Bile composition in Alagille Syndrome and PFIC patients having Partial External Biliary DiversionReportar como inadecuado

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BMC Gastroenterology

, 8:47

First Online: 20 October 2008Received: 08 April 2008Accepted: 20 October 2008


BackgroundPartial 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.

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

ResultsPre-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.

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

Electronic supplementary materialThe online version of this article doi:10.1186-1471-230X-8-47 contains supplementary material, which is available to authorized users.

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Autor: Karan M Emerick - Marc S Elias - Hector Melin-Aldana - Sandra Strautnieks - Richard J Thompson - Laura N Bull - AS Knis


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