Pulmonary Vascular Disease in CF (NCT04549077)
Pulmonary Vascular Disease in Cystic Fibrosis
🩺 Plain-English Summary (8th-Grade Level)
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.
Who can join: Review the eligibility criteria below.
Location: Riley Hospital for Children — Indianapolis, Indiana
Age range: 5 Years to 21 Years
📋 "Do I Qualify?" — 1-Minute Self Screener
Instant ChecklistFull eligibility criteria are available on the official NIH protocol. View the criteria ↗
📍 All Participating Trial Locations (2 Sites)
- Riley Hospital for Children — Indianapolis, Indiana
- Cincinnati Children's Hospital Medical Center — Cincinnati, Ohio
💰 Cost, Insurance & Patient Rights
- 100% Free Items: The investigational treatment and protocol-required procedures are supplied at zero cost by the sponsor.
- Routine Patient Care: Under federal law (ACA § 2709) and many state statutes, routine care costs are covered during participation.
- Voluntary Participation: You may withdraw at any time without affecting your standard medical care.
More studies in Cystic Fibrosis
- Genetic Modifiers of Cystic Fibrosis (CF) Liver Disease — Chapel Hill, North Carolina
- Evaluation of VX-121/Tezacaftor/Deutivacaftor in Cystic Fibrosis (CF) Participants 1 Through 11 Years of Age — Orange, California
- Hyperpolarized Imaging for New Treatments — Cincinnati, Ohio
- Monitoring Response to Orkambi in Cystic Fibrosis Lung Disease by Inhaled Xenon MRI — Cincinnati, Ohio
- Evaluation of Long-term Safety and Efficacy of ELX/TEZ/IVA in Cystic Fibrosis Participants 12 Months of Age and Older — Nedlands,
- Non-contrast Lung Perfusion Mapping Applied for New Insights in Cystic Fibrosis — Cincinnati, Ohio
Explore by condition: All Cystic Fibrosis clinical trials
💡 How to Participate
- Check the checklist above to see if you may qualify.
- Review the official NIH record below to find the coordinating site.
- Ask about the visit schedule, what is covered at no cost, and any travel support.
- Confirm with your own doctor before making a decision.