Alpha 1-antitrypsin deficiency and augmentation therapy in emphysema.
Since the first description of alpha 1-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 alpha 1-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 alpha 1-antitrypsin deficiency in a relatively unselected population.
Stoller
J K
JK
D004334:Drug Administration Schedule; D004646:Emphysema / Q000188:drug therapy* / Q000209:etiology / Q000235:genetics; D006801:Humans; D007262:Infusions, Intravenous; D000515:alpha 1-Antitrypsin / Q000235:genetics / Q000627:therapeutic use; D019896:alpha 1-Antitrypsin Deficiency
D000515:alpha 1-Antitrypsin