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Seldin and Giebisch's The Kidney: Physiology and Pathophysiology, fifth edition
Robert J. Alpern, Orson W. Moe and Michael Caplan, Editors
 SELDIN AND
GIEBISCH’S THE
KIDNEY
PHYSIOLOGY AND
PATHOPHYSIOLOGY
FIFTH EDITION
VOLUME 1
ROBERT J. ALPERN
Yale University School of Medicine
New Haven, CT, USA

ORSON W. MOE
The University of Texas Southwestern Medical Center at Dallas
Dallas, TX, USA

MICHAEL CAPLAN
Yale University School of Medicine, New Haven, CT, USA

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12 13 14 15 16 10 9 8 7 6 5 4 3 2 1
 Dedication

This book is dedicated to Steven C. Hebert. Steve was concepts of kidney function and disease. He was an
at his heart a kidney physiologist, applying cutting extraordinary scientist, clinician, mentor, leader and
edge technologies to address the most fundamental friend.
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 CONTENTS

9. Microvascular Permeability and the Exchange

VOLUME 1 of Water and Solutes Across Microvascular
Walls 263
C. CHARLES MICHEL
Foreword xiii
Preface xv
List of Contributors xvii 10. External Balance of Electrolytes and Acids
and Alkali 291
MAN S. OH

I 11. Renal Cilia Structure, Function, and

EPITHELIAL AND NONEPITHELIAL Physiology 319
JAY N. PIECZYNSKI AND BRADLEY K. YODER
TRANSPORT AND REGULATION
12. Intercellular Junctions 347
1. Epithelial Cell Structure and Polarity 3 ALAN S.L. YU, FIONA HANNER AND JÁNOS PETI-PETERDI
KARL S. MATLIN AND MICHAEL J. CAPLAN
13. Principles of Cell Signaling 369
2. Mechanisms of Ion Transport across Cell LLOYD CANTLEY

Membranes 45
LUIS REUSS AND GUILLERMO A. ALTENBERG 14. Scaffolding Proteins in Transport Regulation 405
PAUL A. WELLING

3. Renal Ion-Translocating ATPases 67

DOMINIQUE ELADARI, UDO HASLER AND ERIC FÉRAILLE 15. The Renin
Angiotensin System 427
THU H. LE, STEVEN D. CROWLEY, SUSAN B. GURLEY AND

4. Mechanisms of Water Transport Across Cell THOMAS M. COFFMAN

Membranes and Epithelia 95

GUILLERMO A. ALTENBERG AND LUIS REUSS 16. Neural Control of Renal Function 451
EDWARD J. JOHNS AND ULLA C. KOPP

5. Cell Volume Control 121

FLORIAN LANG 17. Eicosanoids and Renal Function 487
MATTHEW D. BREYER, RAYMOND C. HARRIS AND
RICHARD M. BREYER
6. Solute Transport, Energy Consumption, and
Production in the Kidney 143
TAKASHI SEKINE AND HITOSHI ENDOU
18. Extracellular Nucleotides and Renal
Function 511
DAVID G. SHIRLEY, MATTHEW A. BAILEY, SCOTT S.P. WILDMAN,
7. Electrophysiological Analysis of Transepithelial FREDERICK W.K. TAM AND ROBERT J. UNWIN
Transport 177
HENRY SACKIN AND LAWRENCE G. PALMER
19. Paracrine Regulation of Renal Function by
Dopamine 539
8. Renal Ion Channels, Electrophysiology of PEDRO A. JOSE, ROBIN A. FELDER AND
Transport, and Channelopathies 217 GILBERT M. EISNER
JAMES D. STOCKAND

