Impact d'un Programme coordonné diététique-activité Physique adaptée Sur le Pourcentage de Masse Maigre d'Adultes Atteints de Mucoviscidose traités Par Elexacaftor-Tezacaftor-Ivacaftor : Essai contrôlé randomisé Multicentrique
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 100
- 试验地点
- 8
- 主要终点
- Evolution of percentage of patients lean mass as a percentage of body mass
研究概览
简要总结
Cystic fibrosis is an autosomal recessive inherited disease linked to various mutations in the gene coding for the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein, with respiratory and digestive disorders conditioning the prognosis.
Digestive damage may be responsible for malnutrition of multifactorial origin (insufficient energy intake, increased energy losses, increased basal metabolic rate), and studies show a correlation between reduced lean body mass and respiratory function.
In 2019, the French National Authority for Health (HAS) redefined undernutrition by including "quantified reduction in muscle mass and/or function" as a phenotypic diagnostic criterion.
Elexacaftor-Tezacaftor-Ivacaftor, an innovative therapy (authorization in 2021) for this population, aims to restore the function of CFTR protein. Significant improvements in lung function and weight gain were observed from the first weeks of treatment. These improvements have also led to the emergence of lesser-known nutritional problems in these patients, such as overweight and the development of metabolic complications. Nonetheless, new management options in terms of dietary adjustments and adapted physical activity for these patients are possible, given the development of their abilities.
Adapted Physical Activity (APA) helps to improve general muscular function by strengthening respiratory and skeletal muscles, improving aerobic capacity, and aiding bronchial drainage through muscle strengthening and endurance work. Maintaining or even increasing muscle mass depends not only on appropriate food intake and optimal dietary management, but also on regular physical activity, as recommended by the HAS.
Our hypothesis is therefore that a structured dietetic/adapted physical activity program (DIAPASOM program) can increase the percentage of lean body mass at 12 months in adult cystic fibrosis patients treated with Elexacaftor-Tezacaftor-Ivacaftor.
详细描述
Cystic fibrosis is an autosomal recessive inherited disease linked to various mutations in the gene coding for the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein, with respiratory and digestive disorders conditioning the prognosis.
Digestive damage may be responsible for malnutrition of multifactorial origin (insufficient energy intake, increased energy losses, increased basal metabolic rate), and studies show a correlation between reduced lean body mass and respiratory function.
In 2019, the French National Authority for Health (HAS) redefined undernutrition by including "quantified reduction in muscle mass and/or function" as a phenotypic diagnostic criterion.
Elexacaftor-Tezacaftor-Ivacaftor, an innovative therapy (authorization in 2021) for this population, aims to restore the function of CFTR protein. Significant improvements in lung function and weight gain were observed from the first weeks of treatment. These improvements have also led to the emergence of lesser-known nutritional problems in these patients, such as overweight and the development of metabolic complications. Nonetheless, new management options in terms of dietary adjustments and adapted physical activity for these patients are possible, given the development of their abilities.
Adapted Physical Activity (APA) helps to improve general muscular function by strengthening respiratory and skeletal muscles, improving aerobic capacity, and aiding bronchial drainage through muscle strengthening and endurance work. Maintaining or even increasing muscle mass depends not only on appropriate food intake and optimal dietary management, but also on regular physical activity, as recommended by the HAS.
研究设计
- 研究类型
- Interventional
- 分配方式
- Randomized
- 干预模型
- Parallel
- 主要目的
- Supportive Care
- 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
- •Subject aged 18 or over
- •Suffering from cystic fibrosis
- •Treated with Elexacaftor-Tezacaftor-Ivacaftor for at least 6 months
- •Affiliated to a social security scheme
- •with a signed Informed Consent form.
排除标准
- •Pregnant and breast-feeding women
- •Subject under legal protection, guardianship or curatorship
- •Subject whose physical activity is not medically authorised or whose physical and motor capacities do not allow them to take part in physical activity.
- •Subject who is unable to comply with the requirements of the DIAPASOM program
- •Difficulty in understanding the self-questionnaires
- •Wearing a pacemaker or metal prosthesis
- •Fluid retention
结局指标
主要结局
Evolution of percentage of patients lean mass as a percentage of body mass
时间窗: From randomization, up to 12 months
Impedancemetry
次要结局
- Cardio-respiratory endurance(From randomization, up to 12 months)
- Bilateral Handgrip strength(From randomization, up to 12 months)
- Evolution of percentage of patients fat mass as a percentage of body mass(From randomization, up to 12 months)
- Weight evolution(From randomization, up to 12 months)
- Body Mass Index (BMI) evolution(From randomization, up to 12 months)
- Lower limb muscle power(From randomization, up to 12 months)
- Upper limb muscle power(From randomization, up to 12 months)
- Static trunck extensors muscle endurance(From randomization, up to 12 months)
- Evolution of quality of life(From randomization, up to 12 months)
- Static trunck flexors muscle endurance(From randomization, up to 12 months)
- Balance between static muscular endurance of extensors and flexors(From randomization, up to 12 months)
- Posterior chain flexibility (Hamstring, hips and lower back)(From randomization, up to 12 months)
- Upper limb flexibility(From randomization, up to 12 months)
- Forced Expiratory Volume in 1 second (FEV1)(From randomization, up to 12 months)
- Physical activity volume and sedentary time(From randomization, up to 12 months)
- Program feedback questionnaire(12 months after randomization)