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- W4312614344 abstract "<b>Objective:</b> CFTR modulators elexacaftor/tezacaftor/ivacaftor has changed the treatment approach. It has been shown to improve FEV<sub>1</sub>. The impact on respiratory physiology needs to be investigated further, since lung function has only been thoroughly evaluated by spirometry. <b>Methods:</b> This retrospective, single center, observational study has looked at cystic fibrosis patients before the initiation of triple therapy and at follow-up after 12 weeks. Spirometry, full body plethysmography, diffusion capacity, health related quality of life (HRQL) and exercise testing were performed. <b>Results:</b> 38 patients were included: 52.6% female, age 37.2±11.6 years. The analysis confirms significant improvements in FEV<sub>1</sub> and VC; consequently, the Tiffenau-Index only sparsely increased from 59±14% to 62±13%. Hyperinflation significantly improved following treatment. The primary outcome, sRAWtot, massively improved following treatment. The current analysis confirms improvements in exercise capacity and HRQL (Table 1). Finally, the strongest association for improvements in exercise capacity was found for sRAWtot (0.593;<0.001). <b>Conclusion:</b> The combination of CFTR modulation therapy and physiotherapy aimed at airway clearance improves airway resistance and pulmonary hyperinflation. This is most reliably assessed by measuring sRAWtot. Thus, futures studies should also incorporate body plethysmography assessments." @default.
- W4312614344 created "2023-01-05" @default.
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- W4312614344 date "2022-09-04" @default.
- W4312614344 modified "2023-10-13" @default.
- W4312614344 title "Impact of elexacaftor/tezacaftor/ivacaftor combination therapy on lung function in adults with cystic fibrosis: Beyond FEV1" @default.
- W4312614344 doi "https://doi.org/10.1183/13993003.congress-2022.369" @default.
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