vii
viii CONTENTS

II 31. Anion Channels 1019

OWEN M. WOODWARD AND WILLIAM B. GUGGINO
STRUCTURAL AND FUNCTIONAL
ORGANIZATION OF THE KIDNEY 32. Physiology and Pathophysiology of the NaCl
Co-Transporters in the Kidney 1047
GERARDO GAMBA
20. Structural Organization of the Mammalian
Kidney 595 33. Sodium and Chloride Transport: Proximal
WILHELM KRIZ AND BRIGITTE KAISSLING Nephron 1081
ALAN M. WEINSTEIN
21. Biophysical Basis of Glomerular Filtration 693
SCOTT C. THOMSON AND ROLAND C. BLANTZ 34. Sodium Chloride Transport in the Loop of Henle,
Distal Convoluted Tubule, and Collecting Duct 1143
22. Glomerular Cell Biology 721 GERARDO GAMBA, WENHUI WANG AND LAURENT SCHILD
YOSHIRO MAEZAWA, DAVIDE CINA AND SUSAN E. QUAGGIN
35. Mineralocorticoid Action in the Aldosterone
23. Function of the Juxtaglomerular Apparatus: Sensitive Distal Nephron 1181
Control of Glomerular Hemodynamics and Renin OLIVIER STAUB AND JOHANNES LOFFING
Secretion 757
JÜRGEN B. SCHNERMANN AND HAYO CASTROP 36. Inherited Disorders of Renal Salt Homeostasis:
Insights from Molecular Genetics Studies 1213
24. Renal Cortical and Medullary Microcirculations: UTE I. SCHOLL AND RICHARD P. LIFTON
Structure and Function 803
THOMAS L. PALLONE AND CHUNHUA CAO 37. Natriuretic Hormones 1241
DAVID L. VESELY
25. Molecular and Cellular Mechanisms of Kidney
Development 859 38. Pathophysiology of Sodium Retention and
KEVIN T. BUSH, HIROYUKI SAKURAI AND SANJAY K. NIGAM
Wastage 1283
BIFF F. PALMER, ROBERT J. ALPERN AND DONALD W. SELDIN
26. Molecular and Cellular Mechanisms of
Glomerular Capillary Development 891 39. Physiology and Pathophysiology of
JEFFREY H. MINER AND DALE R. ABRAHAMSON
Hypertension 1319
JOHN E. HALL, JOEY P. GRANGER AND MICHAEL E. HALL
27. Postnatal Renal Development 911
MICHEL BAUM, JYOTHSNA GATTINENI AND LISA M. SATLIN
40. Physiology and Pathophysiology of Diuretic
28. Renal Hyperplasia and Hypertrophy 933 Action 1353
PAUL T. BRINKKOETTER, SIAN V. GRIFFIN AND STUART J. SHANKLAND DAVID H. ELLISON

29. Stem Cells and Generation of New Cells in the 41. Aquaporin Water Channels in Mammalian
Adult Kidney 959 Kidney 1405
JUAN A. OLIVER AND QAIS AL-AWQATI SØREN NIELSEN, TAE-HWAN KWON, HENRIK DIMKE, MARTIN SKOTT
AND JØRGEN FRØKIÆR

42. Thirst and Vasopressin 1441

III GARY L. ROBERTSON

FLUID AND ELECTROLYTE 43. The Urine Concentrating Mechanism and Urea
REGULATION AND DYSREGULATION Transporters 1463
JEFF M. SANDS AND HAROLD E. LAYTON

30. Epithelial Na1 Channels 983 44. Hyponatremia 1511

SHAOHU SHENG, KENNETH R. HALLOWS AND THOMAS R. KLEYMAN RICHARD H. STERNS, STEPHEN M. SILVER AND J. KEVIN HIX
 CONTENTS ix
45. Hypernatremic States 1541 48. Extrarenal Potassium Metabolism 1629
CHRISTOPHER J. RIVARD, WEI WANG AND LAURENCE CHAN VAIBHAV SAHNI, ALEKSANDRA GMURCZYK AND ROBERT M. ROSA

49. Regulation of K1 Excretion 1659

46. Polyuria and Diabetes Insipidus 1571
GERHARD MALNIC, GERHARD GIEBISCH, SHIGEAKI MUTO, WENHUI
DANIEL G. BICHET
WANG, MATTHEW A. BAILEY AND LISA M. SATLIN

1
47. The Molecular Biology of Renal K 50. Physiopathology of Potassium Deficiency 1717
Channels 1601 FRANCESCO TREPICCIONE, MIRIAM ZACCHIA
WENHUI WANG AND CHOU-LONG HUANG AND GIOVAMBATTISTA CAPASSO
x CONTENTS

