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Alagille Syndrome

Persistent jaundice may signal poor prognosis in ALGS

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Patients with Alagille syndrome (ALGS) who have jaundice past the age of 2 may have a worse prognosis than those who are jaundice-free, according to a study.

Researchers classified 25 children with ALGS into 2 groups, the first of children who had jaundice resolve after 2 years of age (n = 9) and the second of children who had progressive disease, including persistent jaundice or progressive cholestasis (n = 16). Patients were followed for a median of 7.3 years.

Overall, 36% of patients in the jaundice-free group had a favorable outcome and retained their native liver longer than patients with progressive disease. In the group with progressive disease, 14 patients received a liver transplant or died.

Reference
Chiang CM, Jeng YM, Ho MC, et al. Different clinical and genetic features of Alagille patients with progressive disease versus a jaundice-free course. JGH Open. 2022;6(12):839-845. doi: 10.1002/jgh3.12830. PMID: 36514505; PMCID: PMC9730729.

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