Tavil Betul, Ozyurek Emel, Gumruk Fatma, Cetin Mualla, Gurgey Aytemiz
Hacettepe University Faculty of Medicine, Pediatric Hematology Unit, 06100 Sihhiye, Ankara, Turkey.
Blood Coagul Fibrinolysis. 2007 Jun;18(4):347-52. doi: 10.1097/MBC.0b013e32809cc95a.
Antiphospholipid syndrome is a systemic disorder characterized by arterial and/or venous thrombosis, thrombocytopenia, recurrent fetal loss, and presence of antiphospholipid antibodies (APA). The importance of APA in Turkish children with thrombosis is unknown. This study aimed to evaluate the frequency of APA positivity, associated risk factors other than APA, and outcome in children with APA and thrombosis. The presence of APA was investigated in 138 children presenting for evaluation of thrombosis; other prothrombotic risk factors were also studied. The frequency of APA positivity among 138 children was 11.6% (16/138). The mean age of these 16 children (10 female, 62.5%) was 9.57 +/- 4.59 years (range, 2.5-18.0 years). The mean follow-up period was 31.7 +/- 21.7 months (range, 5-60 months). Recurrence was observed during follow-up in two patients (12.5%). Ten patients (62.5%) had arterial thrombosis, five patients (31.3%) venous thrombosis, and one patient (6.3%) purpura fulminans. Among the thrombotic children with APA, 11 (68.8%) had more than one prothrombotic risk factor other than circulating APA [five patients (31.3%) had two risk factors, two patients (12.5%) had three, and four patients (25.0%) had four]. Five patients (31.3%) had no additional risk factors. APA should be tested in all children with thrombosis, especially those with arterial thrombosis.
抗磷脂综合征是一种全身性疾病，其特征为动脉和/或静脉血栓形成、血小板减少、反复流产以及抗磷脂抗体（APA）阳性。APA在土耳其血栓形成儿童中的重要性尚不清楚。本研究旨在评估APA阳性的频率、除APA之外的相关危险因素以及患有APA和血栓形成的儿童的预后情况。对138名因血栓形成前来评估的儿童进行了APA检测；同时也研究了其他血栓形成危险因素。138名儿童中APA阳性的频率为11.6%（16/138）。这16名儿童（10名女性，占62.5%）的平均年龄为9.57±4.59岁（范围为2.5 - 18.0岁）。平均随访期为31.7±21.7个月（范围为5 - 60个月）。随访期间观察到2例患者（12.5%）复发。10例患者（62.5%）发生动脉血栓形成，5例患者（31.3%）发生静脉血栓形成，1例患者（6.3%）发生暴发性紫癜。在患有APA的血栓形成儿童中，11例（68.8%）除循环中的APA外还有一种以上血栓形成危险因素[5例患者（31.3%）有两种危险因素，2例患者（12.5%）有三种，4例患者（25.0%）有四种]。5例患者（31.3%）没有其他危险因素。所有血栓形成的儿童都应检测APA，尤其是那些患有动脉血栓形成的儿童。
