Egidio Fabiani J, Squiquera L, Leoni J, Simkin G, Casala A
Instituto Argentino de Alergia e Inmunología.
Med Cutan Ibero Lat Am. 1987;15(6):461-7.
12 patients (7 males and 5 females) suffering from the common form of HAE were included in the study protocol. All patients were older than 18 years. They were evaluated Cl INH, C4, Clq, Cls, C3, C5, C8, Bf, CH 50% and CIC. For the purpose of the study they were only considered Cl INH, C4, CH 50%, CIs and CIC levels. The rest of the complement components were among normal values. Data were recorded at day 0 and after 10 days on treatment with 400 mg/day of Danazol. Results were as follows: 50% of the patients had CIC when CH 50% values were below 120 U/ml., after treatment CIC disappeared and CH 50, Cls, C4 and Cl INH increased significatively. C4 seemed to increase more and quicker than Cl INH in terms of absolute values. We postulate that in the group of patients with CIC, the primary cause of the disease may be an alteration in C4 synthesis and that Cl INH may be lowered because of its consumption. We postulate that Danazol could act in these cases stimulating C4 synthesis independently or in accordance with Cl INH.
12名患有常见类型遗传性血管性水肿(HAE)的患者(7名男性和5名女性)被纳入研究方案。所有患者年龄均超过18岁。对他们进行了C1酯酶抑制物(Cl INH)、C4、C1q、C1s、C3、C5、C8、B因子(Bf)、50%补体溶血活性(CH 50%)和循环免疫复合物(CIC)的评估。出于研究目的,仅考虑Cl INH、C4、CH 50%、C1s和CIC水平。其余补体成分均在正常范围内。在第0天以及使用400毫克/天达那唑治疗10天后记录数据。结果如下:当CH 50%值低于120 U/ml时,50%的患者存在CIC,治疗后CIC消失,CH 50、C1s、C4和Cl INH显著升高。就绝对值而言,C4似乎比Cl INH升高得更多、更快。我们推测,在存在CIC的患者组中,疾病的主要原因可能是C4合成改变,而Cl INH可能因消耗而降低。我们推测达那唑在这些病例中可能通过独立作用或与Cl INH协同作用来刺激C4合成。