Liver disease associated with canalicular transport defects: current and future therapies.
Progressive familial intrahepatic cholestasis (PFIC) is a cholestatic liver disease of childhood. Pruritus secondary to increased bile salts in the serum may not respond to medical treatment. Partial external biliary diversion (PEBD), which reduces the serum bile salt level in the enterohepatic cycle, is used in the treatment of this symptom. In this study, our experience in performing this technique and the early promising results of PEBD in two children with PFIC are reported along with a review of the current literature. Partial external biliary diversion was performed by interposing a 15-cm jejunum between the gallbladder and abdominal wall. Biliary drainage through a stoma began in the fi rst postoperative day and reached 120–200 ml/day. Pruritus improved and then stopped on the 15th postoperative day, while the serum bile acid concentration also decreased. Partial external biliary diversion by jejunal interposition provides an excellent control of pruritus in children with PFIC with no adverse effects. A cholecystectomy should therefore be avoided in patients with PFIC.