Eriksson S, Wu M C
Dept of Medicine, University of Lund, Malmo General Hospital, Sweden.
Eur Respir J Suppl. 1990 Mar;9:39s-43s.
Data were analysed on 158 adult Swedish alpha 1-antitrypsin deficient individuals homozygous for the proteinase inhibitor allele (PiZZ), followed from 1963-1982. The initial forced expiratory volume in one second (FEV1) increased from 1.4 l in the early cohort to 1.9 l (p less than 0.001) in the late cohort of the study period but mean age (47 yrs) and the percentage of smokers (72%) remained unchanged. Smokers were found to have significantly lower FEV1 levels (p less than 0.01) and higher (p less than 0.005) mortality than non-smokers. The differences between current and ex-smokers in mortality and FEV1 level, however, were not significant. Longitudinal analysis of FEV1 rates of decline in 80 cases with follow-up FEV1 measurements failed to detect any significant difference between current and ex-smokers but investigations were performed late in the disease process. Low initial FEV1 value was found to be significantly associated with increased mortality (p less than 0.005). In order to detect a 40% difference in the rate of FEV1 decline as an outcome variable, 253 patients plus controls must be included in follow-up studies of replacement therapy. With mortality of severely affected patients as outcome variable only, 144 treated and untreated patients would be required. The need for earlier diagnosis and intervention against smoking in these cases is emphasized.
对1963年至1982年期间随访的158名瑞典成年α1 -抗胰蛋白酶缺乏症患者（蛋白酶抑制剂等位基因纯合子，PiZZ）的数据进行了分析。研究期间，早期队列的初始一秒用力呼气量（FEV1）从1.4升增加到晚期队列的1.9升（p<0.001），但平均年龄（47岁）和吸烟者比例（72%）保持不变。发现吸烟者的FEV1水平显著较低（p<0.01），死亡率较高（p<0.005）。然而，当前吸烟者和既往吸烟者在死亡率和FEV1水平上的差异并不显著。对80例有随访FEV1测量值的患者进行FEV1下降率的纵向分析，未发现当前吸烟者和既往吸烟者之间有任何显著差异，但这些研究是在疾病过程后期进行的。发现初始FEV1值低与死亡率增加显著相关（p<0.005）。为了检测作为结果变量的FEV1下降率有40%的差异，替代疗法的随访研究必须纳入253例患者加对照。仅将重度受影响患者的死亡率作为结果变量时，需要144例接受治疗和未接受治疗的患者。强调了在这些病例中早期诊断和戒烟干预的必要性。
