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持续输注重组因子VIIa对有抑制物的血友病患者的疗效。

Efficacy of recombinant factor VIIa administered by continuous infusion to haemophilia patients with inhibitors.

作者信息

Mauser-Bunschoten E P, Koopman M M W, Goede-Bolder A D E, Leebeek F W G, van der Meer J, van Marwijk Kooij G M, van der Linden P W G

机构信息

Van Creveldkliniek, University Medical Centre Utrecht, The Netherlands.

出版信息

Haemophilia. 2002 Sep;8(5):649-56. doi: 10.1046/j.1365-2516.2002.00667.x.

Abstract

We have prospectively monitored treatment of haemophilia patients with inhibitors by recombinant factor VIIa (rFVIIa) administered by continuous infusion to obtain more insight in the underlying factors of the clinical efficacy of this administration method. At present, 43 treatment episodes of 14 different Dutch haemophilia inhibitor patients are included in the database. Analysis of the data showed a discrepancy between the efficacy of rFVIIa continuous infusion treatment of acute and surgical bleeds in the oral cavity [one (14%) effective, two (29%) partially effective, four (57%) not effective] and other parts of the body [29 (80%) effective, four (11%) partially effective, two (6%) not effective, one (3%) impossible to classify]. Patients who had acute or surgical oral cavity bleeds, uncontrolled by rFVIIa continuous infusion, reacted favourably to rFVIIa continuous infusion in other locations of the body. Acute bleeding episodes in the oral cavity, which could not be controlled by rFVIIa continuous infusion, stopped when the treatment regimen was switched to rFVIIa bolus injections. Finally, haemostatic control during dental extractions was excellent after the initial rFVIIa bolus injection preceding the continuous infusion, but rebleeds occurred in all patients within 48 h under rFVIIa continuous infusion coverage. These observations suggest that the efficacy of rFVIIa continuous infusion depends, at least in part, on the location of the body in which the bleeding occurs and that rFVIIa bolus injections are more effective than rFVIIa continuous infusion in the oral cavity. We hypothesize that the inability of rFVIIa continuous infusion treatment to sufficiently inhibit fibrinolysis is the underlying cause of the decreased efficacy of rFVIIa continuous infusion treatment in the oral cavity.

摘要

我们前瞻性地监测了通过持续输注重组凝血因子VIIa(rFVIIa)治疗血友病抑制物患者的情况,以更深入了解这种给药方法临床疗效的潜在因素。目前,数据库中纳入了14名不同的荷兰血友病抑制物患者的43次治疗疗程。数据分析显示,rFVIIa持续输注治疗口腔急性出血和手术出血的疗效[1例(14%)有效,2例(29%)部分有效,4例(57%)无效]与身体其他部位的疗效[29例(80%)有效,4例(11%)部分有效,2例(6%)无效,1例(3%)无法分类]存在差异。口腔急性或手术出血且未被rFVIIa持续输注控制的患者,在身体其他部位对rFVIIa持续输注反应良好。口腔内无法被rFVIIa持续输注控制的急性出血发作,在治疗方案改为rFVIIa推注后停止。最后,在持续输注前进行初始rFVIIa推注后,拔牙期间的止血控制良好,但在rFVIIa持续输注覆盖下,所有患者在48小时内均出现再出血。这些观察结果表明,rFVIIa持续输注的疗效至少部分取决于出血发生的身体部位,并且rFVIIa推注在口腔内比rFVIIa持续输注更有效。我们推测,rFVIIa持续输注治疗无法充分抑制纤维蛋白溶解是其在口腔内疗效降低的根本原因。

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