Understanding pulmonary vascular disease in cystic fibrosis

Is this trial a fit for you?

A plain-English reading of the study's rules. Only the study team can confirm whether you qualify.

What this trial studies

This observational study aims to investigate the role of endothelial cells in lung disease associated with cystic fibrosis (CF). Researchers will conduct a cross-sectional analysis to assess the morphology of the pulmonary circulation in CF patients, focusing on the impact of dysfunctional CFTR on vascular and epithelial functions. The study will test hypotheses regarding the early loss of small pulmonary blood vessels and the interactions between VEGFR2 and CFTR in endothelial cells. By utilizing advanced imaging techniques, the study seeks to clarify the relationship between pulmonary vascular changes and lung function in CF.

Why it matters

Who can join: the full rules

Cystic Fibrosis Patients:

Inclusion Criteria:

* 5-21 years of age
* diagnosis of CF based on a positive sweat test and genetic testing
* Baseline pulmonary condition defined as a) Absence of signs and symptoms of pulmonary exacerbation, b) Baseline pulmonary function test (PFT) defined as FEV1% that is no less than 5% of the best PFT in the previous 6 months, c) Patients should be off acute antibiotics for 2 weeks or longer.
* Subjects should be able to perform an acceptable and reproducible spirometry
* Study population will be equally divided between three groups based on FEV1%, (FEV1% ≥ 90); moderate (60 ≤ FEV1% \< 90)

Exclusion Criteria:

* Enrollment in clinical trials of CFTR correctors and or potentiator
* Enrollment in gene therapy trial
* Pregnancy.

Historical Controls

Inclusion Criteria:

* solid tumor diagnosis
* had chest CT scan to survey possible metastasis or any other lung disease
* age and gender matched to Cystic Fibrosis patients

Where it's running

• Riley Hospital for Children
  Indianapolis, Indiana, United States Recruiting
• Cincinnati Children's Hospital Medical Center
  Cincinnati, Ohio, United States Recruiting

Study contacts

• Study coordinator: Raouf Amin, MD
• Email: raouf.amin@cchmc.org
• Phone: 513-636-7945

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How to take part

1. Review the eligibility criteria above with your treating physician.
2. Check the official listing on ClinicalTrials.gov for the latest contact details and recruitment status.
3. Contact the study coordinator or principal investigator to ask for pre-screening. Pre-screening is free and never obligates you to enroll.