Abstract
Background. Alagille syndrome is a genetic disorder with autosomal dominant transmission but highly variable expression. It is clinically defined by the association of at least three of the major clinical features (chronic cholestasis, congenital heart disease, “butterfly-like” vertebrae, posterior embryotoxon and peculiar facies). Liver biopsy classically shows the presence of intrahepatic bile duct paucity. It is one of the most common genetic causes of chronic liver disease in childhood. Ursodeoxycholic acid (UDCA) might exert beneficial effects in cholestatic disease.
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