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An Overview of the Pulmonary Features of  1-Antitrypsin Deficiency
Martin J. Tobin, MB, MRCPI;
Duncan C. S. Hutchison, BM, FRCPG
Arch Intern Med. 1982;142(7):1342-1348.
Abstract
• Extensive research, stimulated by the recognition of an association between  1-antitrypsin (1-AT) deficiency and emphysema, has greatly advanced our understanding of emphysema in general. In this article, we review the literature concerning the basic defect, inheritance, pathogenesis of lung disease, clinical, physiologic, and roentgenographic findings in patients with severe (Pi Z) and intermediate (Pi SZ) deficiency of 1-AT. Data obtained in relatives with 1-AT deficiency, who have not been seen by a physician, should more truly reflect the risk of having lung disease. These studies confirm the increased risk of developing lung disease in cigarette smokers. The implications of the finding that subjects with Pi SZ are probably not at an increased risk of lung disease is discussed with regards to replacement therapy. The natural history of unselected subjects with 1-AT deficiency remains unknown.
(Arch Intern Med 1982;142:1342-1348)
Author Affiliations
From the Division of Pulmonary Disease, Mount Sinai Medical Center, Miami Beach, Fla (Dr Tobin); and the Chest Unit, Kings College Hospital Medical School, London (Dr Hutchison).
Footnotes
Accepted for publication March 11, 1982.
Reprints not available.
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