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Alagille Syndrome

New study evaluates efficacy of therapies for cholestatic pruritus

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New findings from a systematic review provide valuable insights into the efficacy of both guideline-based approaches and newer therapies for cholestatic pruritus. While initial results regarding IBATi are promising, further clinical trials are necessary to determine its full potential in treating other common CLDs. The study serves as a foundation for future research and underscores the importance of continued investigation into the management and treatment of cholestatic liver diseases.

Researchers screened and reviewed articles according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. The methodological quality of the included studies was assessed using established tools for interventional and observational studies.

Out of the 3293 screened articles, 92 studies were eligible for inclusion in the qualitative synthesis. The findings revealed that some patients experienced improvements in HRQoL with evidence-based standard therapy. However, individuals with severe and refractory cholestatic pruritus often required experimental noninvasive or extracorporeal liver support, such as the use of ondansetron or liver support devices, to alleviate their symptoms. Moreover, a newer class of drugs known as ileal bile acid transporter inhibitors (IBATi) demonstrated effectiveness in reducing serum bile acid levels and alleviating cholestatic pruritus. Notably, sustained improvement was observed in patients with progressive familial intrahepatic cholestasis and Alagille syndrome, 2 inherited childhood cholestatic disorders.

Reference
Ebhohon E, Chung RT. Systematic review: efficacy of therapies for cholestatic pruritus. Therap Adv Gastroenterol. 2023;16:17562848231172829. doi: 10.1177/17562848231172829. PMID: 37255856; PMCID: PMC10226044.

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