Not provided
| ID | Type | Description | Link |
|---|---|---|---|
| 2024-A02709-38 | Registry Identifier | ID-RCB |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Class |
|---|---|
| Vaincre la Mucoviscidose | OTHER |
Not provided
Not provided
Not provided
Not provided
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.
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.
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| patients treated with ETI (Kaftrio-Kalydeco©) | Experimental |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Measurement of resistance through forced oscillations and continuous measurement of ventilation and inspiratory capacity during TM6 | Device | Measurement of resistance through forced oscillations and continuous measurement of ventilation and inspiratory capacity during TM6 |
| Measure | Description | Time Frame |
|---|---|---|
| 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. | 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% | duration of 56 weeks starting in December 2025 |
| Measure | Description | Time Frame |
|---|---|---|
| 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. | The assessment criterion is 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 (VE in L/min) using a portable spirometer during exercise divided by the theoretical VM x100 less than 15%. |
Not provided
Inclusion Criteria:
Exclusion Criteria:
Medical contraindication or inability to perform a stress test according to ERS recommendations
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
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Camille AUDOUSSET, Doctor | Contact | +33 3.20.44.41.45 | +33 | DRC@chu-lille.fr |
Not provided
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University Hospital | Recruiting | Lille | 59037 | France |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
No control group. Patients treated with ETI (Kaftrio-Kalydeco©) are treated as part of the management of their condition in accordance with the indications for marketing authorisation (MA), temporary authorisation for use (ATU) or French compassionate access.
Not provided
Not provided
Not provided
Not provided
| 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 | The criterion for judgement is a decrease in inspiratory capacity (L) of more than 150 mL within 30 seconds after the end of the effort compared to the pre-effort inspiratory capacity (L) measured by spirometry. | duration of 56 weeks starting in December 2025 |
| ID | Term |
|---|---|
| D003550 | Cystic Fibrosis |
| D009043 | Motor Activity |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |
| D001519 | Behavior |
Not provided
Not provided