One year effect of tezacaftor and ivacaftor on functional exercise capacity and muscle strength in people with cystic fibrosis
Background: The 1-min sit-to-stand test (1STST) is a practical tool to evaluate physical capacity. The aim of this study was to assess the impact of tezacaftor and ivacaftor on functional exercise capacity, muscle strength and symptoms in people with cystic fibrosis (PwCF). Methods: The assessments...
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| Format: | Article |
| Language: | English |
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Elsevier
2024-03-01
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| Series: | Heliyon |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2405844024027609 |
| _version_ | 1797259902638358528 |
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| author | Aubriot Anne-Sophie Morgane Penelle Gonçalvès Clémence Silvia Berardis Christophe Goubau Gregory Reychler Sophie Gohy |
| author_facet | Aubriot Anne-Sophie Morgane Penelle Gonçalvès Clémence Silvia Berardis Christophe Goubau Gregory Reychler Sophie Gohy |
| author_sort | Aubriot Anne-Sophie |
| collection | DOAJ |
| description | Background: The 1-min sit-to-stand test (1STST) is a practical tool to evaluate physical capacity. The aim of this study was to assess the impact of tezacaftor and ivacaftor on functional exercise capacity, muscle strength and symptoms in people with cystic fibrosis (PwCF). Methods: The assessments were performed during the first year of tezacaftor and ivacaftor using the 1STST, 6-min walk test (6MWT), MicroFET2 dynamometer®, CF Questionnaire-Revised (CFQ-R), Leicester Cough Questionnaire (LCQ). Forced expiratory volume in 1 s (FEV1), body mass index (BMI), pancreatic sufficiency status, genotype and microbiologic data were also collected. Results: Fifty-four PwCF participated to the study and took at least one dose of tezacaftor-ivacaftor. Mean age was 26y±10 (±SD), median BMI 20.9 kg/m2 (interquartile range) (19.4; 23.5) and mean FEV1 82 percent of predicted values (%PV) ± 21. Significant correlations were found at baseline between the 1STST and the 6MWT (r = 0.617, p < 0.0001), the quadriceps strength (r = 0.6556, p < 0.0001) and the FEV1 (r = 0.29, p = 0.03). After one year of treatment, the 1STST increased significantly in terms of number of repetitions (n) (median 50 versus 58.5, p < 0.0001), %PV (101.1 versus 115.2%PV, p = 0.0003) and n times weight in kg (2885 versus 3389nxkg, p < 0.0001). The 6MWT distance and quadriceps strength were not modified after treatment but during the 6MWT, oxygen desaturation decreased significantly. FEV1, BMI, CFQ-R, LCQ improved as previously demonstrated. Conclusion: After one year of tezacaftor and ivacaftor, the 1STST improves, suggesting that the 1STST seems more responsive than the 6MWT and the MicroFET2 dynamometer® to assess the effects of CFTR modulators. |
| first_indexed | 2024-03-07T20:04:36Z |
| format | Article |
| id | doaj.art-f0a8163849b742329c597260d78a0938 |
| institution | Directory Open Access Journal |
| issn | 2405-8440 |
| language | English |
| last_indexed | 2024-04-24T23:16:49Z |
| publishDate | 2024-03-01 |
| publisher | Elsevier |
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| series | Heliyon |
| spelling | doaj.art-f0a8163849b742329c597260d78a09382024-03-17T07:56:06ZengElsevierHeliyon2405-84402024-03-01105e26729One year effect of tezacaftor and ivacaftor on functional exercise capacity and muscle strength in people with cystic fibrosisAubriot Anne-Sophie0Morgane Penelle1Gonçalvès Clémence2Silvia Berardis3Christophe Goubau4Gregory Reychler5Sophie Gohy6Département de pédiatrie, Cliniques universitaires Saint-Luc, Brussels, Belgium; Cystic Fibrosis Reference Centre, Cliniques universitaires Saint-Luc, Brussels, BelgiumDépartement de pédiatrie, Cliniques universitaires Saint-Luc, Brussels, Belgium; Cystic Fibrosis Reference Centre, Cliniques universitaires Saint-Luc, Brussels, BelgiumDépartement de pédiatrie, Cliniques universitaires Saint-Luc, Brussels, Belgium; Cystic Fibrosis Reference Centre, Cliniques universitaires Saint-Luc, Brussels, BelgiumDépartement de pédiatrie, Cliniques universitaires Saint-Luc, Brussels, Belgium; Cystic Fibrosis Reference