NCT04549077Active, not recruiting
Pulmonary Vascular Disease in CF
Pulmonary Vascular Disease in Cystic Fibrosis
Source: ClinicalTrials.gov · Last updated 14 Sept 2026
Brief summary (registry, in English)
In this project, the investigators seek to understand the role of endothelial cells in Cystic Fibrosis (CF) lung disease. This objective will be achieved by conducting a cross sectional clinical study to define the morphology of the pulmonary circulation across a range of lung function coupled with a mechanistic study of the effect of dysfunctional cystic fibrosis transmembrane conductance regulator (CFTR) in endothelial cells on vasculogenesis, epithelial morphogenesis and epithelial CFTR function. Toward that end, the investigators propose the following hypotheses; (a). Loss of pulmonary small blood vessels begins early in the CF lung and worsens with disease progression, (b).VEGFR2-CFTR interactions happen at the plasma membrane of endothelial cells and is likely to be involved in transendothelial ion transport (c) impaired VEGFR2-CFTR interactions on the endothelial cells will have a profound effect on vasculogenesis, epithelial morphogenesis and ion transport. The first hypotheses will be tested through this clinical study. The following 2 hypotheses will be tested through laboratory studies that do not involve human subjects.
- Status (registry)
- Active, not recruiting
- Study type
- Observational
- Enrollment
- 86 (actual)
- First submitted
- 9 Aug 2020
- Start date
- 29 Jul 2020
- Primary completion date
- 14 Nov 2024
- Lead sponsor
- Children's Hospital Medical Center, Cincinnati
- Diseases
- Cystic fibrosis
- NCT number
- NCT04549077
What you commit to
- Study type: Observational
- Participants enrolled: 86
- From 29 Jul 2020 to the primary completion date, 14 Nov 2024
- 2 sites in 1 country
- Visits and tests: ask the trial team, the registry does not list them
Built from the registry fields of ClinicalTrials.gov. It describes the study, not your situation.
Who cannot take part
Copied verbatim from ClinicalTrials.gov and grouped by topic. Only the trial team decides who can take part.
Prior treatment
- Enrollment in gene therapy trial
Other
- Enrollment in clinical trials of CFTR correctors and or potentiator
- Pregnancy.
- Historical Controls
Who the study is looking for
- 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)
- solid tumor diagnosis
- had chest CT scan to survey possible metastasis or any other lung disease
- age and gender matched to Cystic Fibrosis patients
Criteria the registry did not label
- Cystic Fibrosis Patients:
Age in the registry: 5 years to 21 years · Sex: All · Does not accept healthy volunteers
Sites (2)
Sites in registry order. Pick your city on the disease page to sort them by distance.
| Site | City | Country | Site status |
|---|---|---|---|
| Cincinnati Children's Hospital Medical Center Coordinator of this site? Confirm its status | Cincinnati | United States | Unknown status |
| Riley Hospital for Children Coordinator of this site? Confirm its status | Indianapolis | United States | Unknown status |
This page is information, not medical advice. Discuss any trial with your doctor.