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Two Cases of Congenital Hepatic Fibrosis

Journal of the Korean Pediatric Society 1992;35(4):569-574.
Published online April 15, 1992.
Two Cases of Congenital Hepatic Fibrosis
Cue Jung Hwang, Young Hun Kim, Dae Kyun Koh, Byung Churl Lee
Department of Pediatrics, Catholic University Medical College, Seoul, Korea
선천성 간섬유증 2례
황규정, 김영훈, 고대균, 이병철
가톨릭의과대학 소아과학교실
Abstract
The congenital hepatic fibrosis is a rare condition defined pathologically by the presence within the line of bands of fibrous tissue which after contain linear or circular spaces lined by bile duct cells, It is commonly associated with intrahepatic portal hypertension but hepatocellular function is almost always preserved. The prinicipal clinical features of this disease are abdominal distension, firm hepatomegaly, splenomegaly, hypersplenism and hematemesis or melena due to alimentary bleeding secondary to portal hypertension. We experienced two cases of congenital hepatic fibrosis confirmed by liver biopsy and present with the brief review of related literature.
Key Words: Congenital hepatic fibrosis


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