Prevalence of Exercise-induced Ventilatory Limitation and Associated Factors in Patients With Cystic Fibrosis Receiving Elexacaftor-Tezacaftor-Ivacaftor
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 130
- 试验地点
- 2
- 主要终点
- Estimate the prevalence of ventilatory limitation measured by a portable spirometer during a submaximal exercise test (6-minute walk test) in patients with cystic fibrosis undergoing ETI.
研究概览
简要总结
Cystic fibrosis is a genetic disorder affecting the entire body and associated with respiratory exacerbations, impaired quality of life and reduced life expectancy. The therapeutic management of cystic fibrosis has been profoundly changed by the recent arrival of a combination of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulators, Elexacaftor-Tezacaftor-Ivacaftor (ETI), which improve quality of life, respiratory function and reducing the number of exacerbations. The impact of these treatments on exercise adaptation has not been clearly identified.
The main objective is to estimate the prevalence of ventilatory reserve amputation during submaximal exercise testing assessed by the 6-minute walk test (6MWT) in patients with cystic fibrosis treated with ETIs.
详细描述
Cystic fibrosis is a genetic disorder affecting the entire body and associated with respiratory exacerbations, impaired quality of life and reduced life expectancy. The therapeutic management of cystic fibrosis has been profoundly changed by the recent arrival of a combination of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulators, Elexacaftor-Tezacaftor-Ivacaftor (ETI), which improve quality of life, respiratory function and reducing the number of exacerbations. The impact of these treatments on exercise adaptation has not been clearly identified.
The main objective is to estimate the prevalence of ventilatory reserve amputation during submaximal exercise testing assessed by the 6-minute walk test (6MWT) in patients with cystic fibrosis treated with ETIs.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Health Services Research
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Male or female
- •Adult aged 18 or over
- •Suffering from cystic fibrosis
- •Treated at the CRCM in Lille and Créteil
- •Treated by ETI
- •Be covered by social security
- •Be able to understand the requirements of the study, provide written informed consent, and comply with the study's data collection procedures
排除标准
- •Medical contraindication or inability to perform a stress test according to ERS recommendations
- •Absolute contraindications
- •Relative contraindications:
- •Exacerbation of the condition in the 4 weeks preceding the V1 visit (27).
- •Pregnant or breastfeeding women
- •Administrative reasons
- •Persons deprived of their liberty
- •Minors or protected adults
- •Persons who have refused or are unable to give informed consent
- •Persons in emergency situations
研究组 & 干预措施
patients treated with ETI (Kaftrio-Kalydeco©)
干预措施: Measurement of resistance through forced oscillations and continuous measurement of ventilation and inspiratory capacity during TM6 (Device)
结局指标
主要结局
Estimate the prevalence of ventilatory limitation measured by a portable spirometer during a submaximal exercise test (6-minute walk test) in patients with cystic fibrosis undergoing ETI.
时间窗: duration of 56 weeks starting in December 2025
The primary endpoint is ventilatory limitation defined as the difference between the estimated theoretical maximun minute ventilatory volume (VMM in L/min) estimated by multiplying FEV1 (L) x 35 and the measurement of external ventilation during exercise (VE, L/min) using a portable spirometer divided by the theoretical VM x100 less than 15%. Ventilatory limitation = (MMV (L/min) - VE (L/min) /MMV (L/min))\*100 \< 15%
次要结局
- Estimate the prevalence of ventilatory limitation measured using a portable spirometer during a maximal incremental step test (A-STEP) with progressive steps in patients with cystic fibrosis undergoing ETI.(duration of 56 weeks starting in December 2025)
- Assessment of the prevalence of dynamic distension during a submaximal (TM6) or maximal (incremental step test according to the A-Step protocol) exercise test by spirometry(duration of 56 weeks starting in December 2025)