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Three cases of Watson-Alagille syndrome.

Journal of the Korean Pediatric Society 1991;34(12):1699-1706.
Published online December 31, 1991.
Three cases of Watson-Alagille syndrome.
Eun Kyeong Bom, Dae Hyun Kim, Dong Hoon Koh, Young Youn Choi, Jae Sook Ma, Tae Ju Hwang
Department of Pediatrics, College of Medicine, Chonnam National University, Kwangju, Korea
Watson-Alagille 증후군 3례
범은경, 김대현, 고동훈, 최영륜, 마재숙, 황태주
전남대학교 의과대학 소아과학교실
Received: 3 June 1991   • Accepted: 25 July 1991
Abstract
Watson-Alagille syndrome is a kind of familial intrahepatic biliary atresia associated with charac- teristic face and cardiovascular anomaly. Growth and developmental delay, anomalies of the eyes such as posterior embryotoxon, deformities of the skeletal system are often the manifestations of this syndrome. We experienced three cases of Watson-Alagille syndrome recently. These patients showed charac- teristic faces and had peripheral pulmonary artery stenosis diagnosed by pulmonary angiography or 2 dimensional echocardiography and intrahepatic biliary atresia confirmed by liver biopsy. Butterfly deformity of vertebrae was detected in case 1 only. We reported three cases of Watson-Alagille syndrome with a brief review of related literatures.
Key Words: Watson-Alagille syndrome, Jaundice, Intrahepatic biliary atresia, Pulmonary artery stenosis


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