A case of 
Congenital Hepatic fibrosis

Keimyung Medical Journal 1999;18(3):470-477.

A case of Congenital Hepatic fibrosis

선천성 간섬유증 1례

곽진영; 강진무; 박근수; 김명성; 김흥식

Abstract

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 withm 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 nephron-ophthisis We present this case with the brief review of literature

Key Words: Congenital hepatic fibrosis, Nephronophthisis