pediatric nephrology - springer978-3-540-76341-3/1 · preface through its past ﬁve editions,...

Pediatric Nephrology

Ellis D. Avner, William E. Harmon, Patrick Niaudet, Norishige Yoshikawa (Eds.)

Pediatric NephrologySixth Completely Revised, Updated andEnlarged Edition

With 632 Figures and 260 Tables

Editors:

Senior Editor

Ellis Avner

Director, Childrens Research Institute, CHHS,

Associate Dean for Research,

Professor of Pediatrics and Physiology

Medical College of Wisconsin

Wisconsin

USA

[email protected]

Co-Editors

William Harmon

Professor of Pediatrics,

Harvard Medical School,

Director, Pediatric Nephrology

Childrens Hospital Boston

300 Longwood Avenue

Boston, Massachusetts 02115

USA

[email protected]

Patrick Niaudet

Service de Nephrologie Pediatrique

Centre de reference des Maladies Renales Hereditaires

de lEnfant et de lAdulte (MARHEA)

Hopital NeckerEnfants Malades

149, rue de Sevres

75743 Paris Cedex 15

France

[email protected]

Norishige Yoshikawa

Professor and Chair

Department of Pediatrics

Wakayama Medical University

PediatricianinChiefWakayama Medical University Hospital

64185108111 Kimiidera, Wakayama City

Japan

[email protected]

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Preface

Through its past five editions, Pediatric Nephrology has become the standard medical reference for health care

professionals treating children with kidney disease. This new edition, published only five years since the previous

version, reflects the tremendous increase of critical information required to clinically translate molecular and cellular

pathophysiology into the prevention, diagnosis, and therapy of childhood renal disorders. This text is particularly

targeted to pediatricians, pediatric nephrologists, pediatric urologists, and physicians in training. It is also targeted to

the increased number of health care professionals involved in the multidisciplinary team caring for children with kidney

disease and their families: geneticists, genetic counselors, nurses, dialysis personnel, nutritionists, social workers, and

mental health professionals. Finally, this reference is designed to serve the needs of primary care physicians (internists

and family practitioners), as well as internist nephrologists who are increasingly involved in the initial evaluation and/or

longitudinal care of children with renal disease under managed health care delivery systems evolving throughout

the world.

The new, sixth edition of Pediatric Nephrology is organized into twelve main sections. The text begins with an

overview of the basic developmental anatomy, biology and physiology of the kidney which provides the critical

information necessary to understand the developmental nature of pediatric renal diseases. This is followed by a

comprehensive presentation of the evaluation, diagnosis and therapy of specific childhood kidney diseases, including

the extensive use of clinical algorithms. Of particular note is a special section on how rapidly-evolving research advances

in molecular genetics, cell biology, and evidence-based medicine are being translated into new clinical approaches and

therapies for many childhood renal diseases. The final sections focus on comprehensive, state-of-the art reviews of acute

and chronic renal failure in childhood. To keep pace with the dramatic changes in pediatric renal medicine since the

previous edition, the content of the sixth edition has been extensively revised. More than 40% of the sixth edition has

been completely re-written by new authors, all recognized as global authorities in their respective areas. The remainder

of the text has been completely revised and updated, often with new junior authors joining senior authors from the

previous edition. In addition to the Around the World Section, which focuses on unique aspects of pediatric

nephrology practice and the epidemiology of pediatric renal disease in different regions of the world, all of the chapters

have been rewritten to reflect a global, worldwide perspective. This has led to the official endorsement of the sixth

edition of Pediatric Nephrology by the International Pediatric Nephrology Association (IPNA) as the standard

global reference text in the field of childhood kidney disease. We are proud that the IPNA logo adorns the cover of

Pediatric Nephrology in recognition of this endorsement. The Editors look forward to a dynamic interaction with

IPNA to take advantage of future opportunities that such a collaboration may provide in the areas of education and

outreach activities.

