Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies
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Blanco Velasco, Ángela
Sanz Santiago, Verónica
Morales Tirado, Ana
López Neyra, Alejandro
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Abstract
The objective of this study was to analyze respiratory muscle function in children and adolescents with cystic fibrosis (CF) treated with Elexacaftor/Tezacaftor/Ivacaftor (ETI) compared to healthy individuals, based on the hypothesis that CFTR modulators may improve respiratory muscle strength.
Children and adolescents with CF treated with ETI showed respiratory muscle strength comparable to that of healthy controls. Despite differences in lifestyle factors, these findings may reflect a positive impact of CFTR modulators on respiratory muscle function, although further longitudinal and controlled studies are needed.
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García-Pérez-de-Sevilla, G., Blanco Velasco, Á., Yvert, T., Sanz-Santiago, V., Tirado, A. M., López Neyra, A., De Manuel, C., Ruiz Valbuena, M., & Pérez-Ruiz, M. (2025). Respiratory muscle function in children and adolescents with cystic fibrosis in the era of cftr modulator therapies. Children, 12(7), 878. https://doi.org/10.3390/children12070878










