Partial external biliary diversion in bile salt export pump deficiency: Association between outcome and mutation

Figure 1 Bile acid derivatives in bile samples of the two patients. Concentrations of different bile acid derivatives were measured in bile samples of two patients possessing the bile salt export pump (BSEP)-T919del and BSEP-G1032R mutation, respectively, as well as in six non-cholestatic and in six cholestatic control samples (Table 1). A: Relative amounts of unconjugated, taurine- or glycine-conjugated bile acids are shown. In the background of the BSEP-G1032R mutation a very high amount ( > 60%) of unconjugated bile salts is found; B: Relative amounts of cholic, chenodeoxycholic and deoxycholic acid (CA, CDCA, DCA) including their corresponding conjugates are shown. In the background of the BSEP-G1032R less than 5% of bile acids are chenodeoxycholic acid and its conjugates (UDCA and conjugates were omitted from these plots). CA: Cholic acid; DCA: Deoxycholic acid; CDCA: Chenodeoxycholic acid.