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Pi系统中的杂合性作为慢性阻塞性肺疾病(COPD)的致病辅助因素。

Heterozygosity in the Pi-system as a pathogenetic cofactor in chronic obstructive pulmonary disease (COPD).

作者信息

Bartmann K, Fooke-Achterrath M, Koch G, Nagy I, Schütz I, Weis E, Zierski M

出版信息

Eur J Respir Dis. 1985 Apr;66(4):284-96.

PMID:3874784
Abstract

A population (n = 526), consisting of employees with COPD, was compared with 2 control populations for the prevalence of Pi-phenotypes. In the patient group, the proportions of ZZ, SZ and MZ were significantly elevated. Among the patient population a prospective study was carried out to evaluate the role of alpha 1-antitrypsin deficiency as a cofactor in COPD. Severity of disease was estimated by standard pulmonary function tests, X-ray signs for emphysema and clinical assessment. Patients with ZZ, SZ and MZ were significantly worse than their MM partners. An influence of MS cannot be rejected. Phenotyping of all patients with COPD is advocated. Screening can also be by determining the ratio of alpha 1-antitrypsin and acid alpha 1-glycoprotein concentrations, which allowed detection of all ZZ, SZ, MZ and about 60% of the MS patients.

摘要

对一组患有慢性阻塞性肺疾病(COPD)的员工(n = 526)进行了研究,将其与两个对照人群比较Pi表型的患病率。在患者组中,ZZ、SZ和MZ的比例显著升高。在患者人群中开展了一项前瞻性研究,以评估α1-抗胰蛋白酶缺乏作为慢性阻塞性肺疾病辅助因素的作用。通过标准肺功能测试、肺气肿的X线征象和临床评估来估计疾病的严重程度。ZZ、SZ和MZ患者比其MM型同伴的病情明显更严重。不能排除MS型的影响。提倡对所有慢性阻塞性肺疾病患者进行表型分析。筛查也可以通过测定α1-抗胰蛋白酶和酸性α1-糖蛋白浓度的比值来进行,这可以检测出所有的ZZ、SZ、MZ型患者以及约60%的MS型患者。

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