[Arteriohepatic dysplasia (Alagille syndrome)]. / Arteriohepatisk dysplasi (Alagilles syndrom).

Alagille syndrome (AS) is the second most frequent cause of intrahepatic cholestasis in children under one year of age. The disease has an autosomal dominant mode of inheritance with reduced penetrance and variable expressivity. It has been stated that this form of biliary paucity has a good long-term prognosis, but recent studies have revealed that the syndrome may be accompanied by long-term manifestations extending beyond childhood. Based on a case story the syndrome is described, and the diagnostic criteria, treatment and prognosis of AS are discussed.