通过新生儿筛查确定的瑞典α1-抗胰蛋白酶缺乏队列在37至40岁时的健康状况和肺功能。
Health status and lung function in the Swedish alpha 1-antitrypsin deficient cohort, identified by neonatal screening, at the age of 37-40 years.
作者信息
Piitulainen Eeva, Mostafavi Behrouz, Tanash Hanan A
机构信息
Department of Respiratory Medicine and Allergology, Skåne University Hospital, Lund University, Malmö, Sweden.
出版信息
Int J Chron Obstruct Pulmon Dis. 2017 Feb 2;12:495-500. doi: 10.2147/COPD.S120241. eCollection 2017.
BACKGROUND
Severe alpha 1-antitrypsin (AAT) deficiency (genotype PiZZ) is a well-known risk factor for COPD. A cohort of PiZZ and PiSZ individuals was identified by the Swedish national neonatal AAT screening program in 1972-1974 and followed up regularly since birth. Our aim was to study the lung function, respiratory symptoms and health status at the age of 38 years in comparison with a random sample of control subjects selected from the population registry.
METHODS
The study group included 120 PiZZ, 46 PiSZ and 164 control subjects (PiMM), who answered a questionnaire on smoking habits and symptoms and the Saint George Respiratory Questionnaire (SGRQ) on quality of life. A total of 89 PiZZ, 33 PiSZ and 92 PiMM subjects underwent spirometry.
RESULTS
Four percent of the PiZZ, 2% of the PiSZ and 12% of the control subjects were current smokers (=0.008), and 17% of the PiZZ, 9% of the PiSZ and 21% of the control subjects had stopped smoking. The PiZZ current smokers had a significantly higher (ie, poorer) median activity score according to the SGRQ than the PiZZ never-smokers (=0.032). The PiMM current smokers had significantly higher activity score (<0.001), symptom score (<0.001), and total score (=0.001) according to the SGRQ than the PiMM never-smokers. The PiZZ current smokers had a significantly lower postbronchodilator forced expiratory volume in 1 second (FEV)% of predicted value (=0.019) and FEV/forced vital capacity (FVC) ratio (=0.032) than the PiZZ never-smokers. The proportion of subjects with a FEV/FVC ratio of <0.70, indicating COPD, was significantly higher in the PiZZ current smokers than in the PiZZ never-smokers (=0.001). Among the PiSZ and PiMM subjects, the differences in lung function between the smoking subgroups were insignificant.
CONCLUSION
PiZZ current smokers were found to have signs of COPD before 40 years of age. Smoking is less common among the AAT-deficient subjects identified by neonatal screening than among their peers in the general population.
背景
严重的α1-抗胰蛋白酶（AAT）缺乏症（PiZZ基因型）是慢性阻塞性肺疾病（COPD）的一个众所周知的危险因素。1972 - 1974年瑞典国家新生儿AAT筛查项目确定了一组PiZZ和PiSZ个体，并自出生起进行定期随访。我们的目的是与从人口登记处选取的随机对照样本相比，研究38岁时的肺功能、呼吸道症状和健康状况。
方法
研究组包括120名PiZZ个体、46名PiSZ个体和164名对照个体（PiMM），他们回答了关于吸烟习惯和症状的问卷以及关于生活质量的圣乔治呼吸问卷（SGRQ）。共有89名PiZZ个体、33名PiSZ个体和92名PiMM个体接受了肺活量测定。
结果
PiZZ个体中有4%、PiSZ个体中有2%以及对照个体中有12%为当前吸烟者（P = 0.008），PiZZ个体中有17%、PiSZ个体中有9%以及对照个体中有21%已戒烟。根据SGRQ，PiZZ当前吸烟者的活动评分中位数显著更高（即更差）（P = 0.032），高于PiZZ从不吸烟者。根据SGRQ，PiMM当前吸烟者的活动评分（P < 0.001）、症状评分（P < 0.001）和总分（P = 0.001）显著高于PiMM从不吸烟者。PiZZ当前吸烟者的支气管扩张剂后1秒用力呼气量（FEV）占预测值的百分比（P = 0.019）和FEV/用力肺活量（FVC）比值（P = 0.032）显著低于PiZZ从不吸烟者。FEV/FVC比值<0.70（表明患有COPD）的受试者比例在PiZZ当前吸烟者中显著高于PiZZ从不吸烟者（P = 0.001）。在PiSZ和PiMM个体中，吸烟亚组之间的肺功能差异不显著。
结论
发现PiZZ当前吸烟者在40岁之前有COPD的迹象。通过新生儿筛查确定的AAT缺乏个体中吸烟比一般人群中的同龄人少见。
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