J Korean Pediatr Soc.
1998 Mar;41(3):410-414.
A Case of Alagille Syndrome
- Affiliations
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- 1Deprartment of Pediatrics, Chung-ang Gil Hospital, Inchon, Korea.
- 2Deprartment of Radiology, Chung-ang Gil Hospital, Inchon, Korea.
- 3Deprartment of Clinical Pathology, Chung-ang Gil Hospital, Inchon, Korea.
- 4Department of Pediatrics, Red Cross Hospital, Seoul, Korea.
Abstract
- Alagille syndrome is characterized by chronic cholestasis, posterior embryotoxon, skeletal abnormalities, cardiovascular abnormalities, and a typical face with prominent forehead and pointed chin. Its histological feature includes paucity of interlobular bile ducts. We experienced a 49-day-old female infant presenting with frequent upper respiratory tract infection and persistent jaundice. She had a typical face and chronic cholestasis. Echocardiograms revealed peripheral pulmonary stenosis. The histological examination of liver revealed paucity of interlobular bile ducts.
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