{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T01:23:59Z","timestamp":1791509039981,"version":"4.3.4"},"reference-count":53,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2025,7,3]],"date-time":"2025-07-03T00:00:00Z","timestamp":1751500800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Instituto Sanitario Carlos III","award":["Proyecto PI23\/00299"],"award-info":[{"award-number":["Proyecto PI23\/00299"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Objective: 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. Methods: A descriptive, observational, cross-sectional study was conducted with patients with CF treated with ETI aged 6\u201318 years. Lung function, maximal expiratory and inspiratory pressures (MIP and MEP), diet quality (KIDMED), and physical activity levels (PAQ) were assessed. The student\u2019s t-test or the Mann\u2013Whitney U-test was used to compare differences between groups. The effect size was calculated with Cohen\u2019s d. Significance level was set as a p-value &lt; 0.05. Results: A total of 48 children and adolescents (60.4% male) were analyzed in this study (24 healthy and 24 with CF). The participants with CF had mild pulmonary involvement. No significant differences were found in respiratory muscle strength between groups (MEPmax\u00a0p = 0.440, MIPmax\u00a0p = 0.206). Patients with CF showed lower KIDMED (p = 0.022) and PAQ (p = 0.010) scores. However, the MIP and MEP values observed in CF participants were higher than those reported in previous studies conducted before the introduction of ETI modulators. Conclusions: 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.<\/jats:p>","DOI":"10.3390\/children12070878","type":"journal-article","created":{"date-parts":[[2025,7,3]],"date-time":"2025-07-03T06:54:16Z","timestamp":1751525656000},"page":"878","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Respiratory Muscle Function in Children and Adolescents with Cystic Fibrosis in the Era of CFTR Modulator Therapies"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2689-1767","authenticated-orcid":false,"given":"Guillermo","family":"Garc\u00eda-P\u00e9rez-de-Sevilla","sequence":"first","affiliation":[{"name":"Department of Physiotherapy, Faculty of Medicine, Health and Sports, European University of Madrid, 28670 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c1ngela","family":"Blanco Velasco","sequence":"additional","affiliation":[{"name":"ImFINE Research Group, Department of Health and Human Performance, Facultad de Ciencias de la Actividad F\u00edsica y del Deporte, INEF Universidad Polit\u00e9cnica de Madrid, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9451-9799","authenticated-orcid":false,"given":"Thomas","family":"Yvert","sequence":"additional","affiliation":[{"name":"ImFINE Research Group, Department of Health and Human Performance, Facultad de Ciencias de la Actividad F\u00edsica y del Deporte, INEF Universidad Polit\u00e9cnica de Madrid, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ver\u00f3nica","family":"Sanz-Santiago","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Pediatric Pulmonology Department, Hospital Universitario Infantil Ni\u00f1o Jes\u00fas de Madrid, 28009 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2707-3037","authenticated-orcid":false,"given":"Ana Morales","family":"Tirado","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Department of Pediatric Medicine, Faculty of Medicine and Health Sciences, Ramon y Cajal University Hospital, 28034 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4032-1429","authenticated-orcid":false,"given":"Alejandro","family":"L\u00f3pez Neyra","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Unit, Pediatric Pulmonology Department, Hospital Universitario Infantil Ni\u00f1o Jes\u00fas de Madrid, 28009 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6224-1561","authenticated-orcid":false,"given":"Cristina","family":"de Manuel","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology Department and Cystic Fibrosis Unit, Hospital La Paz, 28046 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Ruiz Valbuena","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology Department and Cystic Fibrosis Unit, Hospital La Paz, 28046 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7240-2082","authenticated-orcid":false,"given":"Margarita","family":"P\u00e9rez-Ruiz","sequence":"additional","affiliation":[{"name":"ImFINE Research Group, Department of Health and Human Performance, Facultad de Ciencias de la Actividad F\u00edsica y del Deporte, INEF Universidad Polit\u00e9cnica de Madrid, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,7,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1038\/s41572-024-00538-6","article-title":"Cystic Fibrosis","volume":"10","author":"Mall","year":"2024","journal-title":"Nat. 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