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α1-抗胰蛋白酶缺乏症(PiZ)中慢性咳痰与生理、放射学及健康状况特征的关系。

The relationship of chronic sputum expectoration to physiologic, radiologic, and health status characteristics in alpha(1)-antitrypsin deficiency (PiZ).

作者信息

Dowson Lee J, Guest Peter J, Stockley Robert A

机构信息

Lung Investigation Unit, Nuffield House, Queen Elizabeth University Hospital, Birmingham B15 2TH, UK.

出版信息

Chest. 2002 Oct;122(4):1247-55. doi: 10.1378/chest.122.4.1247.

DOI:10.1378/chest.122.4.1247
PMID:12377849
Abstract

STUDY OBJECTIVES

First, to determine the relationships among chronic sputum expectoration (CSE), exacerbations, airflow obstruction, and emphysema in patients with alpha(1)-antitrypsin deficiency (alpha(1)-ATD) [PiZ]. Second, to use multivariate analysis to determine how these factors influence health status.

DESIGN

Cross-sectional, single-center.

SETTING

UK center for alpha(1)-ATD, university teaching hospital.

PATIENTS

One hundred seventeen nonsmoking patients underwent lung function testing, high-resolution CT (HRCT) scanning with density mask analysis, and health status assessment using the St. George's Respiratory Questionnaire (SGRQ) and short form 36 (SF-36) health survey questionnaire.

RESULTS

Patients with CSE (n = 50) had worse postbronchodilator airflow obstruction than those who did not (p = 0.03), with a median FEV(1) of 1.15 L (interquartile range [IQR], 0.76 to 1.82) vs 1.44 L (IQR, 0.99 to 2.93), respectively, and higher HRCT scan voxel index (VI) values indicating more extensive emphysema (patients with CSE: median lower zone VI, 50; IQR, 28 to 61; patients without CSE: median lower zone VI, 41; IQR, 5 to 53; p = 0.04). Patients with CSE also had worse health status, as assessed by the SGRQ (p < 0.01 for all domains) and SF-36 questionnaire (p < 0.05 for seven of nine domains). Exacerbation frequency was greater in those patients with CSE (p < 0.001), with a median of two episodes per year (IQR, 1 to 3) vs 0.66 episodes per year (IQR, 0 to 2) for those without CSE. Stepwise linear regression analysis revealed FEV(1), exacerbation frequency, and lower zone VI to be the most important predictors of health status.

CONCLUSIONS

Among patients with alpha(1)-ATD, those with CSE expectoration exhibit greater physiologic impairment and more extensive emphysema than those without. This is reflected in an inferior health status, which is also influenced independently by an increased exacerbation frequency in those with CSE.

摘要

研究目的

第一,确定α1-抗胰蛋白酶缺乏症(α1-ATD)[PiZ型]患者的慢性咳痰(CSE)、急性加重、气流受限和肺气肿之间的关系。第二,使用多变量分析确定这些因素如何影响健康状况。

设计

横断面、单中心研究。

地点

英国α1-ATD中心,大学教学医院。

患者

117名不吸烟患者接受了肺功能测试、带密度面罩分析的高分辨率CT(HRCT)扫描,以及使用圣乔治呼吸问卷(SGRQ)和简短健康调查问卷36(SF-36)进行的健康状况评估。

结果

有CSE的患者(n = 50)支气管扩张剂后气流受限情况比无CSE的患者更差(p = 0.03),FEV1中位数分别为1.15 L(四分位间距[IQR],0.76至1.82)和1.44 L(IQR,0.99至2.93),且HRCT扫描体素指数(VI)值更高,表明肺气肿更广泛(有CSE的患者:下叶VI中位数为50;IQR,28至61;无CSE的患者:下叶VI中位数为41;IQR,5至53;p = 0.04)。根据SGRQ(所有领域p < 0.01)和SF-36问卷(九个领域中的七个领域p < 0.05)评估,有CSE的患者健康状况也更差。有CSE的患者急性加重频率更高(p < 0.001),每年中位数为2次发作(IQR, 1至年3次),而无CSE的患者每年为0.66次发作(IQR, 0至2次)。逐步线性回归分析显示,FEV1、急性加重频率和下叶VI是健康状况的最重要预测因素。

结论

在α1-ATD患者中,有CSE咳痰的患者比无咳痰的患者表现出更严重的生理损害和更广泛的肺气肿。这反映在较差的健康状况上,CSE患者急性加重频率增加也独立影响健康状况。

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