Pekdemir Murat, Ersel Murat, Aksay Ersin, Yanturali Sedat, Akturk Aysun, Kiyan Selahattin
Department of Emergency Medicine, Kocaeli University Hospital, Izmit, Turkey.
J Emerg Med. 2007 Aug;33(2):137-9. doi: 10.1016/j.jemermed.2007.02.024. Epub 2007 May 30.
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.
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。