Impact of high-risk thrombophilia status on recurrence among children with a first non-central-venous-catheter-associated VTE: an observational multicentre cohort study
Impact of high-risk thrombophilia status on recurrence among children with a first non-central-venous-catheter-associated VTE: an observational multicentre cohort study
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DOI：
10.1111/bjh.14192
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发表时间：
2016-10-01
影响因子：
6.5
通讯作者：
Nowak-Goettl, Ulrike
中科院分区：
文献类型：
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作者：
Limperger, Verena;Kenet, Gili;Nowak-Goettl, Ulrike
抗凝血酶（AT）、蛋白C（PC）或蛋白S（PS）缺乏是静脉血栓栓塞（VTE）的主要危险因素。需要确定从筛查中获益的复发高风险个体。主要研究目的是确定首次发生非中心静脉导管相关性VTE的儿童的个体复发风险（与其血栓形成倾向状态有关），并评价AT、PC或PS缺陷与无血栓形成倾向的儿童首次发生VTE时的临床表现是否不同。我们计算了161例连续入选的儿童VTE患者的VTE复发绝对风险和经血栓形成倾向、年龄、性别和阳性家族VTE史校正后的无事件生存率。相对于无血栓形成倾向的缺陷的存在被评价为复发的潜在预测因子。复发的预测因素为AT缺乏（风险比/95% CI：65/246-172）和女性（26/11-635）。AT缺陷儿童的年复发率（95% CI）为54%（26-10），PC缺陷患者为13%（03-38），PS缺陷队列为07%（008-24），无血栓形成倾向患者为09%（04-18）。阳性家族VTE史或合并血栓形成倾向不能预测复发。考虑到总的年复发率为15%，我们建议对VTE儿童进行AT缺乏筛查。
Deficiency of antithrombin (AT), protein C (PC) or protein S (PS) constitutes a major risk factor for venous thromboembolism (VTE). Individuals at high risk for recurrence who benefit from screening need to be identified. The primary study objective was to determine the individual recurrence risk among children with a first non-central-venous-catheter-associated VTE with respect to their thrombophilia status and to evaluate if the clinical presentation at first VTE onset differs between children with AT, PC or PS deficiency versus no thrombophilia. We calculated the absolute risk of VTE recurrence and event-free-survival adjusted for thrombophilia, age, sex and positive family VTE history in 161 consecutively enrolled paediatric VTE patients. The presence of a deficiency relative to no thrombophilia was evaluated as a potential predictor of recurrence. Predictors for recurrence were AT deficiency (hazard ratio/95% CI: 65/246-172) and female gender (26/11-635). The annual recurrence rates (95% CIs) were 54% (26-10) in AT-deficient children, 13% (03-38) in patients with PC deficiency, 07% (008-24) in the PS-deficient cohort and 09% (04-18) in patients with no thrombophilia. Positive family VTE history or combined thrombophilias did not predict recurrence. Given the overall annual incidence rate of recurrence of 15% we suggest screening for AT deficiency in children with VTE.