Model of proceedings in children with progressive familial intrahepatic cholestasis Irena Jankowska, Joanna Pawłowska, Hor Ismail, Piotr Kaliciński, Jerzy Socha Medical Science Review - Hepatologia 2009; 9 19-26 aaICID: 902237
Article type: Review article
IC™ Value: 3.04
Abstract provided by Publisher
The authors try to establish a model of procedure in children with progressive familial intrahepatic cholestasis. The first step is UDCA therapy, which in some children is the only effective treatment. Sometimes antipruritic drugs given additionally are very useful. Partial external biliary diversion has became a standard procedure performed on PFIC children (PFIC type 1 or 2) with no response to medical treatment (UDCA). In children who have had cholecytectomy, partial external biliary diversion is impossible and ileal bypass should be considered. In children with progressive cholestasis the only effective therapy is liver transplantation.