{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T08:28:49Z","timestamp":1788251329190,"version":"build-2803163510"},"reference-count":41,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,3,12]],"date-time":"2025-03-12T00:00:00Z","timestamp":1741737600000},"content-version":"vor","delay-in-days":11,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>People with cystic fibrosis (pwCF) often have multifactorial peripheral muscle abnormalities attributed to, for example, malnutrition, steroid use, altered redox balance and, potentially, CF\u2010specific intrinsic alterations. Malnutrition in CF now includes an increasing prevalence of overweight and obesity, particularly in those receiving CF transmembrane conductance regulator (CFTR) modulator therapy (CFTRm). We aimed to characterise peripheral muscle function and body composition in pwCF on Elexacaftor\/Tezacaftor\/Ivacaftor (ETI) CFTRm, compared to healthy controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Fifteen pwCF on ETI, and 15 healthy age\u2010 and sex\u2010matched controls (CON), underwent whole\u2010body dual\u2010energy X\u2010ray absorptiometry scans, and a comprehensive evaluation of peripheral muscle function. Tests included quadriceps maximal isometric force measurement, an intermittent isometric quadriceps fatiguing protocol, handgrip strength dynamometry, squat jump height assessment, and 1\u2010min sit\u2010to\u2010stand testing.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>No significant differences in quadriceps maximal isometric force (CON: 181.60\u2009\u00b1\u200992.90 Nm vs. CF: 146.15\u2009\u00b1\u200952.48\u2009Nm, <jats:italic>p<\/jats:italic>\u2009=\u20090.21, <jats:italic>d<\/jats:italic>\u2009=\u20090.47), handgrip strength (CON: 34\u2009\u00b1\u200915\u2009kg vs. CF: 31\u2009\u00b1\u200911\u2009kg, <jats:italic>p<\/jats:italic>\u2009=\u20090.62, <jats:italic>d<\/jats:italic>\u2009=\u20090.18), peripheral muscle endurance, fatigue, or power were observed between the groups. Moreover, no significant differences in whole\u2010body, trunk or limb lean mass, fat\u2010free mass, fat mass, or whole\u2010body bone mineral density were evident.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Comparable peripheral muscle mass and function has been demonstrated in pwCF on ETI, albeit a group with good lung function. Research is needed to confirm these findings longitudinally in pwCF, including those with more severe lung disease, who are less physically active, and have less optimal nutrition and exercise support.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.71044","type":"journal-article","created":{"date-parts":[[2025,3,12]],"date-time":"2025-03-12T11:30:11Z","timestamp":1741779011000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Peripheral Muscle Function and Body Composition in People With Cystic Fibrosis on Elexacaftor\/Tezacaftor\/Ivacaftor: A Cross\u2010Sectional Single\u2010Centre Study"],"prefix":"10.1002","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4907-257X","authenticated-orcid":false,"given":"Lauren J.","family":"Clayton","sequence":"first","affiliation":[{"name":"School of Psychology, Sport and Health Science, Faculty of Science and Health University of Portsmouth Portsmouth UK"},{"name":"University Hospital Southampton NHS Foundation Trust Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony I.","family":"Shepherd","sequence":"additional","affiliation":[{"name":"School of Psychology, Sport and Health Science, Faculty of Science and Health University of Portsmouth Portsmouth UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo","family":"Corbett","sequence":"additional","affiliation":[{"name":"School of Psychology, Sport and Health Science, Faculty of Science and Health University of Portsmouth Portsmouth UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathieu","family":"Gruet","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Toulon Toulon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gary","family":"Connett","sequence":"additional","affiliation":[{"name":"University Hospital Southampton NHS Foundation Trust Southampton UK"},{"name":"National Institute for Health and Care Research, Southampton Biomedical Research Centre Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Allenby","sequence":"additional","affiliation":[{"name":"University Hospital Southampton NHS Foundation Trust Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian","family":"Legg","sequence":"additional","affiliation":[{"name":"University Hospital Southampton NHS Foundation Trust Southampton UK"},{"name":"National Institute for Health and Care Research, Southampton Biomedical Research Centre Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5249-5100","authenticated-orcid":false,"given":"Thomas","family":"Daniels","sequence":"additional","affiliation":[{"name":"University Hospital Southampton NHS Foundation Trust Southampton UK"},{"name":"National Institute for Health and Care Research, Southampton Biomedical Research Centre Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Don S.","family":"Urquhart","sequence":"additional","affiliation":[{"name":"Department of Paediatric Respiratory and Sleep Medicine Royal Hospital for Children and Young People Edinburgh Scotland UK"},{"name":"Department of Child Life and Health University of Edinburgh  Scotland UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zoe L.","family":"Saynor","sequence":"additional","affiliation":[{"name":"University Hospital Southampton NHS Foundation Trust Southampton UK"},{"name":"School of Health Sciences, Faculty of Environmental and Life Sciences University of Southampton Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,3,12]]},"reference":[{"key":"e_1_2_9_2_1","unstructured":"The Cystic Fibrosis. 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