Alpha-1 Antitrypsin Deficiency: The European Experience

被引:17
作者
Stockley, Robert A. [1 ]
Dirksen, Asger [2 ]
Stolk, Jan [3 ]
机构
[1] Univ Hosp Birmingham NHS Trust, Lung Invest Unit, Birmingham, W Midlands, England
[2] Gentofte Univ Hosp, Dept Resp Med, Hellerup, Denmark
[3] Leiden Univ, Med Ctr, Dept Pulmonol, Leiden, Netherlands
关键词
alpha-1-antitrypsin; deficiency; emphysema; AUGMENTATION THERAPY; LUNG DENSITOMETRY; ALPHA(1)-ANTITRYPSIN; ALPHA1-ANTITRYPSIN; EXACERBATIONS; INHIBITOR; SERUM;
D O I
10.3109/15412555.2013.765838
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
AATD is a European genetic condition that has disseminated along human migration routes. The discovery, function, phenotyping methodologies and biochemical mechanisms have been led by several European countries. The variable availability of augmentation therapy has permitted a better understanding of the natural history and the ability to deliver controlled clinical trials. The establishment of a worldwide registry remains central to the future of understanding and managing AATD.
引用
收藏
页码:50 / 53
页数:4
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