Perioperative use of modified thrombelastography in factor XI deficiency:: a helpful method to assess drug effects
Perioperative use of modified thrombelastography in factor XI deficiency:: a helpful method to assess drug effects
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DOI：
10.1111/j.1399-6576.2007.01284.x
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发表时间：
2007-05-01
影响因子：
2.1
通讯作者：
Peters, J.
中科院分区：
文献类型：
--
作者：
Dirkmann, D.;Hanke, A. A.;Peters, J.
因子XI缺乏症是一种罕见的遗传性出血性疾病，与创伤相关的出血倾向有关，由凝血酶活化纤维蛋白溶解抑制剂（TAFI）产生不足引起纤维蛋白溶解增加引起。我们提出的情况下，一个五岁的女孩纯合子，严重的因子XI缺乏提出手术两次。改良的血栓弹力图（ROTEM（TM））用于评估凝血因子XI缺乏对凝血、内源性纤维蛋白溶解的影响，以及氨甲环酸、抑肽酶和重组活化因子VII在体外纤维蛋白溶解亢进模型中的潜在影响。根据我们的数据，并考虑到因子XI缺乏的机制，我们决定预防性使用氨甲环酸。
Factor XI deficiency is a rare, hereditary bleeding disorder associated with a trauma-related bleeding tendency, caused by insufficient generation of the thrombin activatable fibrinolysis inhibitor (TAFI) evoking increased fibrinolysis. We present the case of a five year old girl with homozygote, severe factor XI deficiency presenting for surgery on two occasions. Modified thrombelastography (ROTEM (TM)) was used to assess effects of factor XI deficiency on coagulation, endogenous fibrinolysis, and potential effects of tranexamic acid, aprotinin and recombinant, activated Factor VII in an in vitro model of hyperfibrinolysis. According to our data and in consideration of the mechanisms of factor XI deficiency we decided on prophylactic use of tranexamic acid.