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新生儿、儿童和青少年有症状的静脉和动脉血栓栓塞的风险因素 - 在过去 20 年里我们学到了什么?

Risk factors for symptomatic venous and arterial thromboembolism in newborns, children and adolescents - What did we learn within the last 20years?

机构信息

The Israel National Hemophilia Centre, Sheba Medical Centre, Tel-Hashomer, The Sackler Medical School, Tel Aviv University, Israel.

Thrombosis and Hemostasis Unit, Department of Clinical Chemistry, Univ. Hospital of Kiel, Germany.

出版信息

Blood Cells Mol Dis. 2017 Sep;67:18-22. doi: 10.1016/j.bcmd.2016.12.003. Epub 2016 Dec 14.

Abstract

Venous thrombosis (VTE) in children is increasingly diagnosed, as advanced medical care has increased treatment intensity of hospitalized pediatric patients. The aim of this review was to summarize the data available and to discuss the controversial issue of thrombophilia screening in the light of the pediatric data available. Follow-up data for VTE recurrence in children suggest a recurrence rate between 3% (neonates) and 21% in individuals with unprovoked VTE. Apart from underlying medical conditions, recently reported systematic reviews on pediatric VTE (70% provoked) have shown significant associations between thrombosis and presence of protein C-, protein S- and antithrombin deficiency, factor 5 (F5: rs6025), factor 2 (F2: rs1799963), even more pronounced when combined inherited thrombophilias [IT] were involved. The F2 mutation, protein C-, protein S-, and antithrombin deficiency did also play a significant role at VTE recurrence. Although we have learned more about the pathophysiology of VTE with the increased discovery of IT evidence is still lacking as to whether IT influence the clinical outcome in pediatric VTE. It still remains controversial as to whether children with VTE or offspring from thrombosis-prone families benefit from IT screening. Thus, IT testing in children should be individualized.

摘要

儿童静脉血栓栓塞症(VTE)的诊断率不断上升,这是由于先进的医疗护理提高了住院儿科患者的治疗强度。本综述的目的是总结现有数据,并根据现有儿科数据讨论血栓形成倾向筛查这一有争议的问题。儿童 VTE 复发的随访数据表明,无诱因 VTE 个体的复发率在 3%(新生儿)至 21%之间。除了基础疾病外,最近关于儿科 VTE(70%为诱因)的系统评价显示,血栓形成与蛋白 C-、蛋白 S-和抗凝血酶缺乏、因子 5(F5:rs6025)、因子 2(F2:rs1799963)之间存在显著关联,当涉及到遗传性血栓形成倾向(IT)时,这种关联更为明显。F2 突变、蛋白 C-、蛋白 S-和抗凝血酶缺乏在 VTE 复发中也起重要作用。尽管随着 IT 证据的不断发现,我们对 VTE 的病理生理学有了更多的了解,但 IT 是否影响儿科 VTE 的临床结局仍缺乏证据。是否应筛查 IT 以预防儿童 VTE 或血栓形成倾向家族的后代发生 VTE 仍存在争议。因此,儿童的 IT 检测应个体化。

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