Revel-Vilk Shoshana, Kenet Gili
Pediatric Hematology/Oncology Unit, Hadassah-Hebrew University Medical Center, POB 12000, Jerusalem il-91200, Israel.
Thromb Res. 2006;118(1):59-65. doi: 10.1016/j.thromres.2005.05.026. Epub 2005 Jul 1.
Venous thromboembolic events (VTEs) in children are usually associated with underlying clinical conditions such as central venous line, cancer and cardiac diseases. The objective of this review is to present the importance of thrombophilia to the occurrence of childhood VTE. The reported prevalence of thrombophilia in children with VTE varies extremely between 10% and 78% in different registries. The variation in the reported prevalence most probably reflects differences in the clinical characteristics of the children studied and differences in study designs. The initial management of children with thrombophilia and VTE is similar to those individuals who do not have a specific inherited thrombophilic risk factor, except in the rare events of homozygous deficiencies of prothrombotic coagulation proteins. The impact of thrombophilic markers on long-term therapy and outcome of children with VTE has not been completely clarified. According to the current guidelines for thrombophilia, all children with VTE should be tested for a full panel of genetic and acquired prothrombotic traits. However, re-evaluation of co-morbid risk factors other than thrombophilic markers and careful consideration of the prognostic value of thrombophilic markers might help to change future attitude from the rigidity of current guidelines to more rational schemes.
儿童静脉血栓栓塞事件（VTEs）通常与潜在的临床状况相关，如中心静脉置管、癌症和心脏疾病。本综述的目的是阐述易栓症对儿童VTE发生的重要性。不同登记处报告的VTE儿童中易栓症的患病率差异极大，在10%至78%之间。报告患病率的差异很可能反映了所研究儿童临床特征的差异以及研究设计的差异。除了罕见的促血栓形成凝血蛋白纯合缺陷情况外，易栓症和VTE儿童的初始治疗与没有特定遗传性易栓风险因素的个体相似。易栓症标志物对VTE儿童长期治疗和结局的影响尚未完全阐明。根据当前的易栓症指南，所有VTE儿童都应进行全套遗传和后天促血栓形成特征检测。然而，重新评估除易栓症标志物之外的共病风险因素，并仔细考虑易栓症标志物的预后价值，可能有助于改变未来的态度，从当前指南的僵化转变为更合理的方案。
