Fibrocystic Diseases of the Liver / Edition 1

Fibrocystic Diseases of the Liver / Edition 1

by Karen F. Murray
     
 

ISBN-10: 1603275231

ISBN-13: 9781603275231

Pub. Date: 03/05/2010

Publisher: Springer-Verlag New York, LLC

In recent years there have been huge advances in the understanding of the genetic and molecular basis of the fibrocystic diseases. This volume provides a thorough review of fibrocyctic diseases that affect the liver. It contains in-depth discussions of the genetics, molecular biology, pathogenesis, histology, clinical presentations, complications of, treatment, and

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Overview

In recent years there have been huge advances in the understanding of the genetic and molecular basis of the fibrocystic diseases. This volume provides a thorough review of fibrocyctic diseases that affect the liver. It contains in-depth discussions of the genetics, molecular biology, pathogenesis, histology, clinical presentations, complications of, treatment, and prognosis of the conditions affecting children and adults, and hence will be the gold-standard reference for these conditions. In addition, the histological features that distinguish these conditions from other potentially fibrosing hepatopathies are illustrated. Conditions with syndromic features involving the kidney or other organ systems are also reviewed. Thorough review of the clinical phenotypes, their presentations, treatment, potential complications of, and prognosis is discussed. Fibrocystic Diseases of the Liver will be an invaluable resource for hepatologists, gastroenterologists, nephrologists, and hepatic surgeons who care for children and adults with liver disease, as well as basic scientists in molecular genetics, hepatobiliary pathophysiology, hepatology and nephrology.

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Product Details

ISBN-13:
9781603275231
Publisher:
Springer-Verlag New York, LLC
Publication date:
03/05/2010
Series:
Clinical Gastroenterology Series
Edition description:
2010
Pages:
512
Product dimensions:
6.10(w) x 9.20(h) x 1.30(d)

Table of Contents

Preface vii

Contents ix

Contributors xi

Part I Pathophysiology and Development 1

1 Embryology and Development of the Ductal Plate Clifford W. Bogue 3

2 Cholangiocyte Biology as Relevant to Cystic Liver Diseases Silvia Lecchi Luca Fabris Carlo Spirli Massimiliano Cadamuro Romina Fiorotto Mario Strazzabosco 23

3 Cholangiocyte Cilia and Basal Bodies Tatyana V. Masyuk Anatoliy I. Masyuk Nicholas F. LaRusso 45

4 Genetics of Fibrocystic Diseases of the Liver and Molecular Approaches to Therapy Maya Tuchman William A. Gahl Meral Gunay-Aygun 71

Part II Diagnosis 103

5 Radiologic Findings in the Fibrocystic Diseases Orpheus Kolokythas Grace Phillips 105

6 Pathology of Fibrocystic Diseases of the Liver Camtu D. Nguyen Truong Matthew M. Yeh 133

Part III Disease States 157

7 Alagille Syndrome and JAGGED1/NOTCH Sequence Binita M. Kamath Kathleen M. Loomes David A. Piccoli 159

8 Biliary Atresia and the Ductal Plate Ronald J. Sokol Cara L. Mack 179

9 Nephronophthisis and Renal-Hepatic-Pancreatic Dysplasia of Ivemark David A. Myers Jordan M. Symons 201

10 Meckel and Joubert Syndromes Melissa A. Parisi Laura S. Finn Ian A. Glass 221

11 Bardet-Biedl and Jeune Syndromes Aoife M. Waters Philip L. Beales 257

12 Congenital Disorders of Glycosylation and Their Effects on the Liver Erik A. Eklund Hudson H. Freeze 287

13 Autosomal Recessive Polycystic Kidney Disease Jodi M. Smith Ruth A. McDonald 319

14 Caroli Disease, Caroli Syndrome, and Congenital Hepatic Fibrosis Ronen Arnon Henrietta Kotlus Rosenberg Frederick J. Suchy 331

15 Simple Hepatic Cysts/Choledochal Cysts André A. S. Dick Patrick J. Healey 359

16 Autosomal Dominant Polycystic Liver Disease Qi Qian Vicente E. Torres Stefan Somlo 371

17 Echinococcal/Hydatid Cysts of the Liver Paul S. Pottinger Austin Spitzer Raymond R. Yeung 387

18 Miscellaneous Cystic Lesions of the Liver Iris W. Liou 411

Part IV Complications 433

19 Vascular Complications of Fibrocystic Liver Disease Maarouf Hoteit Patrick S. Kamath 435

20 Biliary Cystadenoma and Cystadenocarcinoma Paolo Del Poggio Ezio Pezzica Daniela Corti 445

21 Cholangitis Shayan Irani Richard Kozarek 463

Part V Surgical Management 481

22 Surgical Management of Fibrocystic Liver Disease Meelie DebRoy 483

Subject Index 495

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