Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies

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dc.contributor.authorGarcía Pérez de Sevilla, Guillermo
dc.contributor.authorBlanco Velasco, Ángela
dc.contributor.authorSanz Santiago, Verónica
dc.contributor.authorMorales Tirado, Ana
dc.contributor.authorLópez Neyra, Alejandro
dc.contributor.authorManuel, Cristina de
dc.contributor.authorRuiz Valbuena, Marta
dc.contributor.authorPérez Ruiz, Margarita
dc.date.accessioned2025-11-15T09:34:56Z
dc.date.available2025-11-15T09:34:56Z
dc.date.issued2025
dc.description.abstractThe 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.
dc.description.filiationUEMspa
dc.description.impact2.1 Q2 JCR 2024
dc.description.impact0.674 Q2 SJR 2024
dc.description.impactNo data IDR 2024
dc.description.sponsorshipEstos resultados preliminares pertenecen al Proyecto PI23/00299 Instituto Sanitario Carlos III, el cual recibió apoyo financiero de la Unión Europea.
dc.identifier.citationGarcía-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
dc.identifier.doi10.3390/children12070878
dc.identifier.issn2227-9067
dc.identifier.urihttps://hdl.handle.net/11268/16471
dc.language.isoeng
dc.peerreviewedSi
dc.relation.publisherversionhttps://doi.org/10.3390/children12070878
dc.rightsAttribution 4.0 Internationalen
dc.rights.accessRightsopen access
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.subject.sdgGoal 3: Ensure healthy lives and promote well-being for all at all ages
dc.subject.sdgGoal 9: Build resilient infrastructure, promote sustainable industrialization and foster innovation
dc.subject.sdgGoal 10: Reduce inequality within and among countries
dc.subject.unescoCiencias médicas
dc.subject.unescoPediatría
dc.subject.unescoAparato respiratorio
dc.titleRespiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies
dc.typejournal article
dc.type.hasVersionVoR
dspace.entity.typePublication
relation.isAuthorOfPublicationd696164e-9e7a-4208-8acb-9871d9a8f1c8
relation.isAuthorOfPublicationa5c08444-aa82-4924-a71e-de56086bcd7c
relation.isAuthorOfPublication.latestForDiscoveryd696164e-9e7a-4208-8acb-9871d9a8f1c8

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