Other significant changes are also present in this new, sixth edition of Pediatric Nephrology. The textbook has a new

publisher, Springer, which has led to a new look of the cover and printing, as well as the welcome expansion to two

volumes. In addition, in accord with all previous editions of the text, a new Editor, Professor Norishige Yoshikawa of

Wakayama University, Japan, has joined the Editorial Team. This regular change in editors continues to provide a

dynamic mixture of continuity, new ideas, new perspectives and globalism. The current editors are internationally

recognized leaders in complementary areas of pediatric nephrology, and reflect the global nature of the text and the

subspecialty it serves. The Senior Editor, Professor Ellis D. Avner, serves as Associate Dean for Research and Professor of

Pediatrics and Physiology of the Medical College of Wisconsin and Director of the Childrens Research Institute, and has

a focused interest in developmental renal biology, congenital/genetic diseases of the kidney, and research methods in

nephrology. Professor William E. Harmon, serves as Director of Pediatric Nephrology and Professor of Pediatrics at the

Childrens Hospital Boston and Harvard Medical School, and is a leading expert in the field of childhood chronic renal

disease and its treatment. Professor Patrick Niaudet, Director of the Pediatric Nephrology Unit at the University of Paris

# Springer-Verlag Berlin Heidelberg 2009

vi Preface

and the Hopital Necker Enfants Malades, is an internationally-recognized educator and clinician-scientist in childhood

nephropathy with a particular focus on childhood nephrosis. Professor Norishige Yoshikawa serves as Chairman and

Professor of Pediatrics at Wakayama University, and has a focused interest in all aspects of childhood glomerulopathies,

particularly IgA nephropathy.

In closing, the Editors wish to thank a number of individuals whose efforts were critical in the success of this project.

The book would never have reached this sixth edition without the dedication of our professional colleagues at Springer,

Ms. Marion Kramer, Ms. Gabriele Schroder, and particularly Mr. Andrew Spencer, our Managing Development Editor,

who served as our guide for the perplexed in all aspects of project management. We thank our families, and

particularly our wives Jane, Diane, Claire, and Hiro for their support and understanding. In particular, the Senior

Editor wishes to recognize his lifetime partner in all endeavors, Jane A. Avner, PhD for her extraordinary editorial

assistance. And finally, we thank our mentors, our students, and most importantly, our patients and their families.

Without them, our work would lack purpose.

Ellis D. Avner, MD

William E. Harmon, MD

Patrick Niaudet, MD

Norishige Yoshikawa, MD

Table of Contents

Volume 1

Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . v

Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xiii

Section 1 Development . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1

1 Embryology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

# Spri

Adrian S. Woolf . Jolanta E. Pitera

2 Glomerular Circulation and Function . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31

Tracy E. Hunley . Valentina Kon . Iekuni Ichikawa

3 Renal Tubular Development . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65

Michel Baum

4 Perinatal Urology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 95

Richard S. Lee, MD . David A. Diamond, MD

5 Renal Dysplasia/Hypoplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107

Paul Goodyer

6 Syndromes and Malformations of the Urinary Tract . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121

Chanin Limwongse

Section 2 Homeostasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 157

7 Sodium and Water . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159

Howard Trachtman

8 Potassium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185

Lisa M. Satlin

9 Acid-Base Homeostasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 205

Elizabeth Ingulli . Kirtida Mistry . Robert H. K. Mak

10 Calcium and Phosphorus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231

Anthony A. Portale . Farzana Perwad

11 Genetic Disorders of Calcium and Phosphate Homeostasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 267

Amita Sharma . Rajesh V. Thakker . Harald Juppner

nger-Verlag Berlin Heidelberg 2009

viii Table of Contents

12 Nutrition and Metabolism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 307

Lauren Graf . Corina Nailescu . Phyllis J. Kaskel . Frederick J. Kaskel

13 Fluid and Electrolyte Therapy in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325

Michael J. G. Somers

Section 3 Research Methods . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 355

14 Molecular Biology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 357

Francesco Emma . Luisa Murer . Gian Marco Ghiggeri

15 In Vitro Methods in Renal Research . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379

Patricia D. Wilson

16 Animal Models . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 397

Jordan Kreidberg

17 Clinical Investigation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 419

Susan L. Furth . Jeffrey J. Fadrowski

18 Genomic Methods in the Diagnosis and Treatment of Pediatric Kidney Disease . . . . . . . . . . . . . 441

Karen Maresso . Ulrich Broeckel

19 Tissue Engineering . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 457

Anthony Atala

Section 4 Clinical Methods . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 475

