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KMID : 0816120040070010098
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Korean Journal of Pediatric Gastroenterolology and Nutrition
2004 Volume.7 No. 1 p.98 ~ p.101
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A Case of Congenital Hepatic Fibrosis with Variceal Bleeding.
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Shin Dong-Soo
Lim Si-Hong
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Abstract
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Congenital hepatic fibrosis is an inherited, congenital disorder of the liver characterized by portal hypertension and hepatic fibrosis. We experienced a case of congenital hepatic fibrosis with esophageal varix in a 9-year-old male. He complained hematemesis, hematochezia, dizziness. In laboratory examination, AST/ALT was slightly increased. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and hypoechoic lesion of periportal area were seen by abdominal CT scanning. Histologic finding of liver biopsy showed fibrous tracts containing dilated bile ductules connecting adjacent portal spaces that were widened by mature fibrosis. Endocopic sclerotherpy and ligation was done. We summarized a case with review of literatures.
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KEYWORD
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Congenita Hepatic Fibrosis, Esophageal varix
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