Ebeling Freja, Petäjä Jari, Alanko Soile, Hirvasniemi Aune, Holm Tarja, Lähde Marja, Nuutila Auli, Pesonen Helena, Vahtera Elina, Rasi Vesa
Department of Haemostasis, Finnish Red Cross Blood Transfusion Service, Helsinki, Finland.
Eur J Pediatr. 2003 Oct;162(10):678-81. doi: 10.1007/s00431-003-1285-9. Epub 2003 Aug 12.
The binding of autoimmune anticardiolipin antibodies to phospholipid has been shown to require a plasma cofactor, beta-2-glycoprotein 1 (beta2-GPI). Antibodies against beta2-GPI are associated with both venous and arterial thrombosis in adults and have been suggested as a new antiphospholipid syndrome marker. We present six children with cerebral thrombosis at the age of 0-12 months, who had IgG antibodies to beta2-GPI (titers 26, 29, 31, 39, 101 and 109 SGU, when 20 SGU was the cut-off). In at least four patients, the conventional antiphospholipid markers (lupus anticoagulant and IgG and IgM anticardiolipin) were negative. All six children had normal results in the other routine thrombophilia assays (thrombin time, antithrombin, protein C, protein S, factor V Leiden mutation, prothrombin mutation).
the anti-beta-2 glycoprotein 1 assay, requiring only 5 microl serum, may be a useful addition to antiphospholipid-antibody diagnostics in cases of paediatric stroke.
自身免疫性抗心磷脂抗体与磷脂的结合已被证明需要一种血浆辅因子，即β2糖蛋白1（β2-GPI）。抗β2-GPI抗体与成人的静脉和动脉血栓形成有关，并被认为是一种新的抗磷脂综合征标志物。我们报告了6名年龄在0至12个月的患有脑血栓形成的儿童，他们具有抗β2-GPI的IgG抗体（滴度分别为26、29、31、39、101和109 SGU，截断值为20 SGU）。至少4名患者的传统抗磷脂标志物（狼疮抗凝物以及IgG和IgM抗心磷脂）为阴性。所有6名儿童的其他常规血栓形成倾向检测（凝血酶时间、抗凝血酶、蛋白C、蛋白S、因子V Leiden突变、凝血酶原突变）结果均正常。
仅需5微升血清的抗β2糖蛋白1检测，对于小儿中风病例的抗磷脂抗体诊断可能是一项有用的补充。
