Patients with Alagille syndrome (ALGS) with higher levels of the poly-hydroxylated bile acids tauro-2β,3α,7α,12α-tetrahydroxylated bile acid (THBA) and glyco-hyocholic acid (GHCA) had a better prognosis than those with lower concentrations, according to a study currently under review in Hepatology International.

The researchers found that the levels of tauro‐2β,3α,7α,12α-THBA (P =.013), and GHCA (P =.036) were significantly higher in patients with a good prognosis compared to a poor prognosis. A good prognosis was categorized as survival with their native liver and total bilirubin (TB) <85.5 µmol/L while a poor prognosis was categorized as having either a liver transplant, died of liver failure, or TB ≥85.5 µmol/L at last follow-up.

Using an optimal cutoff value of 79.88 nmol/L for tauro-2β,3α,7α,12α-THBA in patients with ALGS 1-year-old or younger yielded a prognostic accuracy for good outcomes of 88.00% (sensitivity, 92.31%; specificity, 83.33%). 

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Univariate analysis found that higher levels of GHCA and tauro‐2β,3α,7α,12α-THBA, as well as tauro‐3α,6β,7α,12α-THBA and tauro-hyocholic acid (THCA), were all associated with better native liver survivability.

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Using multivariate analysis revealed only GHCA as the single independent factor influencing native liver survival (hazard ratio, 6.456; 95% CI, 1.173-35.545; P =.032). Patients with GHCA values less than a cutoff of 607.69 nmol/L had a higher rate of death or liver transplant (7 out of 13 patients) than those with a value above the cutoff (2 out of 33 patients).

“The findings from this study indicate that the blood level of tauro-2β,3α,7α,12α-THBA in ALGS patients before 1-year of age could be an excellent prognostic biomarker and that GHCA can predict native liver survival of such patients,” the authors summarized.

Information from a total of 46 patients with ALGS (21 included in a discovery cohort and 25 from a validation cohort) was included in the study. Discovery cohort samples were collected between January 2015 and December 2017 while the validation cohort samples were collected between January 2018 and October 2020.


Wang MX, Han J, Liu T, et al. Poly-hydroxylated bile acids and their prognostic roles in Alagille syndrome. Res Sq. Published online May 5, 2022. doi:10.21203/