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 ContentsPreface: A. PATHOPHYSIOLOGY & DEVELOPMENT 1. Embryology and development of the ductal plate 2. Cholangiocyte biology as relevant to cystic liver diseases. 3. Cholangiocyte cilia and basal bodies 4. Genetics of fibrocystic diseases of the liver and molecular approaches to therapy. B. DIAGNOSIS 5. Radiologic findings in the fibrocystic diseases. 6. Pathology of fibrocystic diseases of the liver. C. DISEASE STATES 7. Alagille syndrome and JAGGED1/NOTCH sequence. 8. Biliary atresia and the ductal plate. 9. Nephronopthisis and Renal-hepatic-pancreatic dysplasia of Ivemark 10. Meckel and Joubert syndromes 11. Bardet-Biedel, and Jeune Syndromes 12. Congenital disorder of glycosylation and their effects on the liver. 13. Autosomal recessive polycystic kidney disease. 14. Caroli disease, Caroli syndrome, and congenital hepatic fibrosis. 15. Simple hepatic cysts/ Choledochal cysts 16. Autosomal dominant polycystic liver disease. 17. Echinococcal/Hydatid Cysts of the liver. 18 . Miscellaneous cystic lesions of the liver. D. COMPLICATIONS 19. Vascular complications of fibrocystic liver disease. 20. Biliary cystadenoma and cystadenocarcinoma 21. Cholangitis G. TREATMENT 22. Surgical Management of Fibrocystic Liver Disease