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dc.contributor.authorDayman, Naomi
dc.contributor.authorBlyth, Natalie
dc.contributor.authorMadge, Joe
dc.contributor.authorGaillard, Erol
dc.date.accessioned2024-03-05T13:08:17Z
dc.date.available2024-03-05T13:08:17Z
dc.date.issued2024-02-19
dc.identifier.citationAhmed, M. I., Dayman, N., Blyth, N., Madge, J., & Gaillard, E. (2024). Impact of CFTR modulators on exercise capacity in adolescents with cystic fibrosis. ERJ open research, 10(1), 00687-2023. https://doi.org/10.1183/23120541.00687-2023en_US
dc.identifier.other10.1183/23120541.00687-2023
dc.identifier.urihttp://hdl.handle.net/20.500.12904/18271
dc.description.abstractBackground: Exercise capacity is an independent predictor of clinical worsening in cystic fibrosis (CF). There is limited evidence of the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on exercise capacity in children with CF. The aim of the present study was to assess the impact of CFTR modulators on exercise capacity in a cohort of adolescents with CF. Methods: A prospective single-centre cohort study was carried out. Cardiopulmonary exercise testing (CPET) was performed at baseline, prior to starting Symkevi or Kaftrio and between 4 and 8 months after starting treatment. Results: 19 adolescents with CF had CPET performed prior to and after CFTR modulator treatment, between December 2019 and March 2022. Breathing reserve improved in the whole cohort, with greater improvement in the modulator-naïve patients after starting treatment with Kaftrio. There was no improvement in peak oxygen uptake and anaerobic threshold after 4 to 8 months of treatment with CFTR modulators. Conclusion: Exercise testing with CPET can be used as an additional tool to monitor response to CFTR modulators. Breathing reserve on CPET may provide a surrogate marker to monitor the improvement in CF lung disease with CFTR modulator treatment.
dc.description.urihttps://openres.ersjournals.com/content/10/1/00687-2023en_US
dc.language.isoenen_US
dc.subjectcystic fibrosisen_US
dc.subjectCFTR modulatorsen_US
dc.titleImpact of CFTR modulators on exercise capacity in adolescents with cystic fibrosisen_US
dc.typeArticleen_US
rioxxterms.funderDefault funderen_US
rioxxterms.identifier.projectDefault projecten_US
rioxxterms.versionNAen_US
rioxxterms.versionofrecordhttps://doi.org/10.1183/23120541.00687-2023en_US
rioxxterms.typeJournal Article/Reviewen_US
refterms.panelUnspecifieden_US
html.description.abstractBackground: Exercise capacity is an independent predictor of clinical worsening in cystic fibrosis (CF). There is limited evidence of the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on exercise capacity in children with CF. The aim of the present study was to assess the impact of CFTR modulators on exercise capacity in a cohort of adolescents with CF. Methods: A prospective single-centre cohort study was carried out. Cardiopulmonary exercise testing (CPET) was performed at baseline, prior to starting Symkevi or Kaftrio and between 4 and 8 months after starting treatment. Results: 19 adolescents with CF had CPET performed prior to and after CFTR modulator treatment, between December 2019 and March 2022. Breathing reserve improved in the whole cohort, with greater improvement in the modulator-naïve patients after starting treatment with Kaftrio. There was no improvement in peak oxygen uptake and anaerobic threshold after 4 to 8 months of treatment with CFTR modulators. Conclusion: Exercise testing with CPET can be used as an additional tool to monitor response to CFTR modulators. Breathing reserve on CPET may provide a surrogate marker to monitor the improvement in CF lung disease with CFTR modulator treatment.en_US
rioxxterms.funder.project94a427429a5bcfef7dd04c33360d80cden_US


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