Centre, Cliniques universitaires Saint-Luc, Brussels, BelgiumDépartement de pédiatrie, Cliniques universitaires Saint-Luc, Brussels, Belgium; Cystic Fibrosis Reference Centre, Cliniques universitaires Saint-Luc, Brussels, BelgiumService de pneumologie, Cliniques universitaires Saint-Luc, Brussels, Belgium; Pole of Pneumology, ENT and Dermatology, Université catholique de Louvain (UCLouvain), Avenue Hippocrate 54/B1-54.04, B-1200 Brussels, BelgiumCystic Fibrosis Reference Centre, Cliniques universitaires Saint-Luc, Brussels, Belgium; Service de pneumologie, Cliniques universitaires Saint-Luc, Brussels, Belgium; Pole of Pneumology, ENT and Dermatology, Université catholique de Louvain (UCLouvain), Avenue Hippocrate 54/B1-54.04, B-1200 Brussels, Belgium; Corresponding author. Cliniques universitaires Saint-Luc, Avenue Hippocrate 10, 1200 Brussels – Belgium.Background: The 1-min sit-to-stand test (1STST) is a practical tool to evaluate physical capacity. The aim of this study was to assess the impact of tezacaftor and ivacaftor on functional exercise capacity, muscle strength and symptoms in people with cystic fibrosis (PwCF). Methods: The assessments were performed during the first year of tezacaftor and ivacaftor using the 1STST, 6-min walk test (6MWT), MicroFET2 dynamometer®, CF Questionnaire-Revised (CFQ-R), Leicester Cough Questionnaire (LCQ). Forced expiratory volume in 1 s (FEV1), body mass index (BMI), pancreatic sufficiency status, genotype and microbiologic data were also collected. Results: Fifty-four PwCF participated to the study and took at least one dose of tezacaftor-ivacaftor. Mean age was 26y±10 (±SD), median BMI 20.9 kg/m2 (interquartile range) (19.4; 23.5) and mean FEV1 82 percent of predicted values (%PV) ± 21. Significant correlations were found at baseline between the 1STST and the 6MWT (r = 0.617, p < 0.0001), the quadriceps strength (r = 0.6556, p < 0.0001) and the FEV1 (r = 0.29, p = 0.03). After one year of treatment, the 1STST increased significantly in terms of number of repetitions (n) (median 50 versus 58.5, p < 0.0001), %PV (101.1 versus 115.2%PV, p = 0.0003) and n times weight in kg (2885 versus 3389nxkg, p < 0.0001). The 6MWT distance and quadriceps strength were not modified after treatment but during the 6MWT, oxygen desaturation decreased significantly. FEV1, BMI, CFQ-R, LCQ improved as previously demonstrated. Conclusion: After one year of tezacaftor and ivacaftor, the 1STST improves, suggesting that the 1STST seems more responsive than the 6MWT and the MicroFET2 dynamometer® to assess the effects of CFTR modulators.http://www.sciencedirect.com/science/article/pii/S2405844024027609Cystic fibrosisQuadriceps strengthFunctional exercise capacityOne-minute sit-to-stand testCFTR modulators |
| spellingShingle | Aubriot Anne-Sophie Morgane Penelle Gonçalvès Clémence Silvia Berardis Christophe Goubau Gregory Reychler Sophie Gohy One year effect of tezacaftor and ivacaftor on functional exercise capacity and muscle strength in people with cystic fibrosis Heliyon Cystic fibrosis Quadriceps strength Functional exercise capacity One-minute sit-to-stand test CFTR modulators |
| title | One year effect of tezacaftor and ivacaftor on functional exercise capacity and muscle strength in people with cystic fibrosis |
| title_full | One year effect of tezacaftor and ivacaftor on functional exercise capacity and muscle strength in people with cystic fibrosis |
| title_fullStr | One year effect of tezacaftor and ivacaftor on functional exercise capacity and muscle strength in people with cystic fibrosis |
| title_full_unstemmed | One year effect of tezacaftor and ivacaftor on functional exercise capacity and muscle strength in people with cystic fibrosis |
| title_short | One year effect of tezacaftor and ivacaftor on functional exercise capacity and muscle strength in people with cystic fibrosis |
| title_sort | one year effect of tezacaftor and ivacaftor on functional exercise capacity and muscle strength in people with cystic fibrosis |
| topic | Cystic fibrosis Quadriceps strength Functional exercise capacity One-minute sit-to-stand test CFTR modulators |
| url | http://www.sciencedirect.com/science/article/pii/S2405844024027609 |
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