Regional Phenotyping of CF and Non-CF Bronchiectasis
Regional Phenotyping of Cystic Fibrosis Lung Disease and Non-CF Bronchiectasis
Children's Hospital Medical Center, Cincinnati
100 participants
Feb 8, 2021
OBSERVATIONAL
Conditions
Summary
The Investigators propose to study pediatric subjects who are diagnosed with cystic fibrosis (CF) and patients with non-CF bronchiectasis, with the goal of developing markers of CF lung disease severity, progression, and therapy response. The Investigator's central hypothesis is that image-based markers can forecast pathophysiology prior to spirometric changes.
Eligibility
Inclusion Criteria5
- CF Patients: Diagnosis of CF based on sweat chloride >60 mMol/l
- Presence of two disease causing CFTR mutations, or end organ manifestations of disease.
- Age minimum 5 years.
- Care provided by the CCHMC CF Care Center or other regional CF Care Centers if required to achieve recruitment goals.
- Subjects 5 years of age and older with no known history of cardiopulmonary disease.
Exclusion Criteria4
- Patients meeting standard MRI exclusions criteria (non-MRI-compatible metal implants, claustrophobia, etc.)
- Pregnancy or lactation.
- Patients meeting standard MRI exclusions criteria (non-MRI-compatible metal implants, claustrophobia, etc.)
- Pregnancy or lactation.
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Interventions
Subjects will be scanned on a commercial 3T whole-body MRI scanner equipped with high-performance gradient systems. Natural isotopic abundance or isotopically-enriched xenon gas (\~86% 129Xe, Linde Inc.) will be used for all studies. Subjects will be positioned supine in the scanner with a 129Xe RF coil around their chests. Using conventional proton MRI and a breath-hold acquisition, "scout" images will be obtained to localize the subject for subsequent 129Xe acquisitions. 129Xe images will be acquired using the same breath-hold maneuver and a pulse sequence, covering the entire lung (acquisition time \<10 s, approximately 3-mm in-plane resolution, 15-mm slice thickness or less). Additional scans (sequences) may be performed. A maximum of 4 doses of 129Xe will be given throughout the study following the calibration dose.
Locations(1)
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NCT04793867