Age-Related Progression of Microvascular Dysfunction in Cystic Fibrosis: New Detection Ways and Clinical Outcomes

Abstract

There are concerns about altered vascular functions that could play an important role in the pathogenesis and influence theseverity of chronic disease, however, increased cardiovascularrisk in paediatric cystic fibrosis (CF) has not been yet fullyunderstood. Aim was to analyze vascular disease risk andinvestigate changes over times in CF and controls. Weprospectively enrolled 22 CF subjects (a median age of16.07 years), and 22 healthy demographically matched controls(a median age of 17.28 years) and determined endothelialfunction. We utilized a combined diagnostic approach bymeasuring the plethysmographic Reactive Hyperemia Index (RHI)as the post-to preocclusive endothelium-dependent changes ofvascular tone, and biomarkers that are known to be related toendothelial dysfunction (ED): asymmetric dimethyl arginine(ADMA), high-sensitive CRP (hsCRP), VCAM-1 and E-selectin.RHI values were significantly lower in CF young adults(p<0.005). HsCRP (p<0.005), E-selectin (p<0.001) and VCAM-1(p<0.001) were significantly increased in CF patients sincechildhood. The findings have provided a detailed account of theongoing process of microvascular dysfunction with gradualprogression with the age of CF patients, making them further atrisk of advanced vascular disease. Elevations of biomarkers inCF children with not yet demonstrated RHI changes but withsignificantly reduced RHI in adulthood and lipid profile changesindicate the possible occurrence of ED with CF-related specificrisk factors over time and will enable us to provide the bestpossible support