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

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. Chil...

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Detalhes bibliográficos
Autores: García Pérez de Sevilla, Guillermo, Blanco Velasco, Ángela, Sanz Santiago, Verónica, Morales Tirado, Ana, López Neyra, Alejandro, Manuel, Cristina de, Ruiz Valbuena, Marta, Pérez Ruiz, Margarita
Formato: artículo
Fecha de publicación:2025
País:España
Recursos:Universidad Europea (UEM)
Repositorio:ABACUS. Repositorio de Producción Científica
Idioma:inglés
OAI Identifier:oai:abacus.universidadeuropea.com:11268/16471
Acesso em linha:https://hdl.handle.net/11268/16471
Access Level:acceso abierto
Palavra-chave:Ciencias médicas
Pediatría
Aparato respiratorio
Goal 3: Ensure healthy lives and promote well-being for all at all ages
Goal 9: Build resilient infrastructure, promote sustainable industrialization and foster innovation
Goal 10: Reduce inequality within and among countries
Descrição
Resumo: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.