The Cystic Kidney PDF
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The Cystic Kidney The Cystic Kidney edited by Kenneth D. Gardner, Jr. Department of Medicine University of New Mexico Hospital Albuquerque USA Jay Bernstein Department ofA natomic Pathology William Beaumont Hospital Royal Oak USA , ... , KLUWER ACADEMIC PUBLISHERS ~. DORDRECHTI BOSTON I LONDON Distributors for the United States and Canada: Kluwer Academic Publishers, PO Box 358, Accord Station, Hingham, MA 02018-0358, USA for all other countries: Kluwer Academic Publishers Group, Distribution Center, PO Box 322,3300 AH Dordrecht, The Netherlands Library of Congress Cataloging-in-Publication Data The cystic kidney / edited by Kenneth D. Gardner, Jr., Jay Bernstein. p. cm. - - (Developments in Nephrology: 27) Includes index. ISBN-13: 978-94-010-6690-7 e-ISBN-13: 978-94-009-0457-6 DOl: 10.1007/978-94-009-0457-6 1. Kidney, Cystic. 2. Polycystic kidney disease. 1. Gardner, Kenneth D. II. Bernstein, Jay. III. Series. [DNLM: 1. Kidney, Cystic. WI DE998EB v.27 / WJ 358 C9975] RC918.6'I-dc20 DNLM/DLC for Library of Congress 89-15555 Copyright © 1990 by Kluwer Academic Publishers Softcover reprint of the hardcover 1st edition 1990 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, electronic, mechanical, photocopying, recording or otherwise, without prior permission from the publishers, Kluwer Academic Publishers BV, PO Box 17, 3300 AA Dordrecht, The Netherlands. Published in the United Kingdom by Kluwer Academic Publishers, PO Box 55, Lancaster, UK. Kluwer Academic Publishers BV incorporates the publishing programmes of D. Reidel, Martinus Nijhoff, Dr W. Junk and MTP Press. Contents Preface KD. Gardner lr and 1 Bernstein vii List of contributors IX SECTION 1 1 Cysts and cystic kidneys KD. Gardner lr 3 SECTION 2 2 Cyst cells and cyst walls A.P. Evan and lA. McAteer 21 3 The interstitium of the cystic kidney C.l Kelly and E.G. Neilson 43 4 Models of cysts and cystic kidneys E.D.Avner, lA. McAteerandA.P. Evan 55 5 Pathogenesis of cysts and cystic kidneys L. W. Welling 99 6 The genetics of renal cystic disease S. T. Reeders 117 SECTION 3 7 A classification of renal cysts 1 Bernstein 147 8 Radiology of cystic kidneys E. Levine and 11 Grantham 171 9 Systemic manifestations of renal cystic disease V.E. Torres 207 10 Management of cystic kidney disease W.M. Bennett, L. W. Elzinga and 1M. Barry 247 vi 11 Ethical issues and cystic kidneys W.B. Weil 277 SECTION 4 12 Autosomal dominant polycystic kidney disease PA. Gabow 295 13 Autosomal recessive polycystic kidney disease B.R. Cole 327 14 Acquired renal cystic disease 1. Ishikawa 351 15 Medullary sponge kidney E.R. Yendt 379 16 Congenital multicystic kidney c.P. Piel 393 17 Multilocular cystic renal lesions - malformations, benign nephromas or differentiated Wilms tumors? 1M. Kissane 413 Index 437 Preface This is a book about renal cysts and cystic kidneys. Its contributors have created a resource of current information in a field that once aroused only curiosity, but that now stands at the leading edge of molecular nephrology. Its authorship includes 'oldtimers', who bring the wisdom of experience, and 'newcomers', whose presence attests to the contributions made by the investigative and technological advances of the past decade. Its text is organized to carry the reader from renal cyst to cystic renal disease. Each of its chapters defines or explores a challenge or an advance. Cells that line renal cysts are diverse in structure, type, and perhaps function. The cysts themselves lie within an interstitium that is not normal and may influence cyst development and growth. Experimental analogs of human disease offer increasing opportunities to basic researchers to examine, in sequence and under controlled