Endothelial dysfunction in children and young adults with cystic fibrosis – A combined diagnostic approach of plethysmographic and biochemical markers: New applications

Abstract

Introduction: Cystic fibrosis (CF) is a genetic disease that primarily affects the lungs and the digestive system. Asymmetric dimethylarginine (ADMA) is an inhibitor of nitric oxide, which is the key element of endothelial dysfunction (ED).To the authors´knowledge, ED patterns and the risk of cardiovascular disease in these patients are largely unknown. Aim of the study: We assessed ED in CF children and young adults using the combination of plethysmographic Reactive Hyperemia Index (RHI) and specific biomarkers in comparison with the healthy controls (HC). Methods: A total of 22 CF subjects and 19 age-matched controls were enrolled. Mean age of CF patiens was 17.5 years. RHI, plasma concentrations of ADMA, high-sensitive CRP (hsCRP) and E-selectine of the patients were evaluated. Results: RHI values were significantly lower in CF subjects in comparison to HC: mean 1.375±0.5, mean 1.8±0.545, respectively; p Conclusions : Decreased RHI and high levels of serum hsCRP and E-selectine are related with ED in patients with CF. S erum ADMA level might not be related with ED in CF patients. These findings suggest the need for detection of ED and risk assessment of cardiovascular disease and preventive management in CF subjects.