Alpha 1-PI for emphysema due to alpha 1-antitrypsin deficiency.
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.
Horowitz
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Division of Pulmonary and Critical Care Medicine, Hahnemann University Hospital, Philadelphia, Pennsylvania.
Schulman
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D001798:Blood Proteins / Q000235:genetics / Q000494:pharmacology / Q000627:therapeutic use*; D006801:Humans; D011480:Protease Inhibitors / Q000235:genetics / Q000494:pharmacology / Q000627:therapeutic use*; D011656:Pulmonary Emphysema / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D019896:alpha 1-Antitrypsin Deficiency
D001798:Blood Proteins; D011480:Protease Inhibitors