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Main Data
Author: Karen F. Murray, Anne M. Larson
Editor: Karen F. Murray, Anne M. Larson
Title: Fibrocystic Diseases of the Liver
Publisher: Humana Press
ISBN/ISSN: 9781603275248
Edition: 1
Price: CHF 306.70
Publication date: 01/01/2010
Content
Category: Medizin & Pharmazie
Language: English
Technical Data
Pages: 512
Kopierschutz: DRM
Geräte: PC/MAC/eReader/Tablet
Formate: PDF
Table of contents

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
Preface8
Contents10
Contributors12
Part I Pathophysiology and Development16
1 Embryology and Development of the Ductal Plate17
Introduction18
Liver Specification and Budding18
Overview of Biliary Tract Development21
Hepatoblast Differentiation into Biliary Epithelial Cells23
Biliary Tract Morphogenesis27
Cilia A Potential Link Between Development and Disease30
References32
2 Cholangiocyte Biology as Relevant to Cystic Liver Diseases36
Introduction37
Morphology and Secretory Functions of the Normal Biliary Epithelium39
Cholangiocyte Reaction to Damage42
Mechanisms of Cystic Liver Diseases: Cilia and Beyond44
Cellular Mechanisms of Liver Cyst Formation and Growth46
Altered Biliary Developmental Program (Ductal Plate Malformation)46
Altered Epithelial Fluid Secretion47
Cholangiocyte Proliferation48
Autocrine and Paracrine VEGF Signaling49
Extracellular Matrix Remodeling51
New Therapeutic Strategies in Preventing Cyst Growth52
References52
3 Cholangiocyte Cilia and Basal Bodies57
General Introduction to Cilia58
Structure of Mammalian Cilia59
Cilia in Normal Cholangiocytes61
Cilia in Cholangiociliopathies63
Ciliogenesis66
Centrosome, Centrioles, and Pericentriolar Material66
Centrosome Functions67
Centriole Duplication68
Centriole Migration and Docking68
Formation of the Ciliary Axoneme68
Intraflagellar Transport (IFT)69
Ciliogenesis in Cholangiocytes69
Functions of Cholangiocyte Cilia70
Cholangiocyte Cilia as Mechanosensors70
Cholangiocyte Cilia as Chemosensors71
Cholangiocyte Cilia as Osmosensors72
Ciliary Hypothesis of Cystogenesis74
Conclusion75
References76
4 Genetics of Fibrocystic Diseases of the Liver and Molecular Approaches to Therapy83
Introduction84
Autosomal Dominant Polycystic Kidney Disease (ADPKD)92
Autosomal Recessive Polycystic Kidney Disease (ARPKD)94
Autosomal Dominant Polycystic Liver Disease (ADPLD)95
Meckel (Gruber) Syndrome (MKS)97
Joubert Syndrome98
BardetBiedl Syndrome (BBS)99
Alagille Syndrome101
Nephronophthisis (NPHP)102
Jeune Asphyxiating Thoracic Dystrophy103
Oral-Facial-Digital Syndrome103
Molecular Approaches to Therapy103
References107
Part II Diagnosis115
5 Radiologic Findings in the Fibrocystic Diseases116
Biliary Atresia117
Autosomal Recessive Po