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严重因子 XI 缺乏症患者血浆置换后的因子 XI 动力学

Factor XI kinetics after plasma exchange in severe factor XI deficiency.

作者信息

Nováková I R, van Ginneken C A, Verbruggen H W, Haanen C

出版信息

Haemostasis. 1986;16(1):51-6. doi: 10.1159/000215269.

Abstract

Plasma exchange in a patient with factor XI deficiency allowed the determination of the factor XI disappearance time. The decay curve showed a biphasic pattern with a t1/2 of h. After a loading dose by means of plasma exchange with fresh frozen plasma (FFP) up to a factor XI level of 65%, administration of 0.5 liter FFP every 12 h was necessary to maintain the factor XI concentration between 40 and 60%. The clearances of factor XI, calculated from the results of plasma exchange and from the maintenance dose, correlated very well and showed that the t1/2 of factor XI did not change after surgery. The patient underwent major surgery uneventfully but ultimately died 3 1/2 weeks after operation from cerebral damage due to cardiac arrest, which occurred on the 2nd postoperative day.

摘要

对一名患有因子 XI 缺乏症的患者进行血浆置换,从而得以确定因子 XI 的消失时间。衰变曲线呈现双相模式,半衰期为 h。通过用新鲜冰冻血浆(FFP)进行血浆置换给予负荷剂量直至因子 XI 水平达到 65% 后,每 12 小时给予 0.5 升 FFP 以将因子 XI 浓度维持在 40% 至 60% 之间。根据血浆置换结果和维持剂量计算得出的因子 XI 清除率相关性非常好,且表明因子 XI 的半衰期在手术后未发生变化。该患者顺利接受了大手术,但最终在术后第 2 天发生心脏骤停,术后 3 周半因脑损伤死亡。

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