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Alagille Syndrome
Conference Roundup

Catch-up height, weight seen after maralixibat treatment in patients with ALGS

Posted on June 23, 2022

Patients with Alagille syndrome (ALGS) treated with the ileal bile acid transporter inhibitor (IBATi), maralixibat, experienced catch-up height and weight, with the greatest catch-up in height observed in those with the greatest height disadvantage at baseline, according to a presentation at ESPGHAN 2022.

Data from 34 participants from 3 clinical trials of maralixibat for treatment of cholestatic pruritus in ALGS that had height and weight data at baseline and week 204 follow-up were included.

Patients had a baseline mean (SD) age of 6.7 (3.8) years, height Z-score of -1.66 (1.17), and weight Z-score of -1.46 (0.95). At week 204, the mean height Z-score increased to -1.29 (1.03).

There was an increase from -3.1 (0.71) at baseline to -2.38 (0.82) at week 204 amongst patients within the lowest baseline quartile height Z-scores. There was a significant correlation between the change in height and baseline height. In addition, there was a significant correlation between change in weight and baseline weight, with greater catch-up weight gain seen in patients with lower baseline weight Z-scores.

“Increased catch-up height was also seen in patients that achieved lower serum bile acid with maralixibat, suggesting an improvement in bile acid homeostasis may be a factor,” the authors concluded.

Reference
Kamath BM, et al. Maralixibat improves growth in patients with Alagille syndrome: a 4-year analysis. Presented at ESPGHAN 2022.

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