Journal of the Korean Pediatric Society 2002;45(7):923-927.
Published online July 15, 2002.
A Case of Congenital Hepatic Fibrosis Accompanied by Renal Tubular Ectasia, Caroli Syndrome and Choledochal Cyst
Bong Seok Choi1, Sang Nam Bae1, Yong Tak Im1, Jae Hong Park1, Chang Hoon Lee2, Jun Woo Lee3
1Department of Pediatrics, Collage of Medicine, Pusan National University, Pusan, Korea
2Department of Pathology, Collage of Medicine, Pusan National University, Pusan, Korea
3Department of Diagnostic Radiology, Collage of Medicine, Pusan National University, Pusan, Korea
신세뇨관 확장증, Caroli 증후군 및 총 담관낭을 동반한 선천성 간 섬유증 1례
최봉석1, 배상남1, 임영탁1, 박재홍1, 이창훈2, 이준우3
1부산대학교 의과대학 소아과학교실
2부산대학교 의과대학 병리학교실
3부산대학교 의과대학 진단방사선학교실
Correspondence: 
Jae Hong Park, Email: minambong@hanmail.net
Abstract
Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis associated with renal tubular ectasia in a 3-year- old girl, whose chief complaint was abdominal distension. Her liver function test did not reveal any abnormal findings. Hepatosplenomegaly and multiple dilated bile ducts were seen in the abdominal CT scaning. Esophageal varix was not detected by an endoscopic examination. Microscopically, diffuse portal fibrosis and widening with proliferation of blie ductules in the liver specimen and tubular ectasia in renal cortex were seen.
Key Words: Congenital hepatic fibrosis, Renal tubular ectasia


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