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A Case of Congenital Hepatic Fibrosis with Hypersplenism.

Journal of the Korean Pediatric Society 1987;30(12):1456-1460.
Published online December 31, 1987.
A Case of Congenital Hepatic Fibrosis with Hypersplenism.
Hye Suk Hong, Yang Won Lee, Keon su Rhee, Young Hun Chung
Department of Pediatrics, College of Medicine, Chung Nam National University
비장 기능 항진증을 동반한 선천성 간 섬유증 1례
홍혜숙, 이양원, 이건수, 정용헌
충남대학교 의과대학 소아과학교실
Congenital hepatic fibrosis is a relatively rare disease of liver in children and young adults, manifesting a heredofamilial tendency, that is characterized by hepatosplenomegaly, protal hypertension and frequent renal involvement. A generalized portal and interlobular fibrosis of liver are characteristic finding on microscopy, with relative preservation of hepatocyte and liver function. Recently, we have experienced a case of congenital hepatic fibrosis in a 12 years-old boy. He showed marked splenomegaly, mild anemia, thrombocytopenia, leukopenia and normal liver function test without heaptomegaly. There was no evidence of hematemesis and esophageal varices. Familial history was denied. Liver wedge biopasy was characterized by the network of thick collagenous bands that links adjacent portal tracts and bile duct hyperplasia. There was no evidence of parenchymal destruction or regeneration. Hepatocytes were not affected. The patient was discharged in favorable condition after splenectomy for the relief of thrombocytopenia and leukopenia.
Key Words: Hepatic fibrosis, Liver disease, Congenital

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