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急诊科用新鲜冷冻血浆有效治疗遗传性血管性水肿。

Effective treatment of hereditary angioedema with fresh frozen plasma in an emergency department.

作者信息

Pekdemir Murat, Ersel Murat, Aksay Ersin, Yanturali Sedat, Akturk Aysun, Kiyan Selahattin

机构信息

Department of Emergency Medicine, Kocaeli University Hospital, Izmit, Turkey.

出版信息

J Emerg Med. 2007 Aug;33(2):137-9. doi: 10.1016/j.jemermed.2007.02.024. Epub 2007 May 30.

Abstract

Hereditary angioedema (HAE) is a rarely seen disorder of C1 inhibitor (C1-INH) deficiency usually manifested by non-pruritic swelling of the skin. Acute exacerbations are not sensitive to conventional medications, and C1-esterase inhibitor concentrates are recommended as the first-line therapy. However, fresh frozen plasma is the main treatment alternative in many centers due to the lack of C1-esterase inhibitor concentrates. In this report, we present 3 patients with acute exacerbations of hereditary angioedema who were effectively and safely treated with fresh frozen plasma.

摘要

遗传性血管性水肿(HAE)是一种罕见的C1抑制物(C1-INH)缺乏症,通常表现为皮肤非瘙痒性肿胀。急性发作对传统药物不敏感,推荐使用C1酯酶抑制剂浓缩物作为一线治疗。然而,由于缺乏C1酯酶抑制剂浓缩物,在许多中心新鲜冷冻血浆是主要的替代治疗方法。在本报告中,我们介绍了3例遗传性血管性水肿急性发作患者,他们接受新鲜冷冻血浆治疗后有效且安全。

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