Alpha 1-Pi for emphysema due to alpha 1-antitrypsin deficiency
Horowitz, I.D.; Schulman, E.S.
American Family Physician 40(3): 223-228
1989
ISSN/ISBN: 0002-838X PMID: 2672752 Document Number: 344016
Alpha 1-antitrypsin deficiency is a genetic disorder that may result in premature pulmonary emphysema. Affected persons are deficient in the protective protein, alpha 1-proteinase inhibitor (alpha 1-PI). The diagnosis should be considered in patients with signs of emphysema by age 40 (especially nonsmokers), those with predominantly lower lobe disease and those with a family history of premature emphysema. Recently, human alpha 1-PI has been purified and is available to prevent the development of disabling emphysema in affected individuals. Weekly infusion of the concentrate is effective in raising serum levels and is very safe, with only rare minor adverse effects.