선천성 간섬유증 1례 ; A case of Congenital Hepatic fibrosis

Congenital hepatic fibrosis is an unusual congenital disorder of the liver of children, and characterized by hard hepatomegaly, portal hypertension with relative preservation of the liver function and underlying architecture, and frequent renal involvement. Histologically the liver is characterized by portal fibrosis and an increase in portal ductal structure but the hepatocytes are not affected. We have experienced a case of congenital hepatic fibrosis in 5 year old girl. The patient's chief complaints were fever, epigastric pain and abdominal distension. There are marked hepatomegaly and routine CBC. transaminase and bilirubin were within normal limits. Abdominal ultrasonography and CT scan reveal diffusely cystic dilatation of the intrahepatic bile ducts and numerous small calculi in the both medullary portion of kidney(nephronophthisis). Liver biopsy was performed. The microscopic findings are characterized that hepatic lobules surrounded by dense fibrous connective tissue and bile duct hyperplasia, but hepatic parenchyme is normal. Congenital hepatic fibrosis was confirmed by liver biopsy and also she had nephronophthisis. We present this case with the brief review of literature. ; open