Airway obstruction and gender affect arterial stiffness in children with cystic fibrosis.

BACKGROUND

Vascular changes are observed in children with cystic fibrosis (cwCF), and gender-specific differences may impact arterial stiffness. We aimed to compare arterial stiffness and clinical parameters based on gender in cwCF and to determine the factors affecting arterial stiffness in cwCF.

METHODS

Fifty-eight cwCF were included. Pulmonary function, lean body mass, handgrip strength, and peak oxygen uptake (VO2peak) were assessed using a cardiopulmonary exercise test. Arterial stiffness (pulse wave velocity [PWV] and augmentation index [AIx@75]) and hemodynamic parameters (resting heart rate [HR] and stroke volume [SV]) were measured using brachial pulse waves. Endothelial function (ICAM-1, sVCAM-1, sE-selectin, VEGF-A, ET-1) was evaluated using blood samples.

RESULTS

Female cwCF had significantly lower VO2peak, SV, and PWV, and higher resting HR, AIx@75, and AIx@75-z-score than male cwCF (p<0.05). AIx@75-z-score was associated with gender (r=0.516, p<0.001), age (r= -0.345, p=0.008), lean body mass (r= -0.451, p<0.001), forced expiratory volume in one second (FEV1)-z-score (r= -0.332, p=0.011), handgrip strength (r= -0.466, p<0.001), and VO2peak (r= -0.459, p<0.001) and peak workload (r= -0.527, p<0.001). AIx@75-z-score was not associated with ICAM-1, sVCAM-1, sE-selectin, VEGF-A, or ET-1 (p>0.05). The FEV1-z-score and gender explained 34.6% of the variance in AIx@75-z-score (p<0.05).

CONCLUSIONS

Female cwCF have more impaired hemodynamics, less maximal exercise capacity, and increased arterial stiffness, indicating a higher cardiovascular risk compared to male cwCF. FEV1 and gender affect arterial stiffness in cwCF. Further studies are necessary to uncover the underlying factors for arterial stiffness and endothelial dysfunction and their clinical effects in cwCF.