Sveger T, Mazodier P
Thorax. 1979 Jun;34(3):397-400. doi: 10.1136/thx.34.3.397.
In 11 128 apparently healthy 18-year-old men screened for alpha 1-antitrypsin deficiency (AATD) 44 had an alpha 1-antitrypsin (AAT) level of 50% or less of the transferrin reference. In 42 of the 44 the Pi types were: five Pi Z, 10 Pi SZ, three Pi MZ, one presumptive Pi M-, one Pi FM, and 22 Pi M. Probably all Pi Z and most of the Pi SZ subjects were identified. The transferrin reference, however, is probably less reliable for the study of this age group, and alpha 1-antichymotrypsin may provide a more valid reference for the AAT screening procedure. In the clinical investigation the additive risk factor of smoking was discovered in eight of 15 individuals with AATD Pi Z or Pi SZ. We believe screening for Pi Z and probably also Pi SZ AATD should be done before young people start smoking or train for jobs in polluted environments.
在11128名接受α1-抗胰蛋白酶缺乏症(AATD)筛查的表面健康的18岁男性中,44人的α1-抗胰蛋白酶(AAT)水平为转铁蛋白参考值的50%或更低。在这44人中的42人,其Pi类型为:5人是Pi Z,10人是Pi SZ,3人是Pi MZ,1人推测为Pi M-,1人是Pi FM,22人是Pi M。可能所有Pi Z型和大多数Pi SZ型受试者都已被识别。然而,转铁蛋白参考值对于该年龄组的研究可能不太可靠,α1-抗糜蛋白酶可能为AAT筛查程序提供更有效的参考。在临床调查中,在15名AATD Pi Z型或Pi SZ型个体中的8人发现了吸烟这一附加风险因素。我们认为,应该在年轻人开始吸烟或接受污染环境中工作的培训之前,对Pi Z型以及可能还有Pi SZ型AATD进行筛查。