Eden Edward, Hammel Jeffrey, Rouhani Farshid N, Brantly Mark L, Barker Alan F, Buist A Sonia, Fallat Robert J, Stoller James K, Crystal Ronald G, Turino Gerard M
James P. Mara Center for Lung Disease, St Luke's-Roosevelt Hospital Center, New York, NY 10019, USA.
Chest. 2003 Mar;123(3):765-71. doi: 10.1378/chest.123.3.765.
To describe asthma features in a cohort with alpha(1)-antitrypsin (AAT) deficiency, and determine the impact of asthma on FEV(1) decline.
Asthma may be common in those with AAT deficiency, and may lead to accelerated airflow obstruction.
Analysis of data obtained from a 5-year, prospective National Heart, Lung, and Blood Institute registry.
A multicenter registry consisting of 37 clinical centers, a central phenotyping laboratory, and a data analysis center.
A cohort of 1,052 subjects with AAT deficiency.
Asthma was defined as reversible airflow obstruction, recurrent attacks of wheezing, and a reported diagnosis of asthma or allergy with or without an elevated serum IgE level. FEV(1) decline was calculated by least-square means with adjustments for covariables. Asthma was present in 21% of the cohort and in 12.5% of those with a normal FEV(1). Attacks of wheezing were reported in 66%, the first attack occurring at a mean +/- SD age of 31 +/- 16 years. Allergy and asthma was reported in 29% and 38%, respectively. An elevated IgE level occurred in 17% and was significantly associated with signs and symptoms of asthma and an allergy history. Unadjusted FEV(1) decline was less in the group without asthma and a normal IgE level (- 48.5 mL/yr) vs the groups with asthma features (> or = 64 mL/yr) [p = 0.002]. Multivariable analysis showed that bronchodilator response, age, and smoking were significant predictors for FEV(1) decline but not asthma.
Symptoms and signs of asthma are common in AAT deficiency and may start at the age of most rapid FEV(1) loss. Adjusting for other risk factors such as bronchodilator response, asthma as defined does not lead to an accelerated FEV(1) decline. In AAT deficiency, augmentation therapy is not more effective in preventing the loss of lung function in those with asthma compared to those without.
描述α1抗胰蛋白酶（AAT）缺乏症队列中的哮喘特征，并确定哮喘对第一秒用力呼气容积（FEV1）下降的影响。
哮喘在AAT缺乏症患者中可能很常见，并可能导致气流阻塞加速。
对美国国立心肺血液研究所一项为期5年的前瞻性注册研究获得的数据进行分析。
一个多中心注册机构，由37个临床中心、一个中央表型分析实验室和一个数据分析中心组成。
1052名AAT缺乏症受试者队列。
哮喘定义为可逆性气流阻塞、反复发作的喘息，以及报告的哮喘或过敏诊断，无论血清IgE水平是否升高。FEV1下降通过最小二乘法计算，并对协变量进行调整。该队列中21%的患者患有哮喘，FEV1正常的患者中12.5%患有哮喘。66%的患者报告有喘息发作，首次发作的平均年龄为31±16岁。分别有29%和38%的患者报告有过敏和哮喘。17%的患者IgE水平升高，且与哮喘症状体征及过敏史显著相关。未患哮喘且IgE水平正常的组中，未经调整的FEV1下降较少（-48.5 mL/年），而具有哮喘特征的组中FEV1下降较多（≥64 mL/年）[p = 0.002]。多变量分析显示，支气管扩张剂反应、年龄和吸烟是FEV1下降的显著预测因素，但哮喘不是。
哮喘的症状体征在AAT缺乏症中很常见，且可能始于FEV1下降最快的年龄。在调整了支气管扩张剂反应等其他风险因素后，所定义的哮喘不会导致FEV1加速下降。在AAT缺乏症中，与无哮喘者相比，增强治疗在预防哮喘患者肺功能丧失方面并无更显著效果。