VOLUME 2 65. The Hormonal Regulation of Calcium

Metabolism 2249
PETER J. TEBBEN AND RAJIV KUMAR

Foreword xiii 66. Disorders of Calcium Metabolism 2273

Preface xv CRISTINA DUMITRU AND JOHN WYSOLMERSKI
List of Contributors xvii
67. Pathogenesis and Treatment of
Nephrolithiasis 2311
FREDRIC L. COE, ANDREW EVAN AND ELAINE WORCESTER
51. Clinical Disorders of Hyperkalemia 1741
KAMEL S. KAMEL, SHIH-HUA LIN, SUNG-SEN YANG
68. Proximal Tubular Handling of Phosphate 2351
AND MITCHELL HALPERIN
HEINI MURER, IAN C. FORSTER, NATI HERNANDO AND JÜRG BIBER

52. Control of Intracellular pH 1773

69. Clinical Disturbances of Phosphate
MARK O. BEVENSEE AND WALTER F. BORON
Homeostasis 2369
53. SLC4 Sodium-Driven Bicarbonate THERESA J. BERNDT AND RAJIV KUMAR

Transporters 1837
70. Glucose Reabsorption in The Kidney 2393
IRA KURTZ
CHARLES S. HUMMEL AND ERNEST M. WRIGHT

54. The SLC4 Anion Exchanger Gene Family 1861

71. Kidney Transport of Amino Acids and
ANDREW K. STEWART AND SETH L. ALPER
Oligopeptides, and Aminoacidurias 2405
55. Cellular Mechanisms of Renal Tubular SIMONE M.R. CAMARGO, VICTORIA MAKRIDES, ROBERT KLETA
AND FRANÇOIS VERREY
Acidification 1917
L. LEE HAMM, ROBERT J. ALPERN AND PATRICIA A. PREISIG
72. Organic Anion and Cation Transporters in Renal
56. Chemoreceptors, Breathing and pH 1979 Elimination of Drugs 2425
GERHARD BURCKHARDT AND HERMANN KOEPSELL
EUGENE NATTIE

57. Renal Ammonium Ion Production and 73. Renal Filtration, Transport, and Metabolism of
Excretion 1995 Albumin and Albuminuria 2457
ERIK ILSØ CHRISTENSEN, RIKKE NIELSEN AND HENRIK BIRN
NORMAN P. CURTHOYS

58. Clinical Syndromes of Metabolic Alkalosis 2021

ORSON W. MOE, ROBERT J. ALPERN AND DONALD W. SELDIN
IV
59. Clinical Syndromes of Metabolic Acidosis 2049 PATHOPHYSIOLOGY OF RENAL
RETO KRAPF, DONALD W. SELDIN AND ROBERT J. ALPERN
DISEASE
60. Respiratory Acid
Base Disorders 2113
NICOLAOS E. MADIAS AND HORACIO J. ADROGUÉ
74. Physiologic Principles in the Clinical Evaluation
61. Mechanisms and Disorders of Magnesium of Electrolyte, Water, and Acid
Base Disorders 2477
Metabolism 2141 DANIEL BATLLE, SHELDON CHEN AND SYED K. HAQUE

KARL P. SCHLINGMANN, GARY A. QUAMME AND MARTIN KONRAD

75. Biomarkers for Assessment of Renal Function
62. Calcium Channels 2167 During Acute Kidney Injury 2513
JOOST G.J. HOENDEROP AND RENÉ J.M. BINDELS PRASAD DEVARAJAN

63. The Calcium-Sensing Receptor 2187 76. Pathophysiology of Acute Kidney Injury 2527
EDWARD M. BROWN, STEVEN C. HEBERT, DANIELA RICCARDI BRUCE A. MOLITORIS AND ASIF SHARFUDDIN
AND JOHN P. GEIBEL
77. Ischemic Renal Disease 2577
64. Renal Calcium Metabolism 2225 CHRISTOPHER J. COOPER, LANCE D. DWORKIN AND
JOSÉ F. BERNARDO AND PETER A. FRIEDMAN WILLIAM L. HENRICH
 CONTENTS xi
78. Pathophysiology and Pathogenesis of Diabetic 87. Role of Proteinuria in the Progression of Renal
Nephropathy 2605 Disease 2961
SHELDON CHEN, CHARBEL KHOURY AND FUAD N. ZIYADEH MAURO ABBATE, DANIELA MACCONI, GIUSEPPE REMUZZI
AND CARLA ZOJA