20 Clinical Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 477

Mohan Shenoy . Nicholas J. A. Webb

21 Laboratory Assessment and Investigation of Renal Function . . . . . . . . . . . . . . . . . . . . . . . . . . . . 491

Aaron Friedman

22 Evaluation of Growth and Development . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 505

Sandra Amaral . Alicia Neu

23 Diagnostic Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 535

Stephen F. Simoneaux . Larry A. Greenbaum

24 Renal Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 565

Agnes B. Fogo

Section 5 Glomerular Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 599

25 Congenital Nephrotic Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 601

Hannu Jalanko . Christer Holmberg

Table of Contents ix

26 Inherited Glomerular Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 621

Clifford E. Kashtan . Marie-Claire Gubler

27 Idiopathic Nephrotic Syndrome: Genetic Aspects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 643

Eduardo Machuca . Ernie L. Esquivel . Corinne Antignac

28 Idiopathic Nephrotic Syndrome in Children: Clinical Aspects . . . . . . . . . . . . . . . . . . . . . . . . . . . . 667

Patrick Niaudet . Olivia Boyer

29 Immune-mediated Glomerular Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 703

Michio Nagata

30 Acute Postinfectious Glomerulonephritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 743

Bernardo Rodrguez-Iturbe . Sergio Mezzano

31 Immunoglobulin A Nephropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 757

Koichi Nakanishi . Norishige Yoshikawa

32 Membranoproliferative Glomerulonephritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 783

Michael C. Braun . Christoph Licht . C. Frederic Strife

33 Membranous Nephropathy in the Pediatric Population . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 799

J. Ashley Jefferson . William G. Couser

34 Crescentic Glomerulonephritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 815

Arvind Bagga

Section 6 Tubular Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 829

35 Nephronophthisis and Medullary Cystic Kidney Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 831

Friedhelm Hildebrandt

36 Polycystic Kidney Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 849

Katherine MacRae Dell . William E. Sweeney . Ellis D. Avner

37 Aminoaciduria and Glycosuria . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 889

Israel Zelikovic

38 Tubular Disorders of Electrolyte Regulation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 929

Olivier Devuyst . Martin Konrad . Xavier Jeunemaitre . Maria-Christina Zennaro

39 Renal Tubular Acidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 979

Raymond Quigley

40 Nephrogenic Diabetes Insipidus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1005

Nine V. A. M. Knoers . Elena N. Levtchenko

41 Cystinosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1019

William A. Gahl

x Table of Contents

42 Fanconi Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1039

Takashi Igarashi

43 Primary Hyperoxaluria . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1069

Pierre Cochat . Sonia Fargue . Jerome Harambat

44 Tubulointerstitial Nephritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1081

Uri S. Alon

Volume 2

Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . v

Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xiii

Section 7 Systemic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1099

45 Vasculitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1101

Seza Ozen

46 Henoch-Schoenlein Purpura . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1111

Rosanna Coppo . Alessandro Amore

47 Systemic Lupus Erythematosus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1127

Patrick Niaudet . Remi Salomon

48 Hemolytic Uremic Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1155

S. Johnson . C. Mark Taylor

49 Sickle Cell Nephropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1181

Jon I. Scheinman

50 Diabetic Nephropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1199

M. Loredana Marcovecchio . Francesco Chiarelli

51 Renal Manifestations of Metabolic Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1219

William G. vant Hoff

52 Infectious Diseases and the Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1235

Jethro Herberg . Amitava Pahari . Sam Walters . Michael Levin

53 Nephrotoxins . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1275

Deborah P. Jones . Russell W. Chesney

Section 8 Urinary Tract Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1297

54 Urinary Tract Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1299

Albert Bensman . Olivier Dunand . Tim Ulinski

55 Vesicoureteral Reflux and Renal Scarring . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1311

Tej K. Mattoo . Ranjiv Mathews

Table of Contents xi

56 Obstructive Uropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1337

Robert L. Chevalier . Craig A. Peters

57 Bladder Dysfunction in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1379

Michael E. Mitchell, MD . Anthony H. Balcom, MD

58 Urolithiasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1405

Dawn S. Milliner

59 Pediatric Renal Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1431

Elizabeth A. Mullen . Christopher Weldon . Jordan A. Kreidberg

Section 9 Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1457

60 Epidemiology of Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1459

Midori Awazu

61 Pathophysiology of Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1485

Ikuyo Yamaguchi . Joseph T. Flynn

62 Evaluation of Hypertension in Childhood Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1519

Eileen D. Brewer

63 Management of the Hypertensive Child . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1541

Demetrius Ellis

Section 10 Acute Renal Failure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1577

