Regional Phenotyping of Cystic Fibrosis Lung Disease and Non-CF Bronchiectasis
Status: Recruiting
Location: See location...
Intervention Type: Drug
Study Type: Observational
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
Participation Requirements
Sex: All
Minimum Age: 5
Maximum Age: 100
Healthy Volunteers: t
View:
• 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.
Locations
United States
Ohio
Penny New
RECRUITING
Cincinnati
Contact Information
Primary
Penny A New, BBA
penny.new@cchmc.org
513-332-7931
Backup
Carrie Stevens, BS
carrie.stevens@cchmc.org
513-636-9973
Time Frame
Start Date: 2021-02-08
Estimated Completion Date: 2027-03-31
Participants
Target number of participants: 100
Treatments
CF and Non-CF Bronchiectasis
In the first arm (Aims 1 \& 2) Up to 50 subjects will be recruited-approximately 25 with normal FEV1 (\>85% predicted) and 25 with mild to moderate disease. All subjects will be asked to undergo longitudinal (i.e., approximately annually) 129Xe and UTE MRI, spirometry, and lung clearance index (LCI) measurement.
Healthy Subjects
Up to 50 age and sex matched control subjects (i.e., subjects with no known cardiopulmonary disorders) may also be recruited to provide a reference data set from healthy subjects for comparison.
Related Therapeutic Areas
Sponsors
Leads: Children's Hospital Medical Center, Cincinnati