42岁严重α-1抗胰蛋白酶缺乏个体的肺过度充气和通气不均一性迹象
Signs of Hyperinflation and Ventilation Heterogeneity in Individuals With Severe Alpha-1-Antitrypsin Deficiency at the Age of 42.
作者信息
Schramm Georg Rüdiger, Wollmer Per, Piitulainen Eeva, Zaigham Suneela, Tanash Hanan
机构信息
Department of Internal Medicine, Skåne University Hospital, Malmö, Sweden, Lund University, Lund, Sweden.
Department of Medical Imaging and Physiology, Skåne University Hospital, Malmö, Sweden, Lund University, Lund, Sweden.
出版信息
Int J Chron Obstruct Pulmon Dis. 2025 Mar 5;20:539-549. doi: 10.2147/COPD.S486575. eCollection 2025.
BACKGROUND
Severe alpha-1-antitrypsin deficiency (AATD) is a known risk factor for early development of emphysema and COPD. By the Swedish national screening program within the years 1972-74 a cohort of individuals with severe AATD (PiZZ) was identified and regularly followed up. The aim of this study was to investigate alveolar volume (V) and the ratio V/Total lung capacity (V/TLC) for the detection of signs of hyperinflation and ventilation heterogeneity in PiZZ individuals compared with an age-matched control group (phenotype PiMM), randomly selected from the population registry.
METHODS
All study participants underwent pulmonary function tests (PFT) including dynamic spirometry and total lung capacity (TLC), residual volume (RV), functional residual capacity (FRC) and alveolar volume (V). They answered a questionnaire on smoking habits and symptoms. Quality of life was assessed by answering the Saint George´s Respiratory Questionnaire (SGRQ).
RESULTS
Fifty-six PiZZ and 66 PiMM individuals participated in the study. The PiZZ individuals had a significantly lower V/TLC ratio (p=0.004) and significantly higher RV (p<0.001) and RV/TLC ratio (p=0.001) than the PiMM individuals. The 13 PiZZ ever-smokers had a significantly higher TLC (p=0.025), RV (p=0.003) and FRC (p=0.003) and a significantly lower V/TLC ratio (p=0.006) than the 24 PiMM ever-smokers. No significant differences in RV, TLC, V, and V/TLC ratio were found between the never-smoking PiZZ and PiMM individuals. The ratio RV/TLC was significantly higher in the PiZZ never-smokers than in the PiMM never-smokers (p=0.026).
CONCLUSION
At the age of 42 the PiZZ ever-smokers have signs of hyperinflation and ventilation heterogeneity.
背景
严重的α-1抗胰蛋白酶缺乏症（AATD）是肺气肿和慢性阻塞性肺疾病（COPD）早期发展的已知风险因素。通过1972 - 1974年瑞典国家筛查项目，确定了一组患有严重AATD（PiZZ型）的个体并对其进行定期随访。本研究的目的是调查PiZZ型个体与从人口登记处随机选取的年龄匹配对照组（PiMM型）相比，肺泡容积（V）以及V/肺总量（V/TLC）比值，以检测肺过度充气和通气异质性的体征。
方法
所有研究参与者均接受了肺功能测试（PFT），包括动态肺量计检查以及肺总量（TLC）、残气量（RV）、功能残气量（FRC）和肺泡容积（V）测定。他们回答了关于吸烟习惯和症状的问卷。通过回答圣乔治呼吸问卷（SGRQ）评估生活质量。
结果
56名PiZZ型个体和66名PiMM型个体参与了研究。PiZZ型个体的V/TLC比值显著更低（p = 0.004），RV和RV/TLC比值显著更高（p < 0.001和p = 0.001），与PiMM型个体相比。13名曾经吸烟的PiZZ型个体的TLC（p = 0.025）、RV（p = 0.003）和FRC（p = 0.003）显著更高，V/TLC比值显著更低（p = 0.006），与24名曾经吸烟的PiMM型个体相比。从不吸烟的PiZZ型个体和PiMM型个体在RV、TLC、V和V/TLC比值方面未发现显著差异。PiZZ型从不吸烟者的RV/TLC比值显著高于PiMM型从不吸烟者（p = 0.026）。
结论
42岁时，曾经吸烟的PiZZ型个体有肺过度充气和通气异质性的体征。
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