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- W4225539604 abstract "Abstract Objective: This study aimed to investigate the safety, tolerability, and efficacy of selexipag in children and young adults with idiopathic and heritable pulmonary arterial hypertension. Methods: This retrospective cohort study included clinical data from five children and six young adults with pulmonary arterial hypertension receiving selexipag as add-on therapy or as a transition from beraprost sodium or epoprostenol infusion therapy. Clinical efficacy was evaluated by measuring improvement in clinical variables from baseline, including hemodynamic parameters. Results: Of the 11 patients, 6 were switched from beraprost sodium to selexipag and one paediatric patient transitioned from epoprostenol to selexipag. The median maintenance dose of selexipag in children was 80 μg/kg/day. In nine patients undergoing repeat catheterisation, statistically significant improvements were observed after the initiation of selexipag in terms of mean pulmonary arterial pressure (p < 0.01), pulmonary vascular resistance index (p < 0.05), and cardiac index (p < 0.01). None of the patients had clinical worsening after selexipag during follow-up, but one young adult patient discontinued treatment due to severe headache. The most common side effect profiles were headache, nausea, abdominal pain, jaw pain, myalgia, and diarrhoea. Conclusions: Selexipag may have a favourable safety profile and potential efficacy in children and young adults with pulmonary arterial hypertension." @default.
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- W4225539604 date "2022-04-06" @default.
- W4225539604 modified "2023-10-17" @default.
- W4225539604 title "Clinical efficacy and safety of selexipag in children and young adults with idiopathic and heritable pulmonary arterial hypertension" @default.
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- W4225539604 doi "https://doi.org/10.1017/s1047951122000415" @default.
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