α-1抗胰蛋白酶缺乏症所致的肺部疾病急性加重
Exacerbations of Lung Disease in Alpha-1 Antitrypsin Deficiency.
作者信息
Smith Daniel J, Ellis Paul R, Turner Alice M
机构信息
Institute of Applied Health Research, University of Birmingham, Birmingham, United Kingdom.
University Hospitals Birmingham, United Kingdom.
出版信息
Chronic Obstr Pulm Dis. 2021 Jan;8(1):162-76. doi: 10.15326/jcopdf.2020.0173.
Abstract
Alpha-1 antitrypsin deficiency (AATD) is an important risk factor for development of chronic obstructive pulmonary disease (COPD). Patients with AATD classically develop a different pattern of lung disease from those with usual COPD, decline faster and exhibit a range of differences in pathogenesis, all of which may be relevant to phenotype and/or impact of exacerbations. There are a number of definitions of exacerbation, with the main features being worsening of symptoms over at least 2 days, which may be associated with a change in treatment. In this article we review the literature surrounding exacerbations in AATD, focusing, in particular, on ways in which they may differ from such events in usual COPD, and the potential impact on clinical management.
摘要
α-1抗胰蛋白酶缺乏症（AATD）是慢性阻塞性肺疾病（COPD）发生的重要危险因素。与普通COPD患者相比，AATD患者典型地表现出不同的肺部疾病模式，病情进展更快，并且在发病机制上存在一系列差异，所有这些差异可能与疾病表型和/或急性加重的影响有关。急性加重有多种定义，其主要特征是症状在至少2天内恶化，这可能与治疗的改变有关。在本文中，我们回顾了有关AATD急性加重的文献，特别关注它们与普通COPD急性加重事件的不同之处，以及对临床管理的潜在影响。
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