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. 2008 Oct 20:8:47.
doi: 10.1186/1471-230X-8-47.

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

Karan M Emerick  1 , Marc S Elias, Hector Melin-Aldana, Sandra Strautnieks, Richard J Thompson, Laura N Bull, A s Knisely, Peter F Whitington, Richard M Green
Affiliations

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

Karan M Emerick et al. BMC Gastroenterol. .

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.

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Figures

Figure 1
Figure 1
Biliary lipid content in AGS and PFIC patients prior to Partial External Biliary Diversion (PEBD). Gallbladder bile was obtained from patients prior to PEBD and analyzed for bile salt, cholesterol and phospholipid content. AGS (n = 3), PFIC (n = 7). Mean ± SD. * p < 0.05 vs AGS.
Figure 2
Figure 2
Change in lipid content of bile resulting from Partial External Biliary Diversion (PEBD). Bile was obtained from patients before and after PEBD and analyzed for bile salt, cholesterol and phospholipid content. The data is presented as the percent change in biliary lipid content. AGS (n = 3), PFIC (n = 7; FIC1: n = 4 and BSEP n = 3) * p < 0.05 vs pre-PEBD levels.
Figure 3
Figure 3
Change in lipid content of bile after Partial External Biliary Diversion (PEBD) in Individual Patients. A = AGS, F = FIC1, B = BSEP. Bile was obtained from PFIC (n = 7) patients before and after PEBD who had been independently classified as Responders (n = 4) or Non-Responders (n = 3) based on clinical criteria. Patients identified as non-responders were F3, B1 and B3.
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