RECRUITINGOBSERVATIONAL

Regional Phenotyping of CF and Non-CF Bronchiectasis

Regional Phenotyping of Cystic Fibrosis Lung Disease and Non-CF Bronchiectasis

About This Trial

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.

Who May Be Eligible (Plain English)

Who May Qualify: - 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. Who Should NOT Join This Trial: - 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 disease. Exclusion Criteria of Healthy Subjects: - Patients meeting standard MRI exclusions criteria (non-MRI-compatible metal implants, claustrophobia, etc.) - Pregnancy or lactation. Always talk to your doctor about whether this trial is right for you.

Original Eligibility Criteria

View original clinical language

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 disease. Exclusion Criteria of Healthy Subjects: * Patients meeting standard MRI exclusions criteria (non-MRI-compatible metal implants, claustrophobia, etc.) * Pregnancy or lactation.

Treatments Being Tested

DRUG

Xenon

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)

Penny New

Cincinnati, Ohio, United States