Recruiting

Observational Study

Sponsor:

Children's Hospital Medical Center, Cincinnati

Code:

NCT04793867

Conditions

Cystic Fibrosis

Non-CF Bronchiectasis

Eligibility Criteria

Sex: All

Age: 5 - 70+

Healthy Volunteers: Accepted

Interventions

Xenon

Study Details

Brief 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.

Conditions

Cystic Fibrosis

Non-CF Bronchiectasis

Study ID

NCT04793867

Start date

Feb 8, 2021

Status verified date

Feb, 2025

Completion date

Mar 31, 2027

Anticipated

Primary completion date

Mar 31, 2026

Anticipated

Eligibility Criteria

Eligibility Criteria

Sex: All

Age: 5 - 70+

Healthy Volunteers: Accepted

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.

Study Design

Enrollment

100 participants

Anticipated

Interventions and Outcome Measures

Arms

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.

Interventions

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.

Primary outcome measure

  • Correlation of ventilated volume predictions obtained with 129Xe MRI vs 1H MRI [ Time Frame: Annually for 5 years ]

Central Contacts and Locations

Central contacts

Penny A New, BBA

513-332-7931penny.new@cchmc.org

Carrie Stevens, BS

513-636-9973carrie.stevens@cchmc.org

Locations

Penny New

Recruiting

Cincinnati, Ohio, United States, 45229

Contacts

Penny A New, BBA

513-332-7931penny.new@cchmc.org

Principal Investigator:

Zackary Cleveland, PhD

More Information

Sponsor

Children's Hospital Medical Center, Cincinnati

Last update posted

Feb 20, 2025

Last verified

Feb, 2025

Keywords

  • Cystic Fibrosis
  • hyperpolarized xenon
  • LCI
  • Non-CF Bronchiectasis

Trial information was received from ClinicalTrials.gov and was last updated on 2026-09-10. This information was provided to ClinicalTrials.gov by Children's Hospital Medical Center, Cincinnati on 2025-02-20.