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30 岁α-1-抗胰蛋白酶缺乏症个体的肺功能。

Lung function in 30-year-old alpha-1-antitrypsin-deficient individuals.

机构信息

Department of Respiratory Medicine, Lund University, University Hospital, Entrance 35, SE-205 02 Malmö, Sweden.

出版信息

Respir Med. 2009 Jun;103(6):861-5. doi: 10.1016/j.rmed.2008.12.021. Epub 2009 Feb 1.

Abstract

BACKGROUND

Alpha-1-antitrypsin (AAT) deficiency increases the risk of emphysema, especially in smokers. In 1972-1974, all 200,000 Swedish new-born infants were screened for AAT deficiency and individuals with severe (PiZZ) and moderate (PiSZ) deficiency have been followed-up regularly. The aim of the present study was to examine their lung function at the age of 30 years, comparing them to a group of age-matched control subjects (PiMM) recruited from the general population, and to compare current smokers with never-smokers.

METHOD

Static and dynamic spirometry, including TLC, FRC, RV, VC, FEV(1,)K(CO) and D(L,CO), was performed for all participants. All values were expressed as percentages of the expected values. FEV(1)/VC was expressed both as percentage of the expected value and in absolute numbers.

RESULTS

Four of 60 PiZZ, none of 19 PiSZ and 9 of 33 PiMM participating individuals were current smokers. All Pi groups had a normal mean FEV(1). The mean (SD) FEV(1)/VC ratio was 75% (7.4) in the PiZZ smokers and 84% (5.5) in the PiZZ never-smokers (p<0.01). The mean (SD) K(CO) was 81 (13) in the PiZZ smokers and 99 (14) in the PiZZ never-smokers (p<0.05).

CONCLUSION

AAT-deficient individuals identified by neonatal screening have normal lung function at the age of 30. The PiZZ smokers had changes in lung function that may be signs of early emphysema.

摘要

背景

α-1-抗胰蛋白酶(AAT)缺乏症会增加肺气肿的风险,尤其是在吸烟者中。1972-1974 年,所有 20 万名瑞典新生儿都接受了 AAT 缺乏症筛查,患有严重(PiZZ)和中度(PiSZ)缺乏症的个体已定期接受随访。本研究旨在 30 岁时检查他们的肺功能,将他们与一组年龄匹配的来自普通人群的对照(PiMM)进行比较,并比较当前吸烟者与从不吸烟者。

方法

所有参与者均进行静态和动态肺量测定,包括 TLC、FRC、RV、VC、FEV(1)、K(CO)和 D(L,CO)。所有值均表示为预计值的百分比。FEV(1)/VC 既表示为预计值的百分比,也表示为绝对值。

结果

60 名 PiZZ 中有 4 名、19 名 PiSZ 中无、33 名 PiMM 中有 9 名参与者是当前吸烟者。所有 Pi 组的平均 FEV(1)均正常。PiZZ 吸烟者的平均(SD)FEV(1)/VC 比值为 75%(7.4),PiZZ 从不吸烟者为 84%(5.5)(p<0.01)。PiZZ 吸烟者的平均(SD)K(CO)为 81(13),PiZZ 从不吸烟者为 99(14)(p<0.05)。

结论

通过新生儿筛查确定的 AAT 缺乏症个体在 30 岁时肺功能正常。PiZZ 吸烟者的肺功能发生变化,可能是早期肺气肿的迹象。

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