Signs of Hyperinflation and Ventilation Heterogeneity in Individuals With Severe Alpha-1-Antitrypsin Deficiency at the Age of 42.

IF 2.7 3区 医学 Q2 RESPIRATORY SYSTEM
Georg Rüdiger Schramm, Per Wollmer, Eeva Piitulainen, Suneela Zaigham, Hanan Tanash
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引用次数: 0

Abstract

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 (VA) and the ratio VA/Total lung capacity (VA/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 (VA). 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 VA/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 VA/TLC ratio (p=0.006) than the 24 PiMM ever-smokers. No significant differences in RV, TLC, VA, and VA/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.

42岁严重α -1-抗胰蛋白酶缺乏症患者的恶性通货膨胀和通气异质性的迹象
背景:严重α -1-抗胰蛋白酶缺乏症(AATD)是肺气肿和COPD早期发展的已知危险因素。1972- 1974年间,瑞典国家筛查项目确定了一组患有严重AATD (PiZZ)的个体,并定期随访。本研究的目的是调查肺泡容积(VA)和VA/总肺活量(VA/TLC)的比值,以检测PiZZ患者与年龄匹配的对照组(表型PiMM)的恶性通货膨胀和通气异质性的迹象。方法:所有研究参与者进行肺功能测试(PFT),包括动态肺活量测定和总肺活量(TLC)、残气量(RV)、功能残气量(FRC)和肺泡容积(VA)。他们回答了一份关于吸烟习惯和症状的问卷。通过回答圣乔治呼吸问卷(SGRQ)来评估生活质量。结果:56名PiZZ和66名PiMM个体参与了研究。PiZZ个体的VA/TLC比(p=0.004)显著低于PiMM人群,RV (pA/TLC)显著高于PiMM人群(p=0.006)。不吸烟PiZZ与不吸烟PiMM个体的RV、TLC、VA、VA/TLC比值无显著差异。PiZZ不吸烟者RV/TLC比值显著高于PiMM不吸烟者(p=0.026)。结论:42岁时,曾经吸烟的PiZZ有恶性充气和通气异质性的迹象。
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来源期刊
CiteScore
4.80
自引率
10.70%
发文量
372
审稿时长
16 weeks
期刊介绍: An international, peer-reviewed journal of therapeutics and pharmacology focusing on concise rapid reporting of clinical studies and reviews in COPD. Special focus will be given to the pathophysiological processes underlying the disease, intervention programs, patient focused education, and self management protocols. This journal is directed at specialists and healthcare professionals
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