Research article

Bile composition in Alagille Syndrome and PFIC patients having Partial External Biliary Diversion

Karan M Emerick^1,2 , Marc S Elias² , Hector Melin-Aldana^1,2 , Sandra Strautnieks³ , Richard J Thompson³ , Laura N Bull⁴ , AS Knisely⁵ , Peter F Whitington^1,2 and Richard M Green²

¹  Children's Memorial Hospital, Chicago, IL, USA

²  Northwestern University Feinberg School of Medicine, Chicago, IL, USA

³  Department of Liver Studies and Transplantation, King's College London School of Medicine, London, UK

⁴  Department of Medicine, San Francisco General Hospital, San Francisco, CA, USA

⁵  Institute of Liver Studies, King's College Hospital, London, UK

author email corresponding author email

BMC Gastroenterology 2008, 8:47doi:10.1186/1471-230X-8-47

Published:	20 October 2008

Abstract

Background

Partial External Biliary Diversion (PEBD) is a surgical intervention to treat children with Progressive Familial Intrahepatic Cholestasis (PFIC) and Alagille syndrome (AGS). PEBD can reduce disease progression, and examining the alterations in biliary lipid composition may be a prognostic factor for outcome.

Methods

Biliary lipid composition and the clinical course of AGS and PFIC patients were examined before and after PEBD.

Results

Pre-PEBD bile from AGS patients had greater chenodeoxycholic/cholic acid (CDCA/CA), bile salt, cholesterol and phospholipid concentrations than PFIC patients. AGS patients, and PFIC patients with familial intrahepatic cholestasis 1 (FIC1) genotype, responded better to PEBD than PFIC patients with bile salt export protein (BSEP) genotype. After successful PEBD, AGS patients have higher biliary lipid concentrations than PFIC patients and PEBD also increases biliary phospholipid concentrations in FIC1 patients.

Conclusion

Both AGS and FIC1 patients can benefit from PEBD, and preserved biliary phospholipid concentrations may be associated with better outcomes post-PEBD.