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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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
Tipo de recurso: artículo
Fecha de publicación:2025
País:España
Institución:Universidad Europea (UEM)
Repositorio:ABACUS. Repositorio de Producción Científica
Idioma:inglés
OAI Identifier:oai:abacus.universidadeuropea.com:11268/16471
Acceso en línea:https://hdl.handle.net/11268/16471
Access Level:acceso abierto
Palabra clave: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
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spelling Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator TherapiesGarcía Pérez de Sevilla, GuillermoBlanco Velasco, ÁngelaSanz Santiago, VerónicaMorales Tirado, AnaLópez Neyra, AlejandroManuel, Cristina deRuiz Valbuena, MartaPérez Ruiz, MargaritaCiencias médicasPediatríaAparato respiratorioGoal 3: Ensure healthy lives and promote well-being for all at all agesGoal 9: Build resilient infrastructure, promote sustainable industrialization and foster innovationGoal 10: Reduce inequality within and among countriesThe 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.20252025-11-1520252025-01-0120252025-01-01journal articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfhttps://hdl.handle.net/11268/16471reponame:ABACUS. Repositorio de Producción Científicainstname:Universidad Europea (UEM)Inglésengopen accesshttp://purl.org/coar/access_right/c_abf2Attribution 4.0 Internationalhttp://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:abacus.universidadeuropea.com:11268/164712026-06-11T12:41:27Z
dc.title.none.fl_str_mv Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies
title Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies
spellingShingle Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies
García Pérez de Sevilla, Guillermo
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
title_short Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies
title_full Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies
title_fullStr Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies
title_full_unstemmed Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies
title_sort Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies
dc.creator.none.fl_str_mv 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
author García Pérez de Sevilla, Guillermo
author_facet 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
author_role author
author2 Blanco Velasco, Ángela
Sanz Santiago, Verónica
Morales Tirado, Ana
López Neyra, Alejandro
Manuel, Cristina de
Ruiz Valbuena, Marta
Pérez Ruiz, Margarita
author2_role author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv
dc.subject.none.fl_str_mv 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
topic 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
description 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.
publishDate 2025
dc.date.none.fl_str_mv 2025
2025-11-15
2025
2025-01-01
2025
2025-01-01
dc.type.none.fl_str_mv journal article
http://purl.org/coar/resource_type/c_6501
VoR
http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://hdl.handle.net/11268/16471
url https://hdl.handle.net/11268/16471
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.source.none.fl_str_mv reponame:ABACUS. Repositorio de Producción Científica
instname:Universidad Europea (UEM)
instname_str Universidad Europea (UEM)
reponame_str ABACUS. Repositorio de Producción Científica
collection ABACUS. Repositorio de Producción Científica
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,812429

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