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(Chest. 1996;110:237S-242S.)
© 1996 American College of Chest Physicians

A 30-Year Perspective on agr1-Antitrypsin Deficiency

Sten Eriksson MD, PhD1

1 From the Department of Medicine, University of Lund, University Hospital of Malmö, Malmö, Sweden

The early history of agr1-antitrypsin deficiency, in the years between 1962 and 1965, and its impact on the understanding of emphysema abnormalities were reviewed by me in 1989. This report discusses the disease spectrum (including emphysema, liver, and vasculitic diseases) and focuses on the variable clinical expression of the deficiency state. Some new findings that may be relevant to the function of agr1-antitrypsin are also considered.

Key Words: agr1-antitrypsin deficiency • emphysema • endoplasmic reticulum storage diseases • serpins • vasculitic syndromes






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Copyright © 1996 by the American College of Chest Physicians.