Prothrombotic disorders in children with moyamoya syndrome.

摘要: Background and Purpose — Moyamoya syndrome is an uncommon chronic occlusive cerebrovascular disease in children. The origin of moyamoya remains undetermined. role the prothrombotic disorders contributing to its pathogenesis has not been completely elucidated. purpose this study was determine frequency a pediatric population with syndrome. Methods From May 1992 April 2000, prospective 10 consecutive children carried out at single center. Evaluation included following assays: protein C, S, antithrombin, plasminogen, activated C resistance, factor V Leiden, prothrombin gene mutations. Lupus anticoagulant, anticardiolipin antibodies, anti–β2-glycoprotein I antibodies assays were also performed. clinical characteristics, underlying diseases, family history thrombosis, radiological findings, treatment, outcome recorded. Results In our series, detected 4 patients (40%). Inherited S deficiency found 1 patient; lupus anticoagulant remaining 3 patients. One presented persistent for 2.7 years until his death. case other 2 patients, maintained 9 months, whereas kept anticardiolipin/anti–β2-glycoprotein months. Conclusions We report hemostatic data largest center western hemisphere. (40%), disorder detected. It tempting speculate that these abnormalities may contribute some