Laurent J, Intrator L, Branellec A, Sobel A, Rahbar K, Lagrue G
Presse Med. 1987 May 2;16(16):762-4.
Although anabolic androgens can be used to prevent acute attacks of hereditary angioneurotic oedema, these are still observed. When they are severe, and even more so when they involve the larynx or the abdominal viscera, an emergency treatment is necessary, ideally with the purified C1-inhibitor (C1-INH) administered by intravenous infusion. In a case with pseudo-surgical abdominal symptoms, this treatment was followed by an unusually dramatic regression of all symptoms. Serial laboratory examinations showed an increase in C1-INH and C2 plasma levels and in the number of basophils within less than 30 minutes; the increase in C4 levels occurred later. Thus, C1-INH is very effective in the treatment of acute hereditary angioneurotic oedema, but the product is not widely available; it should be reserved to severe forms affecting the larynx and the viscera.
尽管合成代谢雄激素可用于预防遗传性血管性水肿的急性发作，但仍会出现发作。当发作严重时，尤其是累及喉部或腹腔脏器时，就需要进行紧急治疗，理想的治疗方法是静脉输注纯化的C1抑制物（C1-INH）。在一例有类似外科急腹症症状的病例中，采用这种治疗后所有症状出现了异常显著的消退。系列实验室检查显示，在不到30分钟内C1-INH和C2血浆水平以及嗜碱性粒细胞数量增加；C4水平的升高出现得较晚。因此，C1-INH在治疗急性遗传性血管性水肿方面非常有效，但该产品供应并不广泛；应仅用于累及喉部和脏器的严重病例。
