Aims Since December 2015, the European/International Fibromuscular Dysplasia (FMD) Registry enrolled 1022 patients from 22 countries. We present their characteristics according to disease subtype, age and gender, as well as predictors of widespread disease, aneurysms and dissections. Methods and results All patients diagnosed with FMD (string-of-beads or focal stenosis in at least one vascular bed) based on computed tomography angiography, magnetic resonance angiography, and/or catheter-based angiography were eligible. Patients were predominantly women (82%) and Caucasians (88%). Age at diagnosis was 46 ± 16 years (12% ≥65 years old), 86% were hypertensive, 72% had multifocal, and 57% multivessel FMD. Compared to patients with multifocal FMD, patients with focal FMD were younger, more often men, had less often multivessel FMD but more revascularizations. Compared to women with FMD, men were younger, had more often focal FMD and arterial dissections. Compared to younger patients with FMD, patients ≥65 years old had more often multifocal FMD, lower estimated glomerular filtration rate and more atherosclerotic lesions. Independent predictors of multivessel FMD were age at FMD diagnosis, stroke, multifocal subtype, presence of aneurysm or dissection, and family history of FMD. Predictors of aneurysms were multivessel and multifocal FMD. Predictors of dissections were age at FMD diagnosis, male gender, stroke, and multivessel FMD. Conclusions The European/International FMD Registry allowed large-scale characterization of distinct profiles of patients with FMD and, more importantly, identification of a unique set of independent predictors of widespread disease, aneurysms and dissections, paving the way for targeted screening, management, and follow-up of FMD.

Obstructive sleep apnoea (OSA) is considered as a risk factor for the development of arterial hypertension, coronary artery disease (CAD), myocardial infarction and stroke. These clinical manifestations are the consequences of elevated sympathetic activity, cardiovascular variability, intrathoracic pressure changes, inflammation, oxidative stress, endothelial dysfunction, insulin resistance and thrombosis provoked by OSA. As a result, OSA is often present in patients with cardiovascular disease (CVD) and the increased prevalence of CVD in OSA population raises both cardiovascular morbidity and mortality and the demand of healthcare resources. Observational cohort studies indicate that untreated patients with OSA have an increased risk of fatal and non-fatal cardiovascular events, an increased risk of sudden cardiac death during the sleeping hours and a higher risk of stroke or death from any cause. Continuous positive airway pressure (CPAP) and oral appliance therapy are the two treatments for OSA whose effects on cardiovascular endpoints have been assessed in randomised trials. There is increasing evidence that adequate CPAP therapy leads to a significant reduction in cardiovascular morbidity.

Diabetes mellitus is associated with endothelial dysfunction and oxidative stress (OS). We investigated whether these abnormalities are interrelated in children and adolescents with type 1 diabetes mellitus (T1DM) and if early OS markers predictive of vascular dysfunction can be identified. Thirty-five T1DM patients were matched for sex, age, height, and weight with nondiabetic subjects as healthy controls (CO). Flow-mediated dilatation (FMD), carotid intima media thickness (IMT), and OS status in fasting blood were measured. Diabetic children had impaired FMD (6.68 Ϯ 1.98 versus 7.92 Ϯ 1.60% in CO, p ϭ 0.004), which was more pronounced in boys. The degree of FMD impairment was not related to the lower plasma levels of antioxidants or to the higher glucose, glycation, lipids, and peroxidation products. Erythrocyte superoxide dismutase activity, copper/zinc superoxide dismutase (Cu/Zn SOD), was higher in diabetic subjects (1008 Ϯ 224 versus 845 Ϯ 195 U/g Hb in CO, p ϭ 0.003) and was positively associated with FMD. After correcting for diabetes and gender, the subgroup of children with high Cu/Zn SOD (Ͼ955 U/g Hb) had a significantly better FMD (p ϭ 0.035). These results suggest that higher circulating Cu/Zn SOD could protect T1DM children and adolescents against endothelial dysfunction. Low Cu/Zn SOD is a potential early marker of susceptibility to diabetic vascular disease. (Pediatr Res 62: 456-461, 2007) D iabetes mellitus is an important risk factor for atherosclerosis and both the incidence and mortality of cardiovascular disease are increased in diabetic patients (1). Among the various pathophysiological mechanisms mediating the atherosclerotic process, both oxidative stress (OS) and endothelial dysfunction occur at an early stage in animal models of diabetes (2).Oxidative stress is defined as the change in the pro-oxidant/ antioxidant balance in favor of the former, potentially leading to biologic damage to macromolecules and cell dysfunction (3). As a result of hyperglycemia, excessive pro-oxidants (free radicals and reactive oxygen species) are formed via autooxidation of glucose, nonenzymatic glycation and formation of advanced glycation end products, increased flux through the polyol and hexosamine pathways, and activation of protein kinase C. These processes also lead to decreased antioxidant defenses. Brownlee (4) has linked all these abnormalities to the excessive production of superoxide by the mitochondria.In children with type 1 diabetes mellitus (T1DM), increased OS has been reported to be present even shortly after diagnosis (5). Other reports showed the parallelism between OS and abnormal markers of endothelial cell function (such as ESelectin and ICAM-1) in young T1DM patients, suggesting a link between these two abnormalities (6). Ultrasound testing of skin microcirculation and of brachial artery flow-mediated dilatation (FMD) have demonstrated early endothelial dysfunction in diabetic children and adolescents (7).Since it has been shown that foam cell accumulation in the vascular w...

It has been shown that in cardiovascular risk management, stiffness of large arteries has a very good predictive value for cardiovascular disease and mortality. This parameter is best known when estimated from the pulse wave velocity (PWV) measured between the common carotid artery (CCA) in the neck and femoral artery in the groin, but may also be determined locally from short-distance measurements on a short vessel segment. In this work, we propose a novel, non-invasive, non-contact laser Doppler vibrometry (LDV) technique for evaluating PWV locally in an elastic vessel. First, the method was evaluated in a phantom setup using LDV and a reference method. Values correlated significantly between methods (R 0.973 (p 0.01)); and a Bland-Altman analysis indicated that the mean bias was reasonably small (mean bias −2.33 ms). Additionally, PWV was measured locally on the skin surface of the CCA in 14 young healthy volunteers. As a preliminary validation, PWV measured on two locations along the same artery was compared. Local PWV was found to be between 3 and 20 m s −1 , which is in line with the literature (PWV = 5-13 m s −1). PWV assessed on two different locations on the same artery correlated significantly (R = 0.684 (p < 0.01)). In summary, we conclude that this new non-contact method is a promising technique to measure local vascular stiffness in a fully non-invasive way, providing new opportunities for clinical diagnosing.