[HTML][HTML] Bile composition in Alagille syndrome and PFIC patients having partial external biliary diversion
KM Emerick, MS Elias, H Melin-Aldana… - BMC …, 2008 - Springer
KM Emerick, MS Elias, H Melin-Aldana, S Strautnieks, RJ Thompson, LN Bull, AS Knisely…
BMC gastroenterology, 2008•SpringerAbstract 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 …
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 …
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.
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