Service de Pédiatrie Générale, Hématologie et Rhumatologie Pédiatrique, Hôpital Bicêtre, 78 rue Général Leclerc, 94270 Le Kremlin Bicêtre, France.
J Rheumatol. 2011 Feb;38(2):387-90. doi: 10.3899/jrheum.100257. Epub 2010 Nov 15.
To describe the characteristics of a group of pediatric patients with Behçet's disease (BD) who presented at least 1 episode of thrombosis during their disease course.
We made a retrospective chart review of the clinical, biological, and radiological data of children with BD who presented at least 1 episode of either arterial or venous thrombosis. Data were extracted from both an international pediatric Behçet cohort and files referred from 7 French centers.
Twenty-one patients were included. Diagnosis of BD was based on the criteria of the International Study Group for BD. Main locations for thrombosis were the cerebral sinuses, in 11 patients (52.4%); and lower limbs, in 9 patients (40.9%). Recurrent episodes were observed in 4 patients (21%). Thrombophilia measurements were normal in 14 patients out of 21, while anticardiolipin antibodies were positive in 4 patients, and 2 out of 21 had protein C deficiency. One patient had lupus anticoagulant. All patients were treated with colchicine. Corticosteroids were also added for variable periods in 13 patients. Five patients out of 21 were treated with anticoagulants (heparin, then anti-vitamin K) and 3 with antiplatelets (acetylsalicylic acid).
Thromboses are a serious complication of BD and may occur early in the disease course. The presence of thrombophilic markers could increase the risk of thrombosis in BD, but the size of our population does not allow any conclusion. An international cohort (PED-BD) is currently in place and will allow study of such cases longitudinally, as well as assessment of the elements that correlate with an increased risk of thrombosis in children with BD.
描述一组在疾病过程中至少出现 1 次血栓形成的儿童贝赫切特病（BD）患者的特征。
我们对至少出现 1 次动脉或静脉血栓形成的 BD 患儿的临床、生物学和放射学数据进行了回顾性图表分析。数据来自一个国际儿科 BD 队列和来自 7 个法国中心的转介文件。
共纳入 21 例患者。BD 的诊断依据是国际 BD 研究组的标准。血栓的主要部位是 11 例患者（52.4%）的脑窦，9 例患者（40.9%）的下肢。4 例患者（21%）出现复发。21 例患者中，14 例血栓形成标志物正常，4 例抗心磷脂抗体阳性，2 例蛋白 C 缺乏。1 例患者存在狼疮抗凝物。所有患者均接受秋水仙碱治疗。13 例患者接受了不同时间的皮质类固醇治疗。21 例患者中有 5 例接受了抗凝治疗（肝素，然后是抗维生素 K），3 例接受了抗血小板治疗（乙酰水杨酸）。
血栓形成是 BD 的严重并发症，可能在疾病早期发生。血栓形成标志物的存在可能会增加 BD 患者发生血栓的风险，但我们的人群规模尚无法得出任何结论。目前正在建立一个国际队列（PED-BD），该队列将允许对这些病例进行纵向研究，以及评估与儿童 BD 患者血栓形成风险增加相关的因素。