79. Renal Failure in Cirrhosis 2633

PERE GINÈS, ANDRÉS CÁRDENAS AND
88. The Inflammatory Response to Ischemic Acute
ROBERT W. SCHRIER Renal Injury 2985
CHRISTOPHER Y. LU

80. Autosomal Dominant Polycystic Kidney 89. Catalytic (Labile) Iron in Kidney Disease 3007
Disease 2645 MOHAN RAJAPURKAR, RADHAKRISHNA BALIGA AND SUDHIR V. SHAH
STEFAN SOMLO, VICENTE E. TORRES AND MICHAEL J. CAPLAN
90. Chronic Kidney Disease: Pathophysiology and the
81. Renal Physiology and Disease in Pregnancy 2689 Influence of Dietary Protein 3021
MARSHALL D. LINDHEIMER, KIRK P. CONRAD AND BIRUH T. WORKENEH AND WILLIAM E. MITCH
S. ANANTH KARUMANCHI
91. Management of Calcium and Bone Disease in
82. Immune and Inflammatory Glomerular Renal Patients 3073
Diseases 2763 L. DARRYL QUARLES
ANUP MANOHARON, JEFFREY R. SCHELLING, MATTHEW DIAMOND,
MOONJA CHUNG-PARK, MICHAEL MADAIO AND JOHN R. SEDOR 92. Hematopoiesis and the Kidney 3087
ARMIN KURTZ, ROLAND H. WENGER AND KAI-UWE ECKARDT

83. Immunologic Mechanisms of Vasculitis 2817 93. Individualizing the Dialysate to Address
JONATHON W. HOMEISTER, J. CHARLES JENNETTE AND RONALD J. FALK
Electrolyte Disturbances in the Dialysis Patient 3125
BIFF F. PALMER
84. Genetic Abnormalities in Glomerular
Function 2847 94. Homeostasis of Solute and Water by the
YOAV SEGAL AND CLIFFORD E. KASHTAN Transplanted Kidney 3151
J. HAROLD HELDERMAN, HEIDI SCHAEFER, ANTHONY J. LANGONE AND
85. Cellular Mechanisms of Drug SIMIN GORAL
Nephrotoxicity 2889
ROBERT JAMES WALKER AND ZOLTÁN HUBA ENDRE 95. Renal Disposition of Drugs and Translation to
Dosing Strategies 3185
86. Role of Glomerular Mechanical Stress in the PÄR MATSSON, KATHLEEN M. GIACOMINI AND D. CRAIG BRATER
Pathogenesis of Chronic Kidney Disease 2933
CLARICE KAZUE FUJIHARA AND ROBERTO ZATZ Index 3215
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 Foreword

The focus of these volumes ever since the publica- In this 5th edition, such new and powerful disci-
tion of the first edition in 1985 has been on renal physi- plines as genetics and cell biology have been deployed
ology, conceived broadly as the analysis of those to deepen and widen further the explanatory frame-
processes by which the kidney maintains the volume work. Not only have previous chapters been exten-
and composition of the body in the face of varied sively updated, but new chapters have been added to
intake, physiologic alterations and pathologic distur- incorporate additional disciplines. Individual chapters,
bances. The text began with a detailed exploration of for example, now provide detailed treatment of the sig-
the basic mechanisms and their regulation that under- nificance of cilia; the role of stem cells is now given
lie the exchanges of water and electrolytes across cell special consideration. Finally, there has been a signifi-
membranes of the body. The mechanisms used by the cant expansion of the section of pathophysiology,
kidney to make appropriate renal homeostatic adjust- incorporating the newer findings of cell biology and
ments were next presented. genetics.
In the early texts the renal response was attributed In a sense, this new edition represents a significant
to the whole organ rather than to discrete molecular advancement in the march of reduction to a more fun-
processes. It was the application of the methods and damental level of understanding of the normal and
theories of the generalizing sciences, physics and deranged function of the kidney.
chemistry, to biologic systems that, in subsequent addi-
tions, facilitated a reduction of the explanatory system Donald W. Seldin
to the more basic level of molecular biology, thereby Gerhard H Giebisch
providing a more comprehensive understanding of
the basic processes that permit the physiologic
adjustments.

