title: [Congenital cystic dilatation of the intrahepatic bile ducts. Caroli's disease and its complications].
abstract: Two cases with congenital dilatations of the intrahepatic bile ducts are reported. The clinical, diagnostic and therapeutic aspects and possible complications of this rare disease are discussed. The well-known frequent occurrence of cholangiocarcinoma in Caroli's disease is illustrated by one of our patients. Because of the limited therapeutic possibilities, the prognosis of this illness remains unfavourable.
mesh_terms: D000328:Adult; D001650:Bile Duct Neoplasms / Q000209:etiology*; D001653:Bile Ducts, Intrahepatic; D001660:Biliary Tract Diseases / Q000150:complications / Q000151:congenital*; D002761:Cholangitis / Q000188:drug therapy; D002763:Cholecystectomy; D002779:Cholestasis / Q000209:etiology; D003560:Cysts / Q000175:diagnosis; D006801:Humans; D006932:Hyperbilirubinemia / Q000209:etiology; D008297:Male; D013256:Steroids / Q000627:therapeutic use
chemical_list: D013256:Steroids
vernacular_title: Kongenitale zystische Dilatation der intrahepatischen Gallenwege. Carolische Krankheit und ihre Komplikationen.