A Case of Alagille Syndrome.
- Author:
Eun Kyung HWANG
1
;
Gwang Hoon LEE
;
Eell RYOO
;
Kang Ho CHO
;
Gil Hyun KIM
;
Hak Soo LEE
;
Ji Hye KIM
;
Sung Hae PARK
;
Hee Sup KIM
Author Information
1. Deprartment of Pediatrics, Chung-ang Gil Hospital, Inchon, Korea.
- Publication Type:Case Report
- Keywords:
Alagille syndrome
- MeSH:
Alagille Syndrome*;
Cardiovascular Abnormalities;
Chin;
Cholestasis;
Female;
Forehead;
Humans;
Infant;
Jaundice;
Liver;
Pulmonary Valve Stenosis;
Respiratory Tract Infections
- From:Journal of the Korean Pediatric Society
1998;41(3):410-414
- CountryRepublic of Korea
- Language:Korean
-
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.
