| Three cases of Watson-Alagille syndrome. |
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Eun Kyeong Bom, Dae Hyun Kim, Dong Hoon Koh, Young Youn Choi, Jae Sook Ma, Tae Ju Hwang |
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Department of Pediatrics, College of Medicine, Chonnam National University, Kwangju, Korea |
| Watson-Alagille 증후군 3례 |
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범은경, 김대현, 고동훈, 최영륜, 마재숙, 황태주 |
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전남대학교 의과대학 소아과학교실 |
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Received: 3 June 1991 • Accepted: 25 July 1991 |
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| Abstract |
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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.
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| Key Words:
Watson-Alagille syndrome, Jaundice, Intrahepatic biliary atresia, Pulmonary artery stenosis |
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