[遗传性α-1抗胰蛋白酶缺乏症患者慢性阻塞性肺疾病的临床表现变异性]
[The variability of clinical presentation of chronic obstructive pulmonary disease in patients with hereditary alpha-1 antitrypsin deficiency].
作者信息
Kuca Paweł, Kamiński Dariusz, Campbell Edward, Kołakowski Jacek, Goljan-Geremek Anna, Puścińska Elzbieta, Bieleń Przemysław, Nowiński Adam, Wojda Emil, Hawryłkiewicz Iwona, Sliwiński Paweł, Górecka Dorota
机构信息
Oddziału Intensywnej Terapii Pneumonologiczno--Kardiologicznej, Warszawa.
出版信息
Pneumonol Alergol Pol. 2004;72(9-10):420-3.
Four patients with alpha-1 antitrypsin (alpha-1 AT) deficiency are presented: one woman with severe (phenotype PiZ) and 3 men with moderate (phenotype PiMZ) deficiency of alpha-1 AT. The variability of clinical presentation of hereditary emphysema is described. In all patients tobacco smoking history, spirometric and 6-minutes walking tests as well as HRCT of the lung were performed and compared. The influence of smoking on the functional status is underlined.
本文介绍了4例α1抗胰蛋白酶（α1AT）缺乏症患者：1例患有严重α1AT缺乏症（PiZ型）的女性和3例患有中度α1AT缺乏症（PiMZ型）的男性。描述了遗传性肺气肿临床表现的变异性。对所有患者进行了吸烟史、肺功能测定和6分钟步行试验以及肺部高分辨率CT（HRCT）检查，并进行了比较。强调了吸烟对功能状态的影响。
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