[与α1-抗胰蛋白酶缺乏病因相关的原发性肺气肿患者的肺外呼吸功能]
[External respiratory function in patients with primary pulmonary emphysema etiologically related to alpha 1-antitrypsin deficiency].
作者信息
Gembitskaia T E, Kuznetsova V K
出版信息
Ter Arkh. 1985;57(3):91-6.
PMID:3873722
Abstract
To improve the diagnosis of primary lung emphysema etiologically linked with alpha-trypsin deficiency, the authors provide the results of a detailed clinico-functional study of 6 patients with an appreciable deficiency of this enzyme. According to the clinical and functional signs, all the patients were diagnosed to have a typical, pronounced lung emphysema. Therefore, the measurement of the alpha-antitrypsin content plays the crucial part in differential diagnosis of primary (hereditary determined) and secondary (obstructive) emphysema.
摘要
为了改善对与α-抗胰蛋白酶缺乏病因相关的原发性肺气肿的诊断，作者提供了对6例该酶明显缺乏患者进行详细临床功能研究的结果。根据临床和功能体征，所有患者均被诊断为典型的、明显的肺气肿。因此，α-抗胰蛋白酶含量的测定在原发性（遗传决定）和继发性（阻塞性）肺气肿的鉴别诊断中起着关键作用。
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