Journal of the Korean Pediatric Society 2003;46(1):86-90.
Published online January 15, 2003.
A Case of Moyamoya Disease in a Child with Alagille Syndrome
Mi Rang Lim, So Yaun Lee, Deok Soo Kim, Kyung Mo Kim, Tae Sung Ko
Department of Pediatrics, Asan Medical Center, College of Medicine, Ulsan University, Seoul, Korea
Alagille 증후군 환아에서 발생한 Moyamoya병 1례
임미랑, 이소연, 김덕수, 김경모, 고태성
울산대학교 의과대학 서울아산병원 소아과
Tae Sung Ko, Email:
Alagille syndrome is a autosomal dominant disorder characterized by intrahepatic bile duct paucity and resultant chronic cholestasis in combination with cardiac(mainly peripheral pulmonary stenosis), skeletal, ocular, and facial abnormalities. In addition to the pulmonary stenosis, in large series, anecdotal reports of vascular lesions have concerned the renal artery, aorta, hepatic artery, carotid artery, celiac artery or subclavian artery. Theses diffuse vascular abnormalities, which appear to be a feature of Alagille syndrome, suggest Notch signaling pathway defects affect angiogenesis. The associations of Alagille syndrome with moyamoya disease, the chronic cerebrovascular occlusive disease, were reported and suggested as additional evidence of vasculopathy of Alagille syndrome. We report another 25 month-old Alagille syndrome girl who presented with acute left hemiparesis and was diagnosed with moyamoya disease through the cerebral angiographic study.
Key Words: Alagille syndrome, Moyamoya disease, Vasculopathy

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