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1963年Laurell及Eriksson报告5例血清电泳中α_1-球蛋白明显缺乏者,其中3例有慢性支气管炎、肺炎和肺气肿等肺部病变,且有家族史,故提出这是一组遗传性缺陷病。1967年Ganrot等报告50例α_1-球蛋白缺乏,30例有慢性肺部病变,另20例无肺部病变者中,2例死于肝硬化和1例死于肝癌。1969年Sharp等将肝硬化、肺气肿和α_1-球蛋白缺乏作为一个特殊的遗传性疾病,现统一命名为α_1-抗胰蛋白酶(简称α_1-AT)缺乏症。常表现为新生儿肝炎、婴幼儿肝硬化、早年发病的阻塞性肺气肿。
Laurell and Eriksson in 1963 reported a clear lack of α 1-globulin in serum electrophoresis, of which 3 cases of chronic bronchitis, pneumonia and emphysema and other lung diseases, and a family history, it is proposed that this is a hereditary Defect disease. In 1967 Ganrot et al reported 50 cases of α 1-globulin deficiency, 30 cases of chronic lung disease, and the other 20 cases of lung disease, 2 died of cirrhosis and 1 died of liver cancer. In 1969, Sharp et al. Characterized cirrhosis, emphysema and a-globulin deficiency as a special genetic disease and are now uniformly named α_1-antitrypsin (α_1-AT) deficiency. Often manifested as neonatal hepatitis, cirrhosis of infants and young children, early onset of obstructive pulmonary emphysema.