Nonsyndromic Paucity of Intrahepatic Bile Ducts

Non-syndromic paucity of interlobular bile ducts includes a heterogeneous group of intrahepatic cholestatic disorders that share similar histological findings, characterized by  absent or reduced number of interlobular bile ducts, without the associated features of Alagille’s syndrome. This category probably includes patients with various diseases that evolve with paucity of interlobular bile ducts. The first patients were reported in the Alagille’s series &.
Clinical features of the disease, diagnosis, medical management and indication to liver transplantation are the same reported for Alagille’s syndrome. The prognosis of the disease is less favourable than those with arteriohepatic displasia, with several patients dying of chirrosis..