Pulmonary Disease in Alpha-1-Antitrypsin Deficiency (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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Snider, MDAffiliations: Boston University Tufts University Schools of Medicine VA Medical Center Boston, Massachusetts Nextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited bySpecific ProteinsSmoking and Lung DiseasePurification and partial characterization of feline α1-proteinase inhibitor (fα1-PI) and the development and validation of a radioimmunoassay for the measurement of fα1-PI in serumSmoking and Lung DiseaseFormoterol Monotherapy Compared with Combined Ipratropium Bromide plus Fenoterol in the Treatment of Chronic Obstructive Pulmonary DiseaseNormal Diffusing Capacity in Patients With PiZ α1-Antitrypsin Deficiency, Severe Airflow Obstruction, and Significant Radiographic EmphysemaOffice Spirometry for Lung Health Assessment in AdultsEstado actual del tratamiento sustitutivo en el enfisema congénito por déficit de alfa-1-antitripsina. 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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 ...