InhaltsangabePreface: 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