64 Pathogenesis of Acute Renal Failure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1579

Rajasree Sreedharan . Prasad Devarajan . Scott K. Van Why

65 Clinical Evaluation of Acute Kidney Injury in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1603

Sharon P. Andreoli

66 Management of Acute Kidney Failure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1619

Michael Zappitelli . Stuart L. Goldstein

Section 11 Chronic Renal Failure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1629

67 Pathophysiology of Progressive Renal Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1631

Allison Eddy

68 Management of Chronic Kidney Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1661

Rene G. VanDeVoorde . Bradley A. Warady

69 Handling of Drugs in Children with Abnormal Renal Function . . . . . . . . . . . . . . . . . . . . . . . . . . 1693

Ihab M. Wahba . Ali J. Olyaei . David Rozansky . William M. Bennett

70 Endocrine and Growth Disorders in Chronic Kidney Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . 1713

Franz Schaefer

xii Table of Contents

71 Chronic Kidney Disease Mineral and Bone Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1755

Katherine Wesseling-Perry . Isidro B. Salusky

72 Peritoneal Dialysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1785

Enrico Verrina

73 Hemodialysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1817

Lesley Rees

74 Transplantation Immunobiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1835

Elizabeth Ingulli . Stephen I. Alexander . David M. Briscoe

75 Pediatric Kidney Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1867

William E. Harmon

76 Immunosuppression in Pediatric Renal Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1903

Jodi M. Smith . Thomas L. Nemeth . Ruth A. McDonald

77 Complications of Renal Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1919

Vikas R. Dharnidharka . Carlos E. Araya

Section 12 Pediatric Nephrology Around The World . . . . . . . . . . . . . . . . . . . . 1941

78 Overview . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1943

Isidro B. Salusky . Matthias Brandis . Ira Greifer

79 Europe . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1947

Christer Holmberg

80 Japan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1951

Takashi Igarashi

81 Pediatric Nephrology Around the World North America . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1955

Lisa M. Satlin . Sharon P. Andreoli . H. William Schnaper

82 Africa . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1961

Felicia U. Eke

83 Latin America . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1969

Nelson Orta-Sibu . Ramon A Exeni . Clotilde Garcia

84 Pediatric Nephrology in Australia and New Zealand . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1975

William Wong . Stephen Alexander

85 Pediatric Nephrology in Asia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1981

Hui-Kim Yap . Arvind Bagga . Man-Chun Chiu

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1991

List of Contributors

Stephen I Alexander

Paediatrics and Child Health

The University of Sydney

Division of Nephrology

Childrens Hospital at Westmead

Sydney

Australia

Uri S Alon

The Childrens Mercy Hospital

Section of Nephrology

2401 Gillham Road

Kansas City, MO 64108

USA

[email protected]

Sandra Amaral

Division of Pediatric Nephrology

Emory Healthcare &

Childrens Healthcare of Atlanta

2015 Uppergate Drive NE

Atlanta, GA 30322

USA

[email protected]

Alessandro Amore

University of Turin

Dialysis and Transplantation Unit

Regina Margherita Childrens

University Hospital

Turin

Italy

[email protected]

Rene G anDeVoorde


Childrens Mercy Hospital

Kansas, MO

USA

# Springer-Verlag Berlin Heidelberg 2009

Sharon P Andreoli

Indians University Medical Center


Riley Research Room 234

699 West Street

Indianapolis, IN 46077

USA

[email protected]

Corinne Antignac

Inserm, U574

6eme etage, Tour Lavoisier

Hopital Necker-Enfants Malades

149 rue de Sevres

75015 Paris

France

[email protected]

Carlos E Araya


University of Florida

College of Medicine

Gainesville, FL

USA

Anthony Atala

Department of Urology

Wake Forest Institute for Regenerative Medicine

Medical Center Boulevard

Winston-Salem, NC 27157

USA

[email protected]

Ellis D Avner

Childrens Research Institute, CHHS


Wisconsin

USA

[email protected]

xiv List of Contributors

Midori Awazu


Keio University School of Medicine

35 Shinanomachi, Shinjuku-ku

Tokyo 1608582Japan

[email protected]

Arvind Bagga


All India Institute of Medical Sciences

New Delhi

India

[email protected]

