TY - JOUR
T1 - Bile composition in Alagille Syndrome and PFIC patients having Partial External Biliary Diversion
AU - Emerick, Karan M.
AU - Elias, Marc S.
AU - Melin-Aldana, Hector
AU - Strautnieks, Sandra
AU - Thompson, Richard J.
AU - Bull, Laura N.
AU - Knisely, A. S.
AU - Whitington, Peter F.
AU - Green, Richard M.
N1 - Funding Information:
This study was supported by grant awards NIDDK R01 DK059580 and T32DK070066.
PY - 2008/10/20
Y1 - 2008/10/20
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=56849131806&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=56849131806&partnerID=8YFLogxK
U2 - 10.1186/1471-230X-8-47
DO - 10.1186/1471-230X-8-47
M3 - Article
C2 - 18937870
AN - SCOPUS:56849131806
SN - 1471-230X
VL - 8
JO - BMC Gastroenterology
JF - BMC Gastroenterology
M1 - 47
ER -