Heller C, Becker S, Scharrer I, Kreuz W
Department of Paediatrics, Johann Wolfgang Goethe University, Frankfurt am Main, Germany.
Eur J Pediatr. 1999 Dec;158 Suppl 3:S117-21. doi: 10.1007/pl00014333.
Many studies have shown a high percentage of venous thromboses in children to be associated with haematological disorders. However, studies assessing the influence of haemostaseological disorders on paediatric stroke are rare. We compared 26 children with cerebral infarction (median age 2 months, range 0-16.2 years) and 17 with venous thrombosis (median age 4.5 years, range 0-17 years) with regard to prothrombotic risk factors. Prothrombotic disorders were found in 8 out of 26 patients with cerebral infarction (FV Leiden mutation: n = 4; protein C deficiency: n = 1; FV Leiden mutation + protein C deficiency: n = 2; prothrombin mutation G20210A: n = 1) and in 13 out of 17 with venous thrombosis (FV Leiden mutation n = 3; protein C deficiency n = 5; elevated HRGP + PAI: n = 1, combined deficiency of AT, protein C and plasminogen: n = 1; F XII deficiency: n = 1; lupus anticoagulans n = 1; FV Leiden + F XII deficiency + lupus anticoagulans + PAI: n = 1). Comparison of these prevalences with those of 150 healthy paediatric controls showed in children with FV Leiden mutation and/or protein C deficiency an increased risk of cerebral infarction (patients vs. controls: 26.9% vs. 6%; OR 5.77; 95%-CI 1.92-17.3; P = 0.0031) as well as of venous thrombosis (53% vs. 5.3% 19.9; 95%-CI 6-65.6; P < 0.0001). This result is in contrast with reports on thrombophilia in cerebral infarction in adult patients.
Our results indicate that FV Leiden mutation and protein C deficiency may contribute to the multifactorial aetiology of stroke in early childhood.
许多研究表明,儿童静脉血栓形成的很大一部分与血液系统疾病有关。然而,评估止血系统疾病对儿童中风影响的研究很少。我们比较了26例脑梗死患儿(中位年龄2个月,范围0 - 16.2岁)和17例静脉血栓形成患儿(中位年龄4.5岁,范围0 - 17岁)的血栓形成前危险因素。在26例脑梗死患者中有8例发现血栓形成前疾病(FV Leiden突变:n = 4;蛋白C缺乏:n = 1;FV Leiden突变 + 蛋白C缺乏:n = 2;凝血酶原突变G20210A:n = 1),在17例静脉血栓形成患者中有13例(FV Leiden突变n = 3;蛋白C缺乏n = 5;HRGP和PAI升高:n = 1,抗凝血酶、蛋白C和纤溶酶原联合缺乏:n = 1;F XII缺乏:n = 1;狼疮抗凝物n = 1;FV Leiden + F XII缺乏 + 狼疮抗凝物 + PAI:n = 1)。将这些患病率与150名健康儿童对照的患病率进行比较,结果显示,携带FV Leiden突变和/或蛋白C缺乏的儿童发生脑梗死的风险增加(患者与对照:26.9%对6%;OR 5.77;95%置信区间1.92 - 17.3;P = 0.0031),发生静脉血栓形成的风险也增加(53%对5.3%;OR 19.9;95%置信区间6 - 65.6;P < 0.0001)。这一结果与关于成年患者脑梗死中血栓形成倾向的报道相反。
我们的结果表明,FV Leiden突变和蛋白C缺乏可能促成幼儿期中风的多因素病因。