Drouet C, Alibeu C, Ponard D, Arlaud G J, Colomb M G
Laboratoire d'Immunologie, Hôpital Sud, CHU Grenoble, Echirolles, France.
Clin Chim Acta. 1988 May 31;174(2):121-30. doi: 10.1016/0009-8981(88)90379-8.
Hereditary angioneurotic edema results from deficiency of complement protein C1- inhibitor. Using a new spectrophotometric assay for C1-s esterase activity on the N-alpha-benzoyl-L-arginine ethyl ester, we describe a routinely available method for quantifying low C1- Inhibitor functional activities in EDTA-treated plasma of hereditary angioneurotic edema patients. C1- Inhibitor activity is deduced from the residual esterase activity of C1-s incubated with 20-80 microliters plasma samples. Arbitrary units (volume of sample inhibiting 50% of C1-s activity) were used to express C1- Inhibitor normal activity which was estimated as 22,500 +/- 5,000 (SD) U/l in 45 healthy individuals. The correlation with C1- Inhibitor antigen in these healthy individuals and 89 patients with varying concentrations of C1 Inhibitor ranging from 0.05-1.05 g/l was r = 0.91. Levels down to 2,000 U/l could be estimated. Specific inhibitory activity is an absolute requirement to distinguish between type I and type II hereditary angioneurotic edema.
遗传性血管性水肿是由补体蛋白C1-抑制剂缺乏引起的。我们使用一种针对N-α-苯甲酰-L-精氨酸乙酯的C1-s酯酶活性的新分光光度测定法，描述了一种常规可用的方法，用于定量遗传性血管性水肿患者经乙二胺四乙酸（EDTA）处理的血浆中低C1-抑制剂的功能活性。C1-抑制剂活性是通过与20-80微升血浆样本孵育的C1-s的残余酯酶活性推导出来的。使用任意单位（抑制50% C1-s活性的样本体积）来表示C1-抑制剂正常活性，在45名健康个体中估计为22,500±5,000（标准差）U/l。在这些健康个体和89名C1抑制剂浓度在0.05-1.05 g/l之间变化的患者中，与C1-抑制剂抗原的相关性为r = 0.91。可以估计低至2,000 U/l的水平。特异性抑制活性是区分I型和II型遗传性血管性水肿的绝对必要条件。
