Bonduel M, Sciuccati G, Hepner M, Torres A F, Pieroni G, Frontroth J P
Hematology-Oncology Department, Hospital de Pediatria "Prof. Dr. Juan P. Garrahan," Buenos Aires, Argentina.
Arch Neurol. 1999 Aug;56(8):967-71. doi: 10.1001/archneur.56.8.967.
Arterial ischemic stroke (AIS) and sinovenous thrombosis (SVT) are relatively rare events in children. The contribution of prethrombotic disorders to the etiology of these entities has not been completely elucidated.
To determine the frequency of inherited and acquired prethrombotic disorders in a pediatric population with AIS and SVT and to report clinical and radiological features.
From May 1992 to April 1997, 30 consecutive children with AIS and 10 children with SVT were assisted at a single institution. Hemostatic evaluation was performed for all the children. Evaluation included the following assays: protein C, protein S, antithrombin, plasminogen, activated protein C resistance, factor V Leiden mutation, and the detection of antiphospholipid antibodies. Data concerning baseline demographics, risk factors, presenting features, family history of thrombosis, and radiological findings were also recorded.
One or more prethrombotic disorders were present in 9 children (30%) with AIS (inherited protein S deficiency, 2 patients; inherited protein C deficiency, 1 patient; acquired antithrombin deficiency, 2 patients; antiphospholipid antibodies, 3 patients; and antiphospholipid antibodies and plaminogen deficiency, 1 patient) and in 5 children (50%) with SVT (inherited protein S deficiency, 1 patient; acquired antithrombin deficiency, 3 patients; and antiphospholipid antibodies, 1 patient).
Most children studied presented both a variety of risk factors for thrombosis and concomitant prethrombotic disorders. Therefore, a complete hemostatic evaluation for all children with AIS and SVT should be performed, despite the presence of obvious clinical risk factors or lack of family history of thrombosis.
动脉缺血性卒中（AIS）和静脉窦血栓形成（SVT）在儿童中相对少见。血栓前状态疾病对这些疾病病因的影响尚未完全阐明。
确定患有AIS和SVT的儿科人群中遗传性和获得性血栓前状态疾病的发生率，并报告临床和放射学特征。
1992年5月至1997年4月，在一家机构对30例连续的AIS患儿和10例SVT患儿进行诊治。对所有患儿进行止血评估。评估包括以下检测：蛋白C、蛋白S、抗凝血酶、纤溶酶原、活化蛋白C抵抗、凝血因子V莱顿突变以及抗磷脂抗体检测。还记录了有关基线人口统计学、危险因素、临床表现、血栓形成家族史和放射学检查结果的数据。
9例（30%）AIS患儿存在一种或多种血栓前状态疾病（遗传性蛋白S缺乏症2例；遗传性蛋白C缺乏症1例；获得性抗凝血酶缺乏症2例；抗磷脂抗体3例；抗磷脂抗体和纤溶酶原缺乏症1例），5例（50%）SVT患儿存在一种或多种血栓前状态疾病（遗传性蛋白S缺乏症1例；获得性抗凝血酶缺乏症3例；抗磷脂抗体1例）。
大多数研究的儿童既存在多种血栓形成危险因素，又伴有血栓前状态疾病。因此，对于所有AIS和SVT患儿，尽管存在明显的临床危险因素或缺乏血栓形成家族史，也应进行全面的止血评估。