circumstances, those events that favor nephron dilation, cyst growth and ultimate renal failure. While models have provided clues important to pathogenesis, it is appro priate that the greatest advance has come from the application of human molecular genetics. Probes have identified the short arm of human chromo some 16 as the site of a mutant gene responsible for autosomal dominant polycystic kidney disease in 95% of affected families. This demonstration has, in turn, opened to debate such questions as the ethics of gene-linkage analysis for in utero diagnosis and preclinical detection, and the question of whether, in the affected popUlation, gene modification or control of gene expression is the more practical therapeutic goal. In the heart of the volume are chapters on classification, radiography, systemic manifestations and management. Their contents reflect the contribu tions made by intellectual dialog, technology, and experience to the detection and treatment of renal cysts and cystic disorders. Finally, there are chapters on the renal cystic diseases themselves, encompassing those disorders that have proven most challenging to clinician, diagnostician and therapeutician, with whom rests the ultimate responsibility for control of morbidity and mortality. We, the editors, have made no attempt through censorship to conceal conflict of opinion, where it exists, among the contributors. We have, however striven to place on display the levels of sophistication that have been achieved in understanding the pathogenesis and in detecting and treating these disor ders. We thank our contributors. We acknowledge our failure to include all the researchers and clinicians who currently continue to make substantial contribu tions to the field. We hope that the essays that appear in this volume convey the excitement that now surrounds the renal cyst and the disorders with which it is associated. Kenneth D. Gardner Jr. Jay Bernstein Albuquerque, New Mexico Royal Oak, Michigan December, 1989 List of Contributors ED Avner APEvan University of Washington School of Department of Anatomy Medicine Indiana University School of Medicine Children's Hospital and Medical Center 635 Barnhill Drive 4800 Sand Point Way NE/PO Box C5371 Indianapolis, IN 46223 Seattle, W A 98105 USA USA PAGabow JMBarry Medical Service Division of Nephrology Denver General Hospital Oregon Health Sciences University 777 Bannock Street 3181 S.W. Sam Jackson Park Road Denver, CO 80204-4507 Portland, OR 97201 USA USA KD Gardner Jr. WMBennett Division of Renal Diseases Division of Nephrology Department of Medicine Oregon Health Sciences University University of New Mexico School of 3181 S.W. Sam Jackson Park Road Medicine Portland, OR 97201 Albuquerque, NM 87131 USA USA J Bernstein JJ Grantham Department of Anatomic Pathology Department of Medicine William Beaumont Hospital University of Kansas Medical Center Royal Oak, MI 48072 Rainbow Blvd at 39th Stret USA Kansas City, KS 66103 USA BR Cole Division of Nephrology I Ishikawa Washington University School of Medicine Department of Internal Medicine 400 South Kingshighway Kanazawa Medical University St Louis, MO 63110 Uchinada, Kahoku USA Ishikawa 920-02 Japan LWElzinga Division of Nephrology CJKelly Oregon Health Sciences University Renal Electrolyte Section 3181 S.W. Sam Jackson Park Road Hospital of University of Pennsylvania Portland, OR 97201 3400 Spruce Street USA Philadelphia, P A 19104 USA x JMKissane STReeders Department of Pathology Howard Hughes Medical Institute Washington University School of Medicine Departments of Nephrology and Human Box 8118 Genetics St Louis, MO 63110 Yale University School of Medicine USA New Haven, CT 16510 USA ELevine Department of Diagnostic Radiology VE Torres University of Kansas