Seersholm N, Kok-Jensen A, Dirksen A
Pulmonary Department P, Bispebjerg Hospital, Copenhagen, Denmark.
Am J Respir Crit Care Med. 1995 Dec;152(6 Pt 1):1922-5. doi: 10.1164/ajrccm.152.6.8520756.
Severe alpha 1-antitrypsin (alpha 1-AT) deficiency is characterized by a decrease in serum alpha 1-AT to values < 20% of normal. Severe airflow obstruction, most commonly due to pulmonary emphysema secondary to the destruction of pulmonary elastic tissue, may develop at an early age in persons with alpha 1-AT deficiency. The purpose of this study was to estimate the annual decline in FEV1 (delta FEV1) in alpha 1-AT-deficient patients, to compare delta FEV1 in a referral population (index cases) with subjects found through family studies (nonindex cases), and to evaluate the role of smoking cessation on delta FEV1. One hundred and sixty-one subjects from the Danish alpha 1-AT-deficiency study who were older than 25 yr and who had a recorded smoking history and at least two spirometric examinations 1 yr apart were studied. The delta FEV1 for each individual was determined by regression analysis of FEV1 on follow-up time. The overall mean delta FEV1 was 81 ml/yr. There was no significant difference in delta FEV1 between 113 index cases and 48 nonindex cases even after controlling for age, initial FEV1, sex, and lifetime tobacco consumption. One hundred of the subjects were ex-smokers, 18 had never smoked, and 43 were current smokers. The mean delta FEV1 among the current smokers was 132 ml/yr, versus 52 ml/yr among the ex-smokers (p < 0.001). For the never-smokers, the mean delta FEV1 was 86 ml/yr.
严重α1-抗胰蛋白酶（α1-AT）缺乏症的特征是血清α1-AT降低至正常水平的<20%。严重气流阻塞最常见于继发于肺弹性组织破坏的肺气肿，可在α1-AT缺乏症患者的早年出现。本研究的目的是估计α1-AT缺乏症患者的第一秒用力呼气容积（FEV1）的年下降率（ΔFEV1），比较转诊人群（索引病例）与通过家族研究发现的受试者（非索引病例）的ΔFEV1，并评估戒烟对ΔFEV1的作用。对来自丹麦α1-AT缺乏症研究的161名年龄超过25岁、有吸烟史记录且至少间隔1年进行过两次肺功能检查的受试者进行了研究。通过对FEV1与随访时间进行回归分析来确定每个个体的ΔFEV1。总体平均ΔFEV1为81 ml/年。即使在控制了年龄、初始FEV1、性别和终生烟草消费量之后，113例索引病例和48例非索引病例之间的ΔFEV1也没有显著差异。其中100名受试者为戒烟者，18名从未吸烟，43名是当前吸烟者。当前吸烟者的平均ΔFEV1为132 ml/年，而戒烟者为52 ml/年（p<0.001）。对于从不吸烟者，平均ΔFEV1为86 ml/年。
