Partial External Biliary Diversion in Children With Progressive Familial Intrahepatic Cholestasis and Alagille Disease

Background: Partial external biliary diversion (PEBD) is a promising treatment for children with progressive familial intrahepatic cholestasis (PFIC) and Alagille disease. Little is known about long-term Outcomes. Patients and Methods: A retrospective chart review of all patients undergoing PEBD in the University Medical Centre of Groningen (UMCG). Results: Between 2000 and 2005. PEBD was performed on 14 children with severe pruritus (PFIC I I. mean age 5.3 +/- 4.4 years: Alagille 3, mean age 7.4 +/- 4.2 years). Stature was Conclusions: At median follow-up of 3.1 years after PEBD, pruritus has been relieved in 75%. Bile salts level and growth are improved in most patients. Longer follow-up is needed to determine whether PEBD can postpone or avoid the demand for fiver transplantation. JPGN 49:216-221, 2009