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The Korean Journal of Pathology 1991;25(1): 50-53.
Congenital Hepatic Fibrosis: A case report.
Weon Young Choi, Sun Hee Yoon, In Sook Lim, Ha Jin Choi
1Department of Pathology, School of Medicine, Inje University, Pusan, Korea.
2Department of Internal Medicine, School of Medicine, Inje University, Pusan, Korea.
ABSTRACT
Congenital hepatic fibrosis is an uncommon disease of children and young adults with two major risks: gastrointestinal hemorrhage caused by portal hypertension, and cholangitis related to bacterial infection of dilated intrahepatic bile ducts. It is characterizeed by stony hard hepatomegaly and portal hypertension with rather well preserved hepatic function and architecture, and frequent association of the renal lesions. We have recently experienced a case of congenital hepatic fibrosis in a 24 year-old Korean male. The chief complaint was hematemesis from esophageal varices. There were marked hepatosplenomegaly, mild pancytopenia and the liver function test was within normal engorgement and dilatation of portal and splenic veins and multiple cysts of both kidneys.
Key Words: Hepatic fibrosis; Congenital hepatic fibrosis
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