Respiratory medicine · 2025
Change in the 6-min walk test among 71 patients with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor.
СтатьяНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Perrone Julien, Rabilloud Muriel, Mely Laurent
Аннотация
BACKGROUND: Elexacaftor/tezacaftor/ivacaftor (ETI) has led to substantial improvements in the clinical outcome of people with cystic fibrosis (pwCF). However, its effects on exercise capacity remain uncertain.
METHODS: This retrospective cohort study included 71 pwCF who started ETI between March 2020 and September 2022. The best performance on the 6-min walk test (6MWT), defined as the peak walking distance achieved, was compared between the 12 months preceding ETI initiation and the first 14 months of treatment. Pulmonary function tests (PFT) and Cystic Fibrosis Questionnaire-Revised (CFQ-R) were analyzed at treatment initiation and after 12 months.
RESULTS: After starting ETI, the 6MWT was performed at a median interval of 356 [296-380] days. The mean 6-min walk distance (6MWD) was 641 m ± 85.5 at baseline. After treatment, the 6MWD showed a significant absolute increase of 15.8 m (P = 0.007). Improvement was greater in pwCF with a percent predicted FEV1 (ppFEV1) ≤40, showing a mean increase of 37.8 m (P = 0.009), and in those without prior CFTR modulator therapy with an increase of 21.6 m (P = 0.016). After 12 months, the absolute increase in ppFEV1 was 15.8 (P < 0.001). The absolute changes from baseline in CFQ-R physical and respiratory scores were 17.9 (P < 0.001) and 27 points (P < 0.001), respectively. No correlation was found between changes in 6MWT and changes in PFT results.
CONCLUSIONS: ETI improved exercise capacity in pwCF, as evidenced by a significant increase in the 6MWD. ETI was also associated with improvements in physical-related quality of life. Changes in PFT results cannot predict changes in 6MWT results after ETI therapy.
METHODS: This retrospective cohort study included 71 pwCF who started ETI between March 2020 and September 2022. The best performance on the 6-min walk test (6MWT), defined as the peak walking distance achieved, was compared between the 12 months preceding ETI initiation and the first 14 months of treatment. Pulmonary function tests (PFT) and Cystic Fibrosis Questionnaire-Revised (CFQ-R) were analyzed at treatment initiation and after 12 months.
RESULTS: After starting ETI, the 6MWT was performed at a median interval of 356 [296-380] days. The mean 6-min walk distance (6MWD) was 641 m ± 85.5 at baseline. After treatment, the 6MWD showed a significant absolute increase of 15.8 m (P = 0.007). Improvement was greater in pwCF with a percent predicted FEV1 (ppFEV1) ≤40, showing a mean increase of 37.8 m (P = 0.009), and in those without prior CFTR modulator therapy with an increase of 21.6 m (P = 0.016). After 12 months, the absolute increase in ppFEV1 was 15.8 (P < 0.001). The absolute changes from baseline in CFQ-R physical and respiratory scores were 17.9 (P < 0.001) and 27 points (P < 0.001), respectively. No correlation was found between changes in 6MWT and changes in PFT results.
CONCLUSIONS: ETI improved exercise capacity in pwCF, as evidenced by a significant increase in the 6MWD. ETI was also associated with improvements in physical-related quality of life. Changes in PFT results cannot predict changes in 6MWT results after ETI therapy.