http://www.ncbi.nlm.nih.gov/pubmed/23099448

Chest. 2012 Oct 22. doi: 10.1378/chest.12-1934. [Epub ahead of print]

Evidence of Vascular Endothelial Dysfunction in Young Patients with Cystic Fibrosis.

Poore1 S, Berry1 B, Eidson2 D, McKie2 KT, Harris1 RA.

Abstract

ABSTRACT BACKGROUND:

Cystic fibrosis (CF) is a genetic disorder that affects not only pulmonary function, but also multiple organ systems. The flow-mediated dilation (FMD) test is a non-invasive assessment of endothelial function and nitric oxide bioavailability. Thus, the purpose of this study was to determine if 1) endothelial dysfunction is present in young patients with CF, and 2) endothelial function is associated with pulmonary function and exercise capacity.

METHODS:

15 patients with CF and 15 demographically matched controls participated in this study. Spirometry, brachial artery FMD, and a maximal exercise capacity test on a cycle ergometer were performed on all subjects to determine pulmonary function, endothelial function, and exercise capacity, respectively.

RESULTS:

No differences (p>0.05) in age, height, or BMI were observed between patients with CF and controls. FEV1 (% predicted), FEV1/FVC, and FEF25-75 were lower in patients with CF. VO2 peak (absolute and relative) was similar between groups; however, VO2 (% predicted and ml/kg fat free mass/min) and peak workload were significantly (p<0.05) lower in patients with CF. FMD (4.9±2.6 vs. 7.5±3.1%; p=0.018) was lower in patients compared to controls, respectively. Relationships between FMD and both pulmonary function and exercise capacity were identified.

CONCLUSIONS:

For the first time, these data provide evidence of vascular endothelial dysfunction in a fairly healthy cohort of young patients with CF. In addition, our data demonstrates the complex relationships between endothelial function and both pulmonary function and exercise capacity in young patients with CF.