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| ID | Type | Description | Link |
|---|---|---|---|
| IDRCB-2013-A01175-40 | Other Identifier | IDRCB |
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| Name | Class |
|---|---|
| Vaincre la Mucoviscidose | OTHER |
| URC-CIC Paris Descartes Necker Cochin | OTHER |
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The purpose of this study is to determine the efficacy of a non invasive method of detecting the pulmonary disease in order to initiate treatment against cystic fibrosis as soon as possible.
Moreover this screening procedure permits to note the improvement following the treatment and to choose the optimal treatment in term of efficacy.
It is mandatory to detect as early as possible lung CF disease in the young child with CF to initiate active therapies limiting irreversible lesions. Lung Clearance Index (LCI) which evaluates gas clearance from the alveolar volume is an easy to-do technique in pre-school children.
Main objective To evaluate correlation between LCI and thoracic low dose computed tomodensitometry (CT) endpoints at initial visit Secondary objectives
This is a prospective multicenter cohort study. Study visits will be performed each year during the annual check up for normal follow up of the child.
Initial visit (Visit 1) takes place during an annual check-up during which a low dose CT was prescribed. It will include following tests: LCI, Lung function tests (LFTs) performed according to age (forced volumes and flows, plethysmographic specific airway resistances and interrupter resistances), and a low dose CT scan performed according a standardized protocol.
Visit 2 will be performed 12 ± 3 months later and will include following tests: LCI , LFTs.
Visit 3 will be performed 24 ± 3 months later and will include following tests: LCI , LFTs and, if judged necessary by the child's referent doctor, a low dose CT scan performed according to a standardized protocol.
Anthropometric data, infection history, treatment will be collected at each visit.
Recruiting period: 1 year Study period: 2 years per patient, 3 years as a whole LCI will help to evaluate early ventilation inhomogeneity due to small bronchi abnormalities in young children with CF. Concordance between LCI and CT endpoints evaluation will help to define the frequency of lung CT administration in young CF children. Predictive value for later abnormal lung function development will be evaluated. This will help to detect the children at risk to develop abnormal lung function and to target those requiring active treatment. Finally this endpoint will be very valuable in the future therapeutic trials for CFTR correctors or potentiators.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| LCI | Other | Measure of lung clearance index |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Lung clearance index determination | Other | Lung clearance index determination |
|
| Measure | Description | Time Frame |
|---|---|---|
| measure of nitrogen washout and Brody 2 score | Month 0 |
| Measure | Description | Time Frame |
|---|---|---|
| measure of FEV (Correlation between the evolution of LCI and respiratory function endpoints and CT) | Correlation between the evolution of LCI and respiratory function endpoints and CT | 2 years |
| measure of FEV (Evaluation of LCI predictive value for respiratory function in preschool children) |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Muriel Le Bourgeois, MD | Service de pneumologie pédiatrique, Hôpital Necker-Enfants Malades Assistance Publique Hôpitaux de Paris 75015 Paris, France | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hôpital Necker-Enfants Malades | Paris | 75015 | France |
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| ID | Term |
|---|---|
| D003550 | Cystic Fibrosis |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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Evaluation of LCI predictive value for respiratory function in preschool children. |
| 2 years |
| D030342 |
| Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |