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Alagille Syndrome
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Poly-hydroxylated bile acids may be prognostic biomarkers in ALGS

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Patients with Alagille syndrome (ALGS) who have increased concentrations of 2 poly-hydroxylated bile acids may have better clinical outcomes than those without increased concentrations, according to a study.

The bile acids were tauro-2β,3α,7α,12α-tetrahydroxylated bile acid (THBA) and glyco-hyocholic acid (GHCA).

In this study, researchers performed bile acid profiling on patients with genetically confirmed JAG1-associated ALGS <1 year of age.

A good prognosis was defined as survival with native liver and total bilirubin (TB) <85.5 μmol/L; poor prognosis was defined as either liver transplantation, death from liver failure, or TB ≥85.5 μmol/L at the last follow-up.

Given the highly variable nature of liver manifestations in ALGS, identifying factors that affect prognosis is important.

Reference
Wang MX, Han J, Liu T, et al. Poly-hydroxylated bile acids and their prognostic roles in Alagille syndrome. World J Pediatr. 2023;doi: 10.1007/s12519-022-00676-5. Epub ahead of print. PMID: 36658452.

 

 

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