Chronic Obstructive Pulmonary Disease and Heterozygous Alpha_1-Antitrypsin Deficiency
Authors: GEORGE A. FALK
Journal: Annals of Internal Medicine (1970)

Editorial Notes1 April 1970Chronic Obstructive Pulmonary Disease and Heterozygous Alpha1-Antitrypsin DeficiencyGEORGE A. FALK, M.D., WILLIAM A. BRISCOE, M.D.GEORGE A. FALK, M.D.Search for more papers by this author, WILLIAM A. BRISCOE, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-72-4-595 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptInterest in alpha1-antitrypsin has been stimulated by finding a high prevalence of chronic obstructive pulmonary disease in patients deficient in this normally occurring serum protein (1). Family studies suggest that the structure and quantity of alpha1-antitrypsin are determined by at least seven codominant alleles (2). These genes have been designated as the Pi (proteinase inhibitor) system. The majority of normal persons have been identified as homozygous for the major Pi type (PiM). Of the less frequent Pi types onlyPiZin the homozygous state (ZZ) has until recently been associated with a predisposition to the disease. Alpha1-antitrypsin constitutes the greater...References1. ERIKSSON S: Studies in α1-antitrypsin deficiency. Acta Med Scand 432 (suppl) :1-85, 1965 Google Scholar2. FAGERHOLTENFJORD MO: Serum Pi types in some European, American, Asian and African populations. Acta Path Microbiol Scand 72:601-608, 1968 CrossrefMedlineGoogle Scholar3. GANROTLAURELLERIKSSON PCS: Obstructive lung disease and trypsin inhibitors in α1-antitrypsin deficiency. Scan J Clin Lab Invest 19:205-208, 1967 CrossrefMedlineGoogle Scholar4. KUEPPERS F: Immunologic assay of alpha1-antitrypsin in deficient subjects and their families. Humangenetik 5:54-58, 1967 MedlineGoogle Scholar5. TALAMOALLENKAHAN RJM: Hereditary alpha1-antitrypsin deficiency. New Eng J Med 278:345-351, 1968 CrossrefMedlineGoogle Scholar6. LAURELL C: Antigen-antibody crossed electrophoresis. Anal Biochem 10:358-361, 1965 CrossrefMedlineGoogle Scholar7. KUEPPERSFALLATLARSON FRR: Obstructive lung disease and α1-antitrypsin deficiency gene heterozygosity. Science 165:899-901, 1969 CrossrefMedlineGoogle Scholar8. LIEBERMAN J: Heterozygous and homozygous alpha1-antitrypsin deficiency in patients with pulmonary emphysema. New Eng J Med 281:279-284, 1969 CrossrefMedlineGoogle Scholar9. BRISCOEKUEPPERSDAVIS WFA: A case of inherited deficiency of serum alpha1-antitrypsin associated with pulmonary emphysema. Amer Rev Resp Dis 94:529-539, 1966 MedlineGoogle Scholar10. TARKOFFKUEPPERSMILLER MFW: Pulmonary emphysema and alpha1-antitrypsin deficiency. Amer J Med 45:220-228, 1968 CrossrefMedlineGoogle Scholar11. HEPPERBLACKGLEICH HLG: The prevalence of alpha1-antitrypsin deficiency in selected groups of patients with chronic obstructive lung disease. Mayo Clin Proc 44: 697-710, 1969 MedlineGoogle Scholar12. RICHARDSONGUENTERWELCH RCM: The pattern of inheritance of alpha1-antitrypsin deficiency and associated pulmonary disease. Amer Rev Resp Dis 100:619-625, 1969 MedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: The New York Hospital-Cornell University Medical College New York, N.Y. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited ByAlpha-1-AntitrypsinMechanism of Action of α-1-AntitrypsinDie Trypsinhemmung in unausgewählten Sera eines poliklinischen PatientengutesCirrhosis Associated with Partial Deficiency of Alpha-1 Antitrypsin in an AdultJOSE L. CAMPRA, M.D., JOHN R. CRAIG, M.D., Ph.D., ROBERT L. PETERS, M.D., TELFER B. REYNOLDS, M.D., F.A.C.P.Pulmonary Function in Alpha,-Antitrypsin Deficiency 1 April 1970Volume 72, Issue 4Page: 595-596KeywordsAllelesChronic obstructive pulmonary diseaseProteinsSerum proteins Issue Published: 1 April 1970 PDF DownloadLoading ...