식도 정맥류 출혈을 동반한 간섬유증 1례
A Case of Congenital Hepatic Fibrosis with Variceal Bleeding.
신동수, 임시홍,
소속 상세정보
신동수 ( Shin Dong-Soo )
한림대학교 의과대학 소아과학교실
임시홍 ( Lim Si-Hong )
한림대학교 의과대학 소아과학교실
KMID : 0816120040070010098
Abstract
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.
키워드
Congenita Hepatic Fibrosis;Esophageal varix
원문 및 링크아웃 정보
등재저널 정보