Evald T, Dirksen A, Keittelmann S, Viskum K, Kok-Jensen A
Department of Pulmonary Medicine P, Bispebjerg Hospital, Copenhagen, Denmark.
Lung. 1990;168 Suppl:579-85. doi: 10.1007/BF02718180.
Sixty-five patients with severe alpha 1-antitrypsin (AAT) deficiency (phenotype PiZ) were followed with spirometry at regular intervals of one year and a median observation period of four years. The annual decline in pulmonary function was adjusted for sex, age and height by division with the predicted normal pulmonary function. The median decline in FEV1 was 1.9% predicted/year. The rate of decline was independent of age and pulmonary function, except for patients with FEV1 below 25% of predicted normal. There was a tendency towards a slower median decline in FEV1 in ex-smokers (1.7% predicted/year) compared to smokers (3.8% predicted/year) and never-smokers (3.7% predicted/year), however, this difference was not significant (p greater than 0.01). At the time of diagnosis smokers and ex-smokers had a lower FEV1 (44 and 38% predicted) than never-smokers (85% predicted) (p less than 0.02), and smokers and ex-smokers were generally younger (median age 44 and 42 years, respectively) than never smokers (median age 55 years) (p greater than 0.1). Our data indicate that smokers as well as nonsmokers with severe AAT deficiency are at risk of developing pulmonary emphysema. The disease seems to appear later in nonsmokers, though once initiated it progresses at the same rate.
对65例严重α1-抗胰蛋白酶（AAT）缺乏症（PiZ表型）患者进行了随访，定期（每年一次）进行肺功能测定，中位观察期为4年。通过将实际肺功能除以预测的正常肺功能，对肺功能的年度下降情况进行了性别、年龄和身高的校正。FEV1的中位下降率为预测值的1.9%/年。下降速率与年龄和肺功能无关，但FEV1低于预测正常值25%的患者除外。与吸烟者（预测值的3.8%/年）和从不吸烟者（预测值的3.7%/年）相比，已戒烟者FEV1的中位下降速率有减缓趋势（预测值的1.7%/年），然而，这种差异并不显著（p>0.01）。在诊断时，吸烟者和已戒烟者的FEV1（分别为预测值的44%和38%）低于从不吸烟者（预测值的85%）（p<0.02），且吸烟者和已戒烟者通常比从不吸烟者年轻（中位年龄分别为44岁和42岁）（p>0.1）。我们的数据表明，患有严重AAT缺乏症的吸烟者和非吸烟者都有患肺气肿的风险。该疾病在非吸烟者中似乎出现得较晚，不过一旦发病，其进展速率相同。
