Is systemic artery function compromised in young patients with cystic fibrosis (CF)?
Why is this important?
Arteries help transport oxygen to tissues throughout the body. Majority of drug therapies for CF target the lungs, the pancreas, and the intestines. These therapies are helping patients with CF live longer and, as a result, additional systemic consequences of this sort may become evident.
What did you do?
The flow-mediated dilation (FMD) test is a non-invasive, ultrasound assessment of artery function. We evaluated pulmonary function, exercise capacity, and FMD in 15 young patients with CF and 15 healthy controls of similar age, sex, and BMI’s of those with CF.
What did you find?
Patients with CF had fairly normal spirometry; however, their lung function was statistically lower than healthy controls. We also found that compared to control subjects, there was evidence of exercise intolerance in young patients with CF. Perhaps the most important findings of this study were that young patients with CF showed signs of systemic artery dysfunction even though their lungs were functioning fairly well.
What does this mean and reasons for caution?
This is the first study to identify artery dysfunction in young patients with CF who exhibit a fairly healthy lung function. Arteries play an essential role in oxygen transport and delivery.
We are working on determining what is causing the artery dysfunction and whether it is only during exercise that the arteries are not working to their fullest. These future studies may result in 1) additional therapies that target systemic artery function, and 2) determining why patients with CF have exercise intolerance in the presence of largely unimpaired lung function.