Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: A Canadian Thoracic Society clinical practice guideline
Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: A Canadian Thoracic Society clinical practice guideline
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DOI：
10.1155/2012/920918
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发表时间：
2012-03-01
影响因子：
2.2
通讯作者：
Muthuri, S.
中科院分区：
文献类型：
--
作者：
Marciniuk, D. D.;Hernandez, P.;Muthuri, S.
Alpha-1 抗胰蛋白酶 (A1AT) 的主要功能是抑制中性粒细胞弹性蛋白酶，缺乏会使个体易患慢性阻塞性肺病 (COPD)。北美人口中，五千分之一到五千五百分之一的人患有严重的 A1AT 缺乏症。虽然确诊 COPD 患者中 A1AT 缺乏症的确切患病率尚不清楚，但小型研究的结果估计为 1% 至 5%。本文件更新了加拿大胸科学会 2001 年的立场声明，该声明的发起是因为对适合 A1AT 缺乏症针对性检测和 A1AT 增强治疗的合适患者缺乏共识和了解。使用修订后的指南制定方法，本临床实践指南文件系统地回顾了已发表的文献并提供了基于证据的更新。有证据支持这样的做法：应考虑对 65 岁之前诊断出患有慢性阻塞性肺病或有吸烟史的个体进行 A1AT 缺乏症的针对性检测。
Alpha-1 antitrypsin (A1AT) functions primarily to inhibit neutrophil elastase, and deficiency predisposes individuals to the development of chronic obstructive pulmonary disease (COPD). Severe A1AT deficiency occurs in one in 5000 to one in 5500 of the North American population. While the exact prevalence of A1AT deficiency in patients with diagnosed COPD is not known, results from small studies provide estimates of 1% to 5%. The present document updates a previous Canadian Thoracic Society position statement from 2001, and was initiated because of lack of consensus and understanding of appropriate patients suitable for targeted testing for A1AT deficiency, and for the use of A1AT augmentation therapy. Using revised guideline development methodology, the present clinical practice guideline document systematically reviews the published literature and provides an evidence-based update. The evidence supports the practice that targeted testing for A1AT deficiency be considered in individuals with COPD diagnosed before 65 years of age or with a smoking history of