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A Case of Alagille Syndrome

Journal of the Korean Pediatric Society 1998;41(3):410-414.
Published online March 15, 1998.
A Case of Alagille Syndrome
Eun Kyung Hwang1, Gwang Hoon Lee1, Eell Ryoo1, Kang Ho Cho1, Gil Hyun Kim1, Hak Soo Lee1, Ji Hye Kim1, Sung Hae Park2, Hee Sup Kim3
1Deprartment of Pediatrics, Chung-ang Gil Hospital, Inchon, Korea
2Deprartment of Clinical Pathology, Chung-ang Gil H ospital, Inchon, Korea
3Department of Pediatrics, Red Cross Hospital, Seoul, Korea
Alagille Syndrome 1예
황은경1, 이광훈1, 류일1, 조강호1, 김길현1, 이학수1, 김지혜1, 박성혜2, 김희섭3
1중앙길병원 소아과
2중앙길병원 해부병리과
3서울적십자병원 소아과
Eun Kyung Hwang, Email: 1
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.
Key Words: Alagille syndrome

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