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Behçet syndrome (BS) is a unique type of vasculitis that affects veins and arteries of all sizes, leading to recurrent vascular events, mostly venous thrombosis. The prevalence of venous thromboembolism in BS patients ranges between 15 and 40%. Thrombosis is usually an early manifestation leading to diagnosis of BS in up to 40% of patients. BS is per se a model of inflammation-induced thrombosis. The primary autoimmune response activates lymphocytes that in turn produce a cytokine cascade that activates neutrophils, which modify the secondary structure of fibrinogen making it less susceptible to plasmin-induced lysis. This leads to endothelial dysfunction, platelet activation and overexpression of tissue factor leading to inflammatory thrombi, usually attached to the wall. The pathogenesis of thrombosis is especially relevant to direct the specific treatment, that is based on immunosuppression rather than anticoagulation. Superficial vein thrombosis (SVT) and deep vein thrombosis (DVT) are the most common form of thrombosis in BS, but thrombosis in atypical sites (cava vein, suprahepatic veins, intracardiac thrombus) and arterial involvement can also occur. We assessed the latest update of the European League Against Rheumatism recommendations for the management of BS. Vascular Behçet treatment is usually based of immunosuppressants, and the role of anticoagulation remains controversial. The use of interventional and surgical procedures should be carefully evaluated, due to the risk of triggering a vascular pathergy phenomenon.
相似文献Sensory-Based Interventions (SBIs) are often recommended to enable function/participation in children with ASD. Still, there are limited studies to evaluate their effectiveness. Acceptability studies are an important step towards establishing effective interventions. We examined parents' perceptions of the uptake and acceptability of such interventions. An online survey was sent to 399 families; response rate was 39%. The most frequently therapist-recommended interventions were trampoline (54.6%), massage (47.8%), and oral-motor tools (43.8%). Highest use was reported for massage (96.3%), trampoline (89.2%) and joint compressions and brushing (89.2%). The majority of parents viewed these interventions to be very important /important, (74.8%) but barriers to their use were identified. High acceptability of SBIs was reported by parents of children with ASD.
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