Alagille syndrome is a disorder that is characterized by paucity of interlobularbile duct, cholestasis, characteristic facial profile, ocular abnormality, cardiovascular abnormalities and vertebral arch defect.
We experienced a case of alagille syndrome of a 22 month-old female. She showed persistent cholestasis, characteristic facies, peripheral pulmonary artery stenosis and vertebral defect, but posterior embryotoxon was not seen. The liver biopsy
showed
paucity of interlobular bile ducts.
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