[Cholangiodysplastic pseudocirrhosis].
Non-cystic cholangiodysplastic pseudocirrhosis of the liver was found in a 7 month old infant. Hepatomegaly was the first clinical sign. The cause of this disease entity ist believed to be a developmental disturbance of the small bile ducts. The etiology is unknown and the clinical course is hard to control. The transition to cirrhosis and the development of hepatocellular carcinoma are described.
D001652:Bile Ducts / Q000002:abnormalities*; D001653:Bile Ducts, Intrahepatic / Q000473:pathology; D001706:Biopsy; D003937:Diagnosis, Differential; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D007223:Infant; D008103:Liver Cirrhosis / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D008107:Liver Diseases / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D008297:Male
Cholangiodysplastische Pseudozirrhose.