Anthony H Balcom

Division of Pediatric Urology

Childrens Hospital of Wisconsin

Milwaukee

USA

Michel Baum


U.T. Southwestern Medical Center

5323 Harry Hines Blvd.

Dallas, Texas 752359063USA

[email protected]

William M Bennett

Northwest Renal Clinic

Legacy Good Samaritan Hospital

Portland, OR

USA

[email protected]

Albert Bensman

Department of Pediatric Nephrology

Armand Trousseau Hospital (APHP)

Paris, Cedex 12

France

[email protected]

Olivia Boyer



Paris

France

Matthias Brandis

Department of Pediatrics and Adolescent Medicine

Freiburg University Hospital

Freiburg

Germany

[email protected]

Michael C Braun

University of Texas Health Science Center

Division of Nephrology and Hypertension

Institute of Molecular Medicine

1825 Pressler St

Houston, TX 77030

USA

[email protected]

Eileen D Brewer

American Society of Pediatric Nephrology

Texas Childrens Hospital

Houston, TX

USA

[email protected]

David M Briscoe


Harvard Medical School



Boston, MA

USA

[email protected]

Ulrich Broeckel

Childrens Research Institute


8701 Watertown Plank Rd.

Milwaukee, WI 53226

USA

[email protected]

Russell W Chesney

The University of Tennessee

College of Medicine - Dept. of Pediatrics

Le Bonheur Childrens Medical Center

50 North Dunlap

Memphis, TN

USA

[email protected]

Robert L. Chevalier

Department of Pediatrics Box 800386

University of Virginia Health System

Charlottesville, VA 22908

USA

[email protected]

List of Contributors xv

Francesco Chiarelli


University of Chieti

Chieti

Italy

Man-Chun Chiu


National University of Singapore

Singapore

Pierre Cochat

Service de pediatrie

Hopital Femme Mere Enfant

59 boulevard Pinel

69677 Bron

France

[email protected]

Rosanna Coppo

Post-Graduated School of Nephrology

Post-Graduated School of Paediatrics

University of Turin

Turin

Italy

[email protected]

William G Couser

Affiliate Professor of Medicine


University of Washington

16050 169th Ave NE

Woodinville, WA 98072

USA

[email protected]

Katherine MacRae Dell



Rainbow Babies and Childrens Hospital and

Case Western Reserve University

11100 Euclid Avenue, RBC 787

Cleveland, OH 44106

USA

[email protected]

Prasad Devarajan



8701 Watertown Plank Road

MACC Fund Research Center

Milwaukee, Wisconsin 532260509

USA

Olivier Devuyst


Universite catholique de Louvain Medical School

B1200 BrusselsBelgium

[email protected]

Vikas R Dharnidharka


University of Florida

College of Medicine

Gainesville, FL

USA

[email protected]

David A Diamond




Boston, MA

USA

[email protected]

Olivier Dunand



Paris, Cedex 12

France

Allison Eddy



Center for Tissue and Cell Sciences

Childrens Hospital & Regional Medical Center

4800 Sand Point Way NE, A7931Seattle, WA 98105

USA

[email protected]

Felicia U Eke

Department of Paediatrics

University of Port Harcourt Teaching Hospital

Port Harcourt

Nigeria

[email protected]

xvi List of Contributors

Demetrius Ellis


Childrens Hospital of Pittsburgh

3705 Fifth Avenue

Pittsburgh, PA

USA

[email protected]

Francesco Emma

Division of Nephrology and Dialysis

Bambino Gesu Childrens Hospital and Research Institute

Rome

Italy

[email protected]

Ernie L Esquivel

Universite Paris Descartes

Faculte de Medecine Paris Descartes

Paris

France

Ramon A Exeni

Service of Pediatric Nephrology

Childrens Hospital and University of Carabobo

Apartado 3273

Valencia

Estado Carabobo

Venezuela

Jeffrey J Fadrowski

Dept. of Pediatrics, Epidemiology

Johns Hopkins University

Welch Center f. Prevention Epidemiology

2024 E. Monument Street

Baltimore, MD

USA

Sonia Fargue

Centre de reference des maladies renales rares

Hospices Civils de Lyon

Lyon

France

Joseph T Flynn


Pediatric Hypertension Program


Childrens Hospital and Regional Medical Center

Seattle, WA

USA

[email protected]

Agnes B Fogo

MCN C3310

Department of Pathology

Vanderbilt University Medical Center

Nashville, TN 37232

USA

[email protected]

