Article
Progressive familial intrahepatic cholestasis.
Acta bio-medica : Atenei Parmensis - 1 Jan 2002
Cavestro Giulia Martina, Frulloni Luca, Cerati Elena, Ribeiro Luciana Andrea, Corrente Vincenzo, Sianesi Mario, Franzè Angelo, Di Mario Francesco
Abstract excerpt
Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of autosomal recessive childhood cholestasis of hepatocellular origin. PFIC 1, also known as Byler disease, was first described in Amish kindred. It is characterized by cholestasis often arising in the neonatal period and it leads to death due to liver failure. PFIC 1, like Benign Recurrent Intrahepatic Cholestasis (BRIC) which is the...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.