Medical Center Division of Nephrology and Internal Rainbow Blvd at 39th Street Medicine Kansas City, KS 66103 Mayo Clinic and Mayo Foundation USA 1734 7th Street S.W. Rochester, MN 55905 JAMcAteer USA Department of Anatomy Indiana University School of Medicine WBWeil 635 Barnhill Drive Department of Pediatrics and Human Indianapolis, IN 46223 Development USA B-240 Life Sciences Building Michigan State University EGNeilson East Lansing, MI48824-1317 Renal Electrolyte Section USA Hospital of University of Pennsylvania 3400 Spruce Street LWWelling Philadelphia, P A 19104 Department of Pathology USA University of Kansas Medical Center 4801 Linwood Boulevard CFPiel Kansas City, MO 64128 Department of Pediatrics USA University of California, San Francisco 400 Parnassus Avenue, Rm A276 E Yendt San Francisco, CA 94143-0314 Division of Endocrinology USA Etherington Hall Queen's University Kingston Ontario K7L 3N6 Canada Developments in Nephrology 1. 1.S. Cheigh, KH. Stenzel and AL. Rubin (eds.): Manual of Clinical Nephrology of the Rogosin Kidney Center. 1981 ISBN 90-247-2397-3 2. KD. Nolph (ed.): Peritoneal Dialysis. 1981 ed.: out of print 3rd revised and enlarged ed. 1988 (not this series) ISBN 0-89838-406-0 3. All. Gruskin and M.E. Norman (eds.): Pediatric Nephrology. 1981 ISB~90-247-2514-3 4. O. Schlick: Examination of the Kidney Function. 1981 ISBN 0-89838-565-2 5. 1. Strauss (ed.): Hypertension, Fluid-electrolytes and Tubulopathies in Pediatric Nephrology. 1982 ISBN 90-247-2633-6 6. 1. Strauss (ed.): Neonatal Kidney and Fluid-electrolytes. 1983 ISBN 0-89838-575-X 7. 1. Strauss (ed.):Acute Renal Disorders and Renal Emergencies. 1984 ISBN 0-89838-663-2 8. L.l.A Didio and P.M. Motta (eds.): Basic, Clinical, and Surgical Nephrol- ogy. 1985 ISBN 0-89838-698-5 9. E.A Freidman and C.M. Peterson (eds.): Diabetic Nephropathy. Strategy for Therapy. 1985 ISBN 0-89838-735-3 10. R. DZlirik, B. Lichardus and W. Guder: Kidney Metabolism and Function. 1985 ISBN 90-247-2749-3 11. 1. Straus (ed.): Homeostasis, Nephrotoxicity, and Renal Anomalies in the Newborn. 1986 ISBN 0-89838-766-3 12. D.G. Oreopoulos (ed.): Geriatric Nephrology. 1986 ISBN 0-89838-781-7 13. E.P. Paganini (ed.):Acute Continuous Renal Replacement Therapy. 1986 ISBN 0-89838-793-0 14. 1.S. Cheigh, KH. Stenzel and AL. Rubin (eds.): Hypertension in Kidney Disease. 1986 ISBN 0-89838-797-3 15. N. Deane, RJ. Wineman and G.A Benis (eds.): Guide to Reprocessing of Hemodialyzers. 1986 ISBN 0-89838-798-1 16. C. Ponticelli, L. Minetti and G. D'Amico (eds):Antiglobulins, Cryoglobu- lins and Glomerulonephritis. 1986 ISBN 0-89838-810-4 17. 1. Strauss (ed.): with the assistance of L. Strauss: Persistent Renal Genito- urinary Disorders. 1987 ISBN 0-89838-845-7 18. V.E. Andreucci and A Dal Canton (eds.): Diuretics. Basic, Pharmacolog- ical, and Clinical Aspects. 1987 ISBN 0-89838-885-6 19. P.H. Bach and E.H. Lock (eds): Nephrotoxicity in the Experimantal and Clinical Situation, Part 1. 1987 ISBN 0-89838-997-1 20. P.H. Bach and E.H. Lock (eds): Nephrotoxicity in the Experimantal and Clinical Situation, Part 2. 1987 ISBN 0-89838-980-2 xii 21. S.M. Gore and B.A. Bradley (eds.): Renal Transplantation. Sense and Sensitization. 1988 ISBN 0-89838-370-6 22. L. Minetti, G. D'Amico and C. Ponticelli: The Kidney in Plasma Cell Dyscrasias. 1988 ISBN 0-89838-385-4 23. A.S. Lindblad, J.W. Novak and K.D. Nolph (eds.): ContinuousAmbula tory Peritoneal Dialysis in the USA. Final Report of the National CAPD Registry 1981-1988.1989 ISBN 0-7923-0 179-X 24. V.E. Andreucci and A. Dal Canton (eds.): Current Therapy in Nephrology. 1989 ISBN 0-7923-0206-0 25. L. Kovaks and B. Lichardus: Vasopressin. Disturbed Secretion and its Effects. 1989 ISBN 0-7923-0249-4
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