並列摘要
Arteriohepatic dysplasia (Alagille syndrome) is a condition of chronic cholestasis in infancy associated with odd fades, pulmonic steaosis, butterfly vertebra and other abnormalities. The pathologic hallmark of arteriohepatic dysplasia is a paucity or absence of intrahepatic bile ducts. We present a 4-year-old boy with MRI study of heart showing peripheral stenosis of the left pulmonary artery and single butterfly vertebra. Ophthalmal examination discovered embryotoxon of both eyes. Scintigraphy revealed normal excretion of Tc-99m IDA into the intestine from the liver and liver biopsy exhibited paucity of intrahepatic bile ducts.