xiii
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 Preface

As described in its preface, the first edition of The to a much broader set of ailments. We continue, there-
Kidney: Physiology and Pathophysiology, published in fore, to broaden the scope of this book so as to place
1985, focused on renal physiology, “conceived broadly greater emphasis on mechanisms of disease.
as the study of those processes by which the kidney The first section of the textbook begins with general
maintains the volume and composition of the body in principles of epithelial and non-epithelial transport
the face of physiologic demands and pathologic distur- and regulation. This extensive section of the book con-
bances.” As noted in the fourth edition, science has tinues a tradition established in the first edition, but
since then become more reductionist, an evolution that extends it to include a more extensive discussion of
has been reflected in the content of subsequent edi- transport regulation.
tions. Dissection of physiologic phenomena at the level The second section of the book describes the organi-
of organs and cells has been enriched by descriptions zation of the kidney with an increased emphasis on the
of the roles of individual molecules. While this trend in glomerulus, as this field continues to evolve. There fol-
science has continued, it has been complemented by a lows an in-depth review of renal growth and develop-
renewed focus on integrating these molecular functions ment, including a discussion of the role of stem cells in
to define their roles in cellular and organ physiology, the kidney.
as well as their role in body homeostasis and disease. This is then followed by Section Three, describing
A complete understanding of physiologic and patho- mechanisms of fluid and electrolyte regulation and
physiologic processes must include knowledge of dysregulation. In no other book can one find this sub-
individual molecules, as well as their integration into ject addressed with the depth and thoroughness found
homeostatic systems that function to maintain body in this textbook. The Fifth Edition includes a more
composition. A thorough understanding of physiologic in-depth discussion of recently described families of
mechanisms endows us with a greater understanding transporters, integrating this information to describe
of pathophysiology and disease. Reciprocally, an their role in physiologic and pathophysiologic
understanding of disease states furnishes us with valu- processes.
able information about normal physiology. The present Section Four, the pathophysiology of renal disease,
edition continues to focus on describing the present has been extended as our knowledge of renal dysfunc-
state of knowledge of the molecules and the systems tions and their contribution to renal ailments has
that contribute to normal physiologic function of the expanded. Many chapters deal with common everyday
kidney, and the homeostatic mechanisms subserved by clinical issues, but are presented in the context of
the kidney. pathophysiologic mechanisms. A series of chapters
The present edition also concentrates on how these focus on mechanisms of progression of renal disease,
mechanisms malfunction, resulting in the diseased as the importance of interrupting or forestalling this
state. Again we will address the pathophysiology of progression has assumed great importance in clinical
disease states from the molecular to the system level. nephrology. A thorough understanding of the roles of
One of the appealing features of nephrology is the abil- glomerular pressure, proteinuria, inflammation, and
ity to utilize our understanding of normal physiology oxidants will help researchers and clinicians prevent
to elucidate principles of pathophysiology, and second- renal failure, decreasing the need for dialysis and
arily develop rational approaches to the diagnosis, transplant.
treatment, and prevention of disease. Thus, the clini- The evolution of our understanding of kidney func-
cian addressing a patient with a fluid and electrolyte tion and dysfunction derives from sequential discover-
disorder need not memorize a list of possible causes or ies made by a series of investigators, each benefiting
algorithms, but can logically deduce a solution through from the accomplishments of their predecessors. The
a thorough understanding of kidney function. As sci- same can be said for this textbook. Originally con-
ence continues to evolve, our understanding of the ceived by two of the greatest renal physiologists of the
pathophysiologic basis of disease can now be applied twentieth century, Donald Seldin and Gerhard

xv
xvi PREFACE

Giebisch, it was passed on to Steve Hebert and one of nephrology, and in so doing to honor the previous edi-
the present editors. As the present editors, it is our tors for all that they have contributed to the book, to
hope to continue the book’s commitment to science, nephrology, to epithelial physiology, and to science in
and its role in our understanding and practice of general.