Seersholm N, Kok-Jensen A, Dirksen A
Department of Pulmonology, Bispebjerg Hospital, Copenhagen, Denmark.
Thorax. 1994 Jul;49(7):695-8. doi: 10.1136/thx.49.7.695.
Previous estimates of the survival times of patients with alpha 1-antitrypsin deficiency have been based on selected patients.
The survival times of 397 patients with severe alpha 1-antitrypsin deficiency identified by pulmonary impairment (index cases) or through family studies (non-index cases) were compared.
The overall median survival time was 54.5 years with no significant difference between men and women. Survival for index cases was less than for the non-index cases regardless of smoking history (49.4 years and 69.3 years respectively). When index and non-index cases were analysed separately there was no difference between the survival of smokers and never smokers in the index group. In the non-index group smokers had a shorter survival time than never smokers. The survival time of never smokers was similar to that of the normal Danish population.
The prognosis of severe alpha 1-antitrypsin deficiency is better than previously assumed and, although smoking is a major risk factor, the development of emphysema in patients with severe alpha 1-antitrypsin deficiency is multifactorial.
先前对α1-抗胰蛋白酶缺乏症患者生存时间的估计是基于特定患者群体。
比较了397例因肺部损伤确诊的重度α1-抗胰蛋白酶缺乏症患者（索引病例）以及通过家族研究确诊的患者（非索引病例）的生存时间。
总体中位生存时间为54.5年，男性和女性之间无显著差异。无论吸烟史如何，索引病例的生存期均短于非索引病例（分别为49.4年和69.3年）。当分别分析索引病例和非索引病例时，索引组中吸烟者和从不吸烟者的生存期无差异。在非索引组中，吸烟者的生存期短于从不吸烟者。从不吸烟者的生存时间与丹麦正常人群相似。
重度α1-抗胰蛋白酶缺乏症的预后比先前设想的要好，尽管吸烟是主要危险因素，但重度α1-抗胰蛋白酶缺乏症患者肺气肿的发展是多因素的。
