Barnes Chris, Deveber Gabrielle
Department of Haematology, Royal Children's Hospital, Melbourne, Australia.
Thromb Res. 2006;118(1):67-74. doi: 10.1016/j.thromres.2005.05.021. Epub 2005 Jul 21.
Childhood ischaemic stroke, incorporating arterial ischaemic stroke and cerebral sinus venous thrombosis, is associated with significant morbidity and mortality in children. The majority of cases in children present with well-recognised risk factors. The appreciation of the role prothrombotic abnormalities have in disease states is developing rapidly. Prothrombotic abnormalities are abnormalities of the coagulation system, fibrinolytic system, endothelial cells or platelets that lead to a reduced threshold for pathological thrombus formation. Our understanding of the role of prothrombotic abnormalities in childhood ischaemic stroke is increasing and has a direct bearing on the development of effective management and prevention strategies. We provide a brief background of prothrombotic abnormalities and review the available literature on prothrombotic markers in childhood ischaemic stroke. Overall, prothrombotic abnormalities have been identified in 20-50% of children presenting with AIS and 33-99% of children with cerebral sinus venous thrombosis. There appear to be a number of associations emerging including an increased frequency of factor V Leiden mutation, elevated lipoprotein (a), protein C deficiency and antiphospholipid antibodies in children presenting with arterial ischaemic stroke. The pathogenic role of prothrombotic abnormalities as predisposing to initial and recurrent childhood ischaemic stroke is becoming increasingly evident. The impact on treatment, however, will only be clarified with carefully designed, multi-institutional prospective studies.
儿童缺血性卒中,包括动脉缺血性卒中和脑静脉窦血栓形成,与儿童的显著发病率和死亡率相关。大多数儿童病例存在公认的危险因素。对血栓前异常在疾病状态中所起作用的认识正在迅速发展。血栓前异常是指凝血系统、纤维蛋白溶解系统、内皮细胞或血小板的异常,这些异常会导致病理性血栓形成的阈值降低。我们对血栓前异常在儿童缺血性卒中中作用的理解正在增加,这对有效管理和预防策略的制定有直接影响。我们提供血栓前异常的简要背景,并回顾关于儿童缺血性卒中血栓前标志物的现有文献。总体而言,在20%至50%的动脉缺血性卒中儿童和33%至99%的脑静脉窦血栓形成儿童中已发现血栓前异常。似乎出现了一些关联,包括在动脉缺血性卒中儿童中因子V莱顿突变频率增加、脂蛋白(a)升高、蛋白C缺乏和抗磷脂抗体。血栓前异常作为儿童初始和复发性缺血性卒中的易感因素的致病作用越来越明显。然而,只有通过精心设计的多机构前瞻性研究才能阐明其对治疗的影响。