Fibrocystic Diseases of the Liver

by Murray, Karen Field, M.D.; Larson, Anne M.

• ISBN13: 9781603275231
• ISBN10: 1603275231
• Edition: 1st
• Format: Hardcover
• Copyright: 2010-04-07
• Publisher: Humana Pr Inc

Summary

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.

Table of Contents

Preface	p. vii
Contents	p. ix
Contributors	p. xi
Pathophysiology and Development	p. 1
Embryology and Development of the Ductal Plate	p. 3
Cholangiocyte Biology as Relevant to Cystic Liver Diseases	p. 23
Cholangiocyte Cilia and Basal Bodies	p. 45
Genetics of Fibrocystic Diseases of the Liver and Molecular Approaches to Therapy	p. 71
Diagnosis	p. 103
Radiologic Findings in the Fibrocystic Diseases	p. 105
Pathology of Fibrocystic Diseases of the Liver	p. 133
Disease States	p. 157
Alagille Syndrome and JAGGED1/NOTCH Sequence	p. 159
Biliary Atresia and the Ductal Plate	p. 179
Nephronophthisis and Renal-Hepatic-Pancreatic Dysplasia of Ivemark	p. 201
Meckel and Joubert Syndromes	p. 221
Bardet-Biedl and Jeune Syndromes	p. 257
Congenital Disorders of Glycosylation and Their Effects on the Liver	p. 287
Autosomal Recessive Polycystic Kidney Disease	p. 319
Caroli Disease, Caroli Syndrome, and Congenital Hepatic Fibrosis	p. 331
Simple Hepatic Cysts/Choledochal Cysts	p. 359
Autosomal Dominant Polycystic Liver Disease	p. 371
Echinococcal/Hydatid Cysts of the Liver	p. 387
Miscellaneous Cystic Lesions of the Liver	p. 411
Complications	p. 433
Vascular Complications of Fibrocystic Liver Disease	p. 435
Biliary Cystadenoma and Cystadenocarcinoma	p. 445
Cholangitis	p. 463
Surgical Management	p. 481
Surgical Management of Fibrocystic Liver Disease	p. 483
Subject Index	p. 495
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