Aaron Friedman


University of Minnesota

Minneapolis

Minnesota

USA

[email protected]

Susan L Furth

Department of Pediatrics, Epidemiology

Johns Hopkins University

Welch Center for Prevention Epidemiology

2024 E. Monument Street

Baltimore, MD

USA

[email protected]

William A Gahl

Section on Human Biochemical Genetics

Medical Genetics Branch

National Human Genome Research Institute

National Institutes of Health

Bethesda, Maryland 208921851USA

[email protected]

Clotilde Garcia



Apartado 3273

Valencia

Estado Carabobo

Venezuela

Gian Marco Ghiggeri

Division of Nephrology, Dialysis and Transplantation

Giannina Gaslini Childrens Hospital and Research Institute

Genoa

Italy

Stuart Goldstein

Pediatrics, Renal Section

Texas Childrens Hospital

List of Contributors xvii

Baylor College of Medicine

6621 Fannin, MC32482Houston, TX 77030

USA

[email protected]

Paul Goodyer

Montreal Childrens Hospital


2300 Tupper Street

Montreal, Quebec H3H 1P3

Canada

[email protected]

Larry A Greenbaum


Emory University School of Medicine

2015 Uppergate Drive, NE

Atlanta, GA 30322

USA

[email protected]

Ira Greifer

Albert Einstein College of Medicine of Yeshiva University

Monte Fiore Medical Center

Bronx

New York

USA

Marie-Claire Gubler


University of Minnesota Medical School

420 Delaware Street SE

Minneapolis, MN

USA

Jerome Harambat

Service de pediatrie

Bordeaux

France

William E Harmon




300 Longwood Avenue

Boston, MA 02115

USA

[email protected]

Friedhelm Hildebrandt

Departments of Pediatrics and of Human Genetics

Howard Hughes Medical Institute

University of Michigan

1150 W Medical Ctr Dr

Ann Arbor, Michigan 481095646USA

[email protected]

William G ant Hoff

Great Ormond Street Hospital for Children

Great Ormond Street Hospital

London WC1N 3JH

UK

[email protected]

Christer Holmberg

Department of Pediatric Nephrology and Transplantation

Helsinki University Central Hospital

PO 281 Hospital District of Helsinki and UUsimaa

Helsinki

Finland

[email protected]

Tracy E Hunley


Tokai University Hospital

Boseidai, Isehara

Kanagawa

Japan

Iekuni Ichikawa



Boseidai, Isehara

Kanagawa

Japan

[email protected]

Takashi Igarashi

Graduate School of Medicine

The University of Tokyo

Tokyo

Japan

[email protected]

Elizabeth Ingulli


University of California

San Diego

USA

xviii List of Contributors

Hannu Jalanko

Hospital for Children and Adolescents

University of Helsinki

PO 281 Hospital District of Helsinki and UUsimaa

Helsinki

Finland

[email protected]

J Ashley Jefferson



Box 356521

1959 NE Pacific Street

Seattle, WA 98195

USA

Xavier Jeunemaitre

Department of Molecular Genetics

Hopital Europeen George Pompidou

Paris

France

[email protected]

S Johnson

Birmingham Childrens Hospital

University of Birmingham

Birmingham

UK

Deborah P Jones

The University of Tennessee

College of Medicine - Department of Pediatrics

Le Bonheur Childrens Medical Center

50 North Dunlap

Memphis, TN

USA

[email protected]

Harald Juppner

Endocrine Unit and Pediatric Nephrology Unit

Departments of Medicine and Pediatrics

Massachusetts General Hospital and Harvard Medical School

Boston, MA

USA

[email protected]

Clifford E Kashtan


University of Minnesota Medical School

420 Delaware Street SE

Minneapolis, MN

USA

[email protected]

Frederick J Kaskel

Section on Nephrology

111 East 210th Street

Childrens Hospital at Montefiore

Albert Einstein College of Medicine

Bronx

New York 104672490USA

[email protected]

Nine V A M Knoers

Departments of Human Genetics

Radboud University Nijmegen Medical Centre

PO Box 9101

6500 HB Nijmegen

The Netherlands

[email protected]

Valentina Kon



Boseidai, Isehara

Kanagawa

Japan

Martin Konrad


Munster University Childrens Hospital

Munster

Germany

[email protected]

