Cystic Fibrosis
Conditions
Keywords
Cystic fibrosis, CFTR modulators, Physical activity, Muscle function
Brief summary
Cystic fibrosis (CF) is characterized by various extrapulmonary manifestations, including altered skeletal muscle function, with both quantitative (e.g. reduced muscle mass) and qualitative (e.g. impaired oxidative function) impairments that may have a negative impact on exercise tolerance and quality of life. These abnormalities have traditionally been related to disease (e.g. systemic inflammation) or behavioral factors (e.g. increased physical inactivity). However, most of the studies that observed these abnormalities and tried to shed light on the underlying factors were either small or conducted before the widespread of CFTR (Cystic fibrosis transmembrane conductance regulator) modulators that have profound impact on the trajectory of the disease. While several studies suggested that the major recent improvements in therapeutics, including highly effective CFTR modulators, may have positive effects on skeletal muscle function, either directly (e.g. improved mitochondrial function) or indirectly (e.g. reduction in physical inactivity), no studies to date have thoroughly investigated this issue in a representative sample of people with CF. The absence of recent data on muscle function and physical activity levels casts doubt on the relevance of recommendations on exercise training in this population that were published before the widespread use of highly effective CFTR modulators. This study aims to compare muscle function, measured according to the latest recommendations of the European Cystic Fibrosis Society (Saynor et al., 2023), and physical activity of children and adults with CF under CFTR modulators, compared to age- and sex-matched healthy individuals. We hypothesize that the strength, endurance, muscle power, and physical activity levels of individuals with cystic fibrosis, treated with CFTR modulators, remain reduced compared to healthy individuals.
Interventions
OTHERVolumetric ultrasound of the quadriceps and fitting of an accelerometer
The research procedure will simply involve a volumetric ultrasound of the quadriceps and the fitting of an accelerometer (watch worn on the wrist) for one week (collection of usual physical activities).
Sponsors
Hospices Civils de Lyon
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
10 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
* Criteria for inclusion of CF patients: * Diagnosis of cystic fibrosis * Treated with CFTR modulator therapy * Men and women (children or adults) aged 10 years or older * Affiliated with or entitled to social security coverage * For people ≥ 18 years old: participant expressing its oral non-opposition * For minor child (\<18 years old): participant and its legal representative expressing their oral non-opposition * Criteria for inclusion of healthy subjects: * Men and women (children or adults) aged 10 years or older * Affiliated with or entitled to social security coverage * For people ≥ 18 years old: participant expressing its oral non-opposition * For minor child (\<18 years old): participant and its legal representative expressing their oral non-opposition
Exclusion criteria
*
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Measurement of isometric quadriceps strength (expressed in Newton), performed at René Sabran Hospital (as part of routine clinical practice for people with CF) | Day 0 | The quadriceps strength will be measured on an isometric chair according to the last recommendations of the European Cystic Fibrosis Society (Saynor et al., 2023). Briefly, participants will perform at least three maximal voluntary contractions of the knee extensors, each lasting 4-6s, with a minimal recovery of 1-min between each attempt, with the aim to obtain three maximal quadriceps strength values varying less than 5% (that is usually obtained in less than 6 trials). The highest strength value among these three attempts will be kept for analysis. |
Countries
France
Contacts
Primary ContactLaurent MD MELY
Backup ContactAntoine-Raphaël MD Bronstein
Outcome results
None listed