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Congenital Hepatic Fibrosis (3 Cases Report) : 선천성 간섬유증 3예
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- Authors
- Issue Date
- 1985-12
- Citation
- Seoul J Med, Vol.26 No.4, pp. 347-354
- Keywords
- Liver disease ; Congenital hepatic fibrosis ; Situs inversus ; Nephronophthisis ; Cystic disease
- Abstract
- Congenital hepatic fibrosis is a relatively rare disease of children and young
adults, that is characterized by hard hepatomegaly, portal hypertension with relative preservation
of liver function and underlying architecture, and frequent renal involvement.
We report 3 cases of congenital hepatic fibrosis with quite different presentations.The first,
aged 2 2/12, presented with hepatomegaly followed by splenomegaly with suspicious renal
involvement. The second, aged 2 8/12, had unique feature that congential hepatic fibrosis was
associated with situs inversus, nephronophthisis and positive family history. The third, aged 9
9/12, had splenomegaly without hepatomegaly. All cases had splenomegaly.but there were no
varices on esophagogram or history of hematemesis or melena.
- ISSN
- 0582-6802
- Language
- English
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