Jordan Kreidberg

Department of Nephrology


Boston, MA 2115

USA

[email protected]

Richard S Lee




Boston, MA

USA

List of Contributors xix

Michael Levin


Imperial College (St Marys Campus)

London

UK

[email protected]

Elena N Levtchenko


University Hospital Leuven

Herestraat 49

B3000 LeuvenBelgium

[email protected]

Christoph Licht


The Hospital for Sick Children

University of Toronto

555 University Ave

Toronto, ON M5G 1X8

Canada

[email protected]

Chanin Limwongse

2 Prannok Rd

Division of Medical Genetics

Department of Medicine

Faculty of Medicine Siriraj Hospital

Mahidol University

Bangkoknoi

Bangkok 10700

Thailand

[email protected]

Eduardo Machuca

Inserm

U574


Paris

France

Robert H K Mak



San Diego

USA

[email protected]

Loredana Marcovecchio


University of Chieti

Chieti

Italy

[email protected]

Karen Maresso

Rm C2388


8701 Watertown Plank Rd.

Milwaukee, WI 53226

USA

[email protected]

Ranjiv Mathews


The Johns Hopkins School of Medicine

Brady Urological Institute

Baltimore, MD

USA

Tej K Mattoo

Wayne State University School of Medicine

Pediatric Nephrology and Hypertension

Childrens Hospital of Michigan

Detroit, MI 48201

USA

[email protected]

Ruth A McDonald


Seattle Childrens University of Washington

Seattle, WA

USA

[email protected]

Sergio Mezzano

Unversidad Austral

Valdivia

Chile

Dawn S Milliner

Mayo Clinic

200 First Street, SW

Rochester, MN 55905

USA

[email protected]

xx List of Contributors

Kirtida Mistry



San Diego

USA

Michael E Mitchell


Childrens Hospital of Wisconsin

Milwaukee

USA

[email protected]

Elizabeth A Mullen


Boston, MA

USA

[email protected]

Luisa Murer

Division of Pediatric Nephrology, Dialysis and Transplantation


University of Padua

Padua

Italy

Michio Nagata

Renal and Vascular Pathology

Graduate School of Comprehensive Human Sciences

University of Tsukuba

Japan

[email protected]

Koichi Nakanishi

Nephrology Division



8111 Kimiidera, Wakayama CityJapan [email protected]

Thomas L Nemeth

Department of Pharmacy


Seattle, WA

USA

Alicia M Neu


Johns Hopkins University School of Medicine

200 North Wolfe Street

David M. Rubenstein Child Health Bldg

Baltimore, MD 212872535USA

[email protected]

Patrick Niaudet


Centre de reference des Maladies Renales Hereditaires de

lEnfant et de lAdulte (MARHEA)


149, rue de Sevres

75743 Paris Cedex 15

France

[email protected]

Ali J Olyaei

Division of Nephrology & Hypertension

Oregon Health Sciences University

3314 SW US Veterans Hospital Road

Portland, Oregon 97201

USA

[email protected]

Nelson Orta-Sibu



Apartado 3273

Valencia

Estado Carabobo

Venezuela

[email protected]

Seza Ozen


Hacettepe University

Ankara

Turkey

[email protected]

Amitava Pahari



London

UK

Farzana Perwad




San Francisco

San Francisco, CA 941430748USA

List of Contributors xxi

Craig A Peters

Department of Urology Box 800422

University of Virginia Health System

Charlottesville, VA 22908

USA

[email protected]

Jolanta E Pitera

Nephro-Urology Unit

University College London Institute of Child Health

30 Guilford Street

London WC1N 1EH

UK

Anthony A Portale




San Francisco

San Francisco, CA 941430748USA

[email protected]

Raymond Quigley


UT Southwestern Medical Center

5323 Harry Hines Blvd.

Dallas

TX 753909063USA

[email protected]

Lesley Rees

Nephrourology Unit

Great Ormond Street Hospital for Children NHS Trust

London WC1N 3JH

UK

[email protected]

Bernardo Rodrguez-Iturbe

Apartado postal 1430

Maracaibo, Estado Zulia 4001-A

Venezuela

[email protected]

David Rozansky



Oregon Health and Science University

Portland, Oregon 97201

USA

Remi Salomon



149, rue de Sevres

75743 Paris, Cedex 15

France

Isidro B Salusky

10833 Le Conte Avenue, Box 951752

General Clinical Research Center

David Geffen School of Medicine at UCLA

Los Angeles, CA 900951752USA

[email protected]

