Regional Phenotyping of Cystic Fibrosis Lung Disease and Non-CF Bronchiectasis
Children's Hospital Medical Center, Cincinnati
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.
Description
Specific Aim 1: Validate functional imaging markers in CF patients with normal spirometry but abnormal ventilation. The Investigators hypothesize imaging phenotype can predict lung function decline, even in CF patients with normal spirometry. The Investigators will test this hypothesis by performing annual HP 129Xe ventilation MRI in a cohort of CF patients of which a subset has normal FEV1 (≥85% predicted) at baseline. Specific Aim 2: Determine the sensitivity and specificity of early structural remodeling in CF lung disease. The Investigator's preliminary studies show that imaging markers o…
Eligibility
- Age range
- 5–100 years
- Sex
- All
- Healthy volunteers
- Yes
Inclusion Criteria: * 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. Exclusion Criteria: * Patients meeting standard MRI exclusions criteria (non-MRI-compatible metal implants, claustrophobia, etc.) * Pregnancy or lactation. Inclusion Criteria of Healthy Subjects: * Subjects 5 years of age and older with no known history of cardiopulmonary di…
Interventions
- DrugXenon
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.
Location
- Penny NewCincinnati, Ohio