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 |
Correspondence:
Eun Kyung Hwang, Email: 1 |
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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. |
Key Words:
Alagille syndrome |
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