Pulmonary Disease in Alpha-1-Antitrypsin Deficiency
Authors: Gordon L. Snider
Journal: Annals of Internal Medicine (1989)

Editorials15 December 1989Pulmonary Disease in Alpha-1-Antitrypsin DeficiencyGordon L. Snider, MDGordon L. Snider, MDAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-111-12-957 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe current acceleration of technologic advances in medicine presents physicians and society with repeated challenges. For physicians, the challenge is how best to apply the new technology; for society, the challenge is how to pay for it. The release in December 1987 of a purified preparation of human alpha-1-antitrypsin (AAT) for augmentation therapy of AAT-deficient persons typifies these problems.The serum protein AAT, also known as alpha-1-protease inhibitor, is an inhibitor of serine proteases. Its main substrate in vivo is the serine protease, neutrophil elastase. Severe AAT deficiency is associated with the development of pulmonary emphysema in early or mid-life....References1. Janoff A. Elastases and emphysema. Current assessment of the protease-antiprotease hypothesis. Am Rev Respir Dis. 1985;132:417-33. MedlineGoogle Scholar2. Snider G, Lucey E, and Stone P. Animal models of emphysema. Am Rev Respir Dis. 1986;133:149-69. CrossrefMedlineGoogle Scholar3. Snider G, Lucey E, and Christensen T. Emphysema and bronchial secretory cell metaplasia induced in hamsters by human neutrophil products. Am Rev Respir Dis. 1984;129:155-60. MedlineGoogle Scholar4. Sveger T. Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants. N Engl J Med. 1976;294:1316-21. CrossrefMedlineGoogle Scholar5. Eriksson S, Carlson J, and Velez R. Risk of cirrhosis and primary liver cancer in alpha1-antitrypsin deficiency. N Engl J Med. 1986;314:736-9. CrossrefMedlineGoogle Scholar6. Brantly M, Nukiwa T, and Crystal R. 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Situación actual y nuevas perspectivas para el tratamientoHigh resolution computed tomography (HRCT) in emphysema associated with alpha-1-antitrypsin deficiencyChronic Obstructive Airways Disease 15 December 1989Volume 111, Issue 12 Page: 957-959 Keywords Emphysema Medical services Neutrophils Protease inhibitor therapy Pulmonary diseases Serine proteases Serum proteins ePublished: 1 December 2008 Issue Published: 15 December 1989 PDF downloadLoading ...