RT Journal Article
SR Electronic
T1 α<sub>1</sub>-Antitrypsin deficiency and augmentation therapy in emphysema
JF Cleveland Clinic Journal of Medicine
JO Cleve Clin J Med
FD Cleveland Clinic
SP 683
OP 689
VO 56
IS 7
A1 James K. Stoller
YR 1989
UL http://www.ccjm.org/content/56/7/683.abstract
AB Since the first description of α1-antitrypsin deficiency as a cause of emphysema in 1963, major strides have been made in understanding the structure and function of this important antiprotease, its role in the defense against emphysema, and in new treatment strategies. In order to elucidate the pathogenesis of antitrypsin-associated emphysema and recent developments in therapy for this disease, this paper considers current understanding of the pathogenesis of emphysema, recent information regarding the structure and function of the molecule, and more recent developments in augmentation therapy. The author also discusses the national registry that has been organized for better understanding of the natural history of α1-antitrypsin deficiency in a relatively unselected population.