Kramps J A, Bakker W, Dijkman J H
Am Rev Respir Dis. 1980 Feb;121(2):253-61. doi: 10.1164/arrd.1980.121.2.253.
The elastase-like activities in whole cell extracts of neutrophilic polymorphonuclear leukocytes of 10 emphysematous patients with alpha 1-antitrypsin deficiency (PiZ) and of 7 subjects with alpha 1-antitrypsin deficiency (PiZ or PiSZ) without emphysema were the same as the activities of healthy nondeficient subjects (PiMM) matched according to age, sex, and smoking history. In 16 emphysematous patients without alpha 1-antitrypsin deficiency (PiMM) however, the leukocyte elastase-like activity tended to be higher than that of normal control subjects. We concluded that in persons with alpha 1-antitrypsin deficiency, the leukocyte elastase-like activity is not a deciding factor in the development of emphysema. In persons without alpha 1-antitrypsin deficiency, however, the leukocyte elastase-like activity may be a contributing factor in the development of this disease. Variations in specific anti-leukocyte-elastase-like activity of alpha 1-antitrypsin, which might play a role in the pathogenesis of emphysema, were not found.
10名患有α1-抗胰蛋白酶缺乏症(PiZ)的肺气肿患者以及7名无肺气肿的α1-抗胰蛋白酶缺乏症(PiZ或PiSZ)受试者的中性多形核白细胞全细胞提取物中的类弹性蛋白酶活性,与根据年龄、性别和吸烟史匹配的健康非缺陷受试者(PiMM)的活性相同。然而,在16名无α1-抗胰蛋白酶缺乏症(PiMM)的肺气肿患者中,白细胞类弹性蛋白酶活性往往高于正常对照受试者。我们得出结论,在α1-抗胰蛋白酶缺乏症患者中,白细胞类弹性蛋白酶活性不是肺气肿发生的决定性因素。然而,在无α1-抗胰蛋白酶缺乏症的患者中,白细胞类弹性蛋白酶活性可能是该疾病发生的一个促成因素。未发现可能在肺气肿发病机制中起作用的α1-抗胰蛋白酶特异性抗白细胞类弹性蛋白酶活性的变化。