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Alagille Syndrome

Serum bile acid levels may be beneficial in evaluating ileal bile acid transporter inhibitors

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According to a new study, serum bile acid levels may be the gold standard when assessing new therapies, including ileal bile acid transporter inhibitors (IBATi), in diseases such as bile salt export pump (ABCB11) deficiency, which causes Progressive familial intrahepatic cholestasis (PFIC2).

In this retrospective, single-center, cohort study, 48 cases of confirmed biallelic pathogenic ABCB11 genotype PFIC2 with ≥2 years follow-up were included.

Over the study period, 18 patients underwent partial external biliary diversion (PEBD) surgery, and 22 patients underwent liver transplantation. Two patients died after developing hepatocellular carcinoma.

Improved survival with native liver was associated with genotype, complete normalization of serum bile acids following PEBD, and improvement of pruritus.

Following normalization, a persistence of mild-to-moderate elevation of bile acids or a secondary rise was associated with liver disease progression and transplantation. The authors suggest that “any prolonged elevation of bile acids worsens the chance of native liver survival.”

Reference
Pfister ED, Jaeger VK, Karch A, et al. Native liver survival in bile salt export pump deficiency: results of a retrospective cohort study. Hepatol Commun. 2023;7(4):e0092. doi: 10.1097/HC9.0000000000000092. PMID: 36995996; PMCID: PMC10069862.

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