重度α1-抗胰蛋白酶缺乏症患者的生存情况，特别提及非索引病例。
Survival of patients with severe alpha 1-antitrypsin deficiency with special reference to non-index cases.
作者信息
Seersholm N, Kok-Jensen A, Dirksen A
机构信息
Department of Pulmonology, Bispebjerg Hospital, Copenhagen, Denmark.
出版信息
Thorax. 1994 Jul;49(7):695-8. doi: 10.1136/thx.49.7.695.
Abstract
BACKGROUND
Previous estimates of the survival times of patients with alpha 1-antitrypsin deficiency have been based on selected patients.
METHODS
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.
RESULTS
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.
CONCLUSIONS
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.
摘要
背景
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。
方法
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。
结果
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。
结论
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。
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