Cystic Fibrosis
Conditions
Keywords
Magnetic resonance imaging, Multidetector computed tomography
Brief summary
The purpose of this study is to compare the performances of ultra-low dose computed tomography (CT) and lung magnetic resonance imaging (MRI) for morphological assessment of cystic fibrosis-related lung disease and to compare their performances to conventional low dose CT
Detailed description
Cystic fibrosis (CF) is a recessive autosomal disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that encodes for an epithelial chloride channel involved in ion and fluid transport. CF is the most common inherited disease in Caucasians and disease severity mainly depends on the degree of lung involvement, which can lead to terminal respiratory failure Disease monitoring of CF-related lung disease rely on functional assessment and complimentary morphological assessment. Conventional low-dose chest computed tomography (CT) is currently the gold standard for the morphological assessment of CF-related lung disease but ultra-low dose chest CT and high-resolution magnetic resonance imaging (MRI) of the lung using UTE sequences have been recently developed and allow important radiation reduction of radiation dose exposure. However the performances of these 2 competing imaging methods remains to be compared.
Interventions
DIAGNOSTIC_TESTCT scanner
1 conventional low dose inspiratory acquisition and 1 ultra-low-dose inspiratory acquisition
DIAGNOSTIC_TESTLung MRI
3 UTE sequences (free breathing, inspiratory and expiratory) and 1 T2 sequence
Sponsors
Assistance Publique - Hôpitaux de Paris
URC-CIC Paris Descartes Necker Cochin
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Age ≥ 18 year-old * Diagnosis of cystic fibrosis provided by genetic and swear test * Chest CT acquisition performed as part of the standard follow-up * Patient with social security or health insurance * Informed consent
Exclusion criteria
* MRI contraindication * Orthopnea * Inability to hold breath for 17 seconds * No spirometry planned the same day * Lung transplant patient
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Reproducibility of visual score between imaging modalities | 1 day | Reproducibility disease severity measured by the Helbich scoring system with conventional CT as gold standard |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Intra and interobserver reproducibility of visual scores | 1 day | Intra and interobserver reproducibility of the Helbich score for each imaging modality |
| Correlation between visual scores and pulmonary function | 1 day | Correlation between the Helbich score for each imaging modality and forced expiratory volume in 1 second (FEV1) |
| Correlation between air trapping at MRI and pulmonary function test | 1 day | Correlation between air trapping, measured by comparing inspiratory to expiratory MRI images, and forced expiratory volume at one second (FEV1) |
Countries
France
Contacts
STUDY_DIRECTORMarie-Pierre REVEL, MD, PhD
Assistance Publique - Hôpitaux de Paris
Outcome results
None listed