1. Cystic Fibrosis in children
Conditions
Interventions
After informed consent children with CF scheduled (for annual check up) for a proton MRI will also undergo a Gadolinium-MRI.
Sponsors
Investigator initiated trial
Eligibility
Inclusion criteria
Inclusion criteria: 1. Age between 6-18 years old; 2. Diagnosis CF confirmed; 3. Stable condition; 4. Ability to perform lung function tests and breath hold tests; 5. CT can made in the year before the MRI; 6. Signed written informed consent; 7. Able to comply with the protocol requirements.
Exclusion criteria
Exclusion criteria: 1. Inability to follow instructions of the investigator; 2. Current respiratory tract infection; 3. Requiring iv antibiotics; 4. Pulmonary complications; 5. Claustrofobia; 6. History of anaphylactic reaction on contrast agent; 7. Any clinical condition which, according to the treating physician, might put the patient at risk; 8. Severe astma and/or severe allergies as determined by physician.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The main study parameter will be the ability of the radiologist and CF-team to determine whether hypo perfusion of the lung is present in the Gd-MRI and whether this matches the area of trapped air on the proton-MRI image. And secondly whether the radiologist and CF-team can determine whether there is progression of peripheral airway disease as evaluated on Gd-MRI relative to the CT made in the previous year. This will be rated by the radiologist and the physician using a standardized evaluation form. To evaluate whether this clinical practice is reproducible, MRI, Gd-MRI and CT scans will be scored in random order by two experienced independent observers. These observers will score independently of each other and they will be blinded as to patient characteristics. The scoring will be done using a semi-quantitative scoring system to estimate the volume% of trapped air and hypo perfusion. The volume% of trapped air on the proton-MRI will be compared to the volume% of hypo perfusion on the Gd-MRI. In addition, volume% of trapped air on the proton-MRI and hypo perfusion on the Gd-MRI will be compared to the volume% of trapped air on the chest CT made in the previous year. | — |
Secondary
| Measure | Time frame |
|---|---|
| To evaluate within observer variability random subsets of ten scans will be scored a second time after one month. To correlate the volume of hypo perfusion on Gd-MRI with volume% of trapped air on CT and with PFTs parameters of peripheral airway disease (FEF75, FEF25-75, LCI) | — |
Contacts
Public ContactE. Wiel van der
Erasmus MC - Sophia Children’s Hospital Room Sb2778 Wytemaweg 80
Outcome results
None listed