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Arteriohepatic Dysplasia (Alagille Syndrome)

Alagille syndrome is a genetic condition affecting 1 in 70,000 newborns. Generally presenting in infancy, this condition is often misdiagnosed as biliary atresia. Occasionally, it can escape medical attention until school age, where it appears as xanthomata (fibrous, fatty tissue or nodules under the skin) or pruritis (itching). Jaundice may or may not be present.

The syndrome is marked by five major features, several of which must be present for diagnosis: cholestatic liver disease, congenital heart disease, vertebral and/or long bone anomalies, ophthalmic (eye) abnormalities and peculiar facies (facial features), such as deep-set eyes and an oversized brow.

There is a 50 percent chance that people with this syndrome will pass it to their children. A prenatal diagnosis is not available.