Farkas Henriette, Jakab László, Temesszentandrási György, Visy Beáta, Harmat György, Füst George, Széplaki Gábor, Fekete Béla, Karádi István, Varga Lilian
Semmelweis University, Faculty of Medicine, 3rd Department of Internal Medicine, Budapest, Hungary.
J Allergy Clin Immunol. 2007 Oct;120(4):941-7. doi: 10.1016/j.jaci.2007.06.026. Epub 2007 Aug 29.
C1-inhibitor (C1-INH) is a serine protease inhibitor regulating the complement, kinin-kallikrein, coagulation, and fibrinolytic systems. Hereditary angioedema (HAE) is caused by an inherited deficiency of C1-INH characterized by sudden, recurrent edematous swellings of the subcutaneous or submucosal tissues. The optional therapy for the acute management of HAE is administration of human C1-INH (hC1-INH) concentrate. However, hC1-INH is not available in many countries, in which case fresh frozen plasma is an alternative.
To summarize our experience with hC1-INH concentrate in patients with HAE.
Clinical and laboratory information on the effectiveness and safety of hC1-INH administered to relieve 468 acute edematous attacks in 61 patients with HAE was analyzed.
Severe abdominal or subcutaneous attacks and laryngeal edema were consistently relieved by the administration of 500 U hC1-INH concentrate. Symptoms improved within 15 to 60 minutes of administration. Progression of the attacks was never observed, and there were no recurrent attacks within 72 hours. hC1-INH concentrate requirements did not change after repeated use. hC1-INH concentrate proved effective in the management of 94 attacks in 22 children and 6 attacks in 4 pregnant women. Adverse reactions, viral infections, and antibody formation against the purified protein did not occur.
The administration of hC1-INH concentrate in HAE is highly effective and safe for the treatment of acute attacks and short-term prophylaxis and in pediatric patients and pregnant women.
Human C1-INH concentrate is effective and safe for the treatment of acute HAE attacks as well as for short-term prophylaxis.
C1抑制剂（C1-INH）是一种丝氨酸蛋白酶抑制剂，可调节补体、激肽-激肽释放酶、凝血和纤溶系统。遗传性血管性水肿（HAE）由C1-INH遗传性缺乏引起，其特征为皮下或黏膜下组织突然反复出现水肿性肿胀。HAE急性发作的首选治疗方法是给予人C1-INH（hC1-INH）浓缩物。然而，许多国家无法获得hC1-INH，在这种情况下，新鲜冷冻血浆是一种替代选择。
总结我们使用hC1-INH浓缩物治疗HAE患者的经验。
分析了61例HAE患者使用hC1-INH缓解468次急性水肿发作的有效性和安全性的临床及实验室信息。
给予500 U hC1-INH浓缩物可持续缓解严重的腹部或皮下发作以及喉头水肿。症状在给药后15至60分钟内改善。未观察到发作进展，且72小时内无复发发作。重复使用后hC1-INH浓缩物的需求量未发生变化。hC1-INH浓缩物在治疗22例儿童的94次发作和4例孕妇的6次发作中被证明有效。未发生不良反应、病毒感染以及针对纯化蛋白的抗体形成。
在HAE患者中给予hC1-INH浓缩物治疗急性发作、短期预防以及儿科患者和孕妇时高效且安全。
人C1-INH浓缩物治疗急性HAE发作以及短期预防有效且安全。
