapter, as compared with a scientific article in a peer look that major developments and important advances are reviewed journal reporting original scientific research. being made steadily. We were almost prophetic, there One can only conclude, therefore, that hundreds of au fore, when we said in the preface to the first edition: thors undertake the task of writing a chapter propelled not The rapid pace of new research on renal therapy continued by the motive of profit but by that of the noble commit during the period that this text was in preparation, and this ment to convey to their fellow physicians the latest ad rapid pace attests to the vitality of nephrology as a discipline. vances in their respective areas of expertise, with the aim We look forward to the preparation of new editions of this of bettering the health of their patients and of raising the volume reflecting substantial advances which will continue to be made. standards of the care they receive. It is fitting then to bear in mind that a text such as this is a tribute to each of the No sooner had the first edition come out in print than authors who contributed to it, and all of us who shall had a process of obsolescence already begun to set in as consult this text, as we tackle the complexities of managing the wheels of progress kept on turning - hopefully lead our patients, are in their debt. ing us all forward. We were almost prophetic in predicting "the preparation of new editions." So now we come back Wadi N. Suki with a new edition to report on some of the advances that Shaul G. Massry were made since the first edition, and luckily for us all, and

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