Porebski Grzegorz, Bilo Barbara, Obtułowicz Krystyna, Obtułowicz Piotr, Nuijens Jan
Zaklad Alergologii Klinicznej i Srodowiskowej, Collegium Medicum Uniwersytetu Jagiellońskiego, Kraków.
Przegl Lek. 2005;62(5):317-20.
Hereditary angioedema (HAE) is a rare condition, resting on attacks of edema in various localizations, potentially life-threatening if in facial, laryngeal, pharyngeal or gastrointestinal area. The disease is caused by deficiency or impaired activity of C1 inhibitor, therefore C1 inhibitor infusion is the the essential treatment and the only efficient method in an acute attack of HAE. Nowadays C1 inhibitor applied in our patients is obtained from human plasma, what restricts the availability of the drug and carries relevant risks. After many years of research it came to the synthesis of the recombinant protein with features of human C1 inhibitor. Its first usage in Poland took place-in two HAE patients with severe edema in 2004. The course and efficiency of this treatment is reported in the paper. Recombinant human C1 inhibitor occurred efficient and safe in presented case of severe angioedema.
遗传性血管性水肿(HAE)是一种罕见疾病,表现为身体不同部位的水肿发作,如果发生在面部、喉部、咽部或胃肠道区域,可能会危及生命。该疾病是由C1抑制剂缺乏或活性受损引起的,因此C1抑制剂输注是HAE急性发作时的主要治疗方法和唯一有效手段。目前我们给患者使用的C1抑制剂是从人血浆中提取的,这限制了药物的可及性并带来相关风险。经过多年研究,已合成出具有人C1抑制剂特性的重组蛋白。2004年,其首次在波兰用于两名患有严重水肿的HAE患者。本文报道了该治疗方法的过程和疗效。在出现的严重血管性水肿病例中,重组人C1抑制剂显示出有效且安全。