SHERP

Congenital Hepatic Fibrosis (3 Cases Report)
선천성 간섬유증 3예

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Authors
Lee, Hoan Jong; Chi, Je G.; Seo, Jeong Kee; Choi, Yong; Ko, Kwang Wook; Moon, Hyung Ro
Issue Date
1985-12
Publisher
Seoul National University College of Medicine
Citation
Seoul J Med 1985;26(4):347-354
Keywords
Liver diseaseCongenital hepatic fibrosisSitus inversusNephronophthisisCystic 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
URI
http://hdl.handle.net/10371/6415
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 26 No.4 (1985)
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