Lisa M Satlin


Mount Sinai School of Medicine

One Gustave L. Levy Place

New York

NY 100296574USA

[email protected]

Franz Schaefer


University Childrens Hospital

Im Neuenheimer Feld 430

69120 Heidelberg

Germany

[email protected]

Jon I Scheinman


The University of Kansas Medical Center

3901 Rainbow Blvd

Kansas City KS

USA

[email protected]

H William Schnaper

Pediatrics

Feinberg School of Medicine, Northwestern University

Chicago, IL

USA

[email protected]

Amita Sharma

Pediatric Nephrology Unit

Departments of Pediatrics

Massachusetts General Hospital and Harvard Medical School

xxii List of Contributors

Boston, MA

USA

[email protected]

Mohan Shenoy

Department of Paediatric Nephrology

Royal Manchester Childrens Hospital

Pendlebury

Manchester M27 4HA

UK

Stephen Simoneaux


Emory University School of Medicine

2015 Uppergate Drive, NE

Atlanta, GA 30322

USA

Jodi M Smith


Seattle Childrens University of Washington

Seattle, WA

USA

Michael J G Somers




Boston, Massachusetts

USA

[email protected]

Rajasree Sreedharan





Milwaukee, Wisconsin 532260509USA

C Frederic Strife

University of Cincinnati College of Medicine

Division of Nephrology and Hypertension

MLC 7022

Cincinnati Childrens Hospital Medical Center

3333 Burnet Ave

Cincinnati, OH 45229

USA

[email protected]

William E Sweeney, Jr

Departments of Pediatrics and Physiology



Childrens Hospital Health System of Wisconsin

Childrens Corporate Center

999 N.92nd Street

Wauwatosa, WI 53226

USA

[email protected]

C Mark Taylor

Department of Nephrology

The Birmingham Childrens Hospital

Birmingham B4 6NH

UK

[email protected]

Howard Trachtman


Long Island Campus for the Albert Einstein College of

Medicine

New Hyde Park

New York

USA

[email protected]

Tim Ulinski



Paris, Cedex 12

France

Enrico Verrina

Nephrology and Dialysis Unit

G. Gaslini Institute

Genova 16148

Italy

[email protected]

Ihab M Wahba

Samaritan Medical Group

Corvallis

Oregon

USA

Sam Walters



London

UK

Bradley A Warady


Childrens Mercy Hospital

List of Contributors xxiii

Kansas, MO

USA

[email protected]

Nicholas Webb

Department of Paediatric Nephrology

Royal Manchester Childrens Hospital

Pendlebury

Manchester M27 4HA

UK

[email protected]

Christopher Weldon


Boston, MA

USA

Katherine Wesseling-Perry


David Geffen School of Medicine at UCLA

Los Angeles

USA

Scott K Van Why





Milwaukee, Wisconsin 532260509USA

[email protected]

Patricia Wilson




Milwaukee, WI 53226

USA

[email protected]

William Wong

Renal Unit

Starship Childrens Hospital

Private Bag 92024

Auckland

New Zealand

[email protected]

Adrian S Woolf

Nephro-Urology Unit

University College London Institute of Child Health

30 Guilford Street

London WC1N 1EH

UK

[email protected]

Ikuyo Yamaguchi



Childrens Hospital and Regional Medical Center

Seattle, WA

USA

Hui-Kim Yap


National University of Singapore

Singapore

[email protected]

Norishige Yoshikawa



8111 KimiideraWakayama City

Japan [email protected]

Michael Zappitelli


Montreal Childrens Hospital

McGill University Health Center

Montreal

Quebec, H3H1P3

Canada

[email protected]

Israel Zelikovic

Faculty of Medicine

Israel Institute of Technology


8 HaAliyah St.

Rambam Medical Center

Haifa 31096

Israel

[email protected]

Maria-Christina Zennaro

Inserm U772

College de France

Paris

France

[email protected]

1Halftitle.pdfPediatric Nephrology

2Title.pdfPediatric Nephrology

4Preface.pdfPreface

5TOC.pdfTable of Contents

6LOC.pdfList of Contributors

pediatric nephrology - springer978-3-540-76341-3/1 · preface through its past ﬁve editions,...

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