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严重α-1-抗胰蛋白酶缺乏症患者的特应性、哮喘和肺气肿

Atopy, asthma, and emphysema in patients with severe alpha-1-antitrypysin deficiency.

作者信息

Eden E, Mitchell D, Mehlman B, Khouli H, Nejat M, Grieco M H, Turino G M

机构信息

Department of Medicine, St. Luke's-Roosevelt Hospital Center, Columbia University College of Physicians and Surgeons, New York, New York, USA.

出版信息

Am J Respir Crit Care Med. 1997 Jul;156(1):68-74. doi: 10.1164/ajrccm.156.1.9508014.

DOI:10.1164/ajrccm.156.1.9508014
PMID:9230728
Abstract

Bronchial asthma is characterized by episodic airway obstruction and associated with wheezing, a bronchodilator response, an elevation in total serum IgE, and atopy. To determine whether asthma is more common in subjects with severe alpha 1-antitrypsin deficiency (alpha 1-ATD) and airway obstruction, we compared 38 patients who had this condition (Group 1) with 22 control patients with chronic obstructive pulmonary disease (COPD) (Group 2) and with five subjects with alpha 1-ATD and normal spirometry (Group 3). Subjects were evaluated with a symptom questionnaire, pulmonary function testing, intradermal allergen testing, and serum IgE measurement. Self-reported wheezing was a common symptom in all patient groups, but attacks of wheezing with dyspnea were significantly more common in Group 1. Of those patients with airway obstruction, more than 50% showed a bronchodilator response whether suffering from alpha 1-ATD or not. Atopy was more common in Group 1 than in Group 2 (48% versus 27%). Mean serum IgE for all groups was similar but significantly greater in patients with atopy. We estimated the prevalence of asthma in the study groups on the basis of the criteria of attacks of wheezing, reversible airway obstruction, atopy, and that increased IgE. The proportion of patients with asthma in Group 1 was significantly greater than that in Group 2 (22% versus 5%, p < 0.05). Our study shows that with control for the degree of airway obstruction, asthma, as defined, is more common in patients with alpha 1-ATD than in those without it. We suggest that a lack of alpha 1-AT in airways increases the propensity to develop asthma.

摘要

支气管哮喘的特征为发作性气道阻塞,并伴有喘息、支气管扩张剂反应、血清总IgE升高及特应性。为了确定哮喘在严重α1-抗胰蛋白酶缺乏症(α1-ATD)和气道阻塞患者中是否更为常见,我们将38例患有这种疾病的患者(第1组)与22例慢性阻塞性肺疾病(COPD)对照患者(第2组)以及5例α1-ATD且肺功能正常的受试者(第3组)进行了比较。通过症状问卷、肺功能测试、皮内过敏原测试和血清IgE测量对受试者进行评估。自我报告的喘息是所有患者组中的常见症状,但伴有呼吸困难的喘息发作在第1组中明显更为常见。在那些气道阻塞的患者中,无论是否患有α1-ATD,超过50%的患者表现出支气管扩张剂反应。特应性在第1组中比在第2组中更为常见(48%对27%)。所有组的平均血清IgE相似,但在特应性患者中显著更高。我们根据喘息发作、可逆性气道阻塞、特应性和IgE升高的标准估计了研究组中哮喘的患病率。第1组中哮喘患者的比例显著高于第2组(22%对5%,p<0.05)。我们的研究表明,在控制气道阻塞程度的情况下,所定义的哮喘在α1-ATD患者中比在无α1-ATD的患者中更为常见。我们认为气道中α1-AT的缺乏增加了患哮喘的倾向。

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