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Copyright 1971. All rights reserved ALPHAl ANTITRYPSIN DEFICIENCY AND PULMONARY EMPHYSEMA C. A. GUENTER, M.D., M. If. WELCH, M.D., AND]. F. IIAMMARSTEN, M.D. Department of Medicine, University of Oklahoma Medical Center, Oklahoma City, Oklahoma The association of familial alphat antitrypsin deficiency and pulmonary emphysema was first recognized by Laurell & Eriksson ( 1). This observa tion was particularly significant because it identified an important relation ship between a biochemical abnormality and a common but poorly under stood type of lung injury, a relationship that may represent the first docu mented disease resulting from a deficiency of proteinase inhibitors. In this review we have summarized the available information regarding the char acteristics of serum proteinase inhibitors, the incidence and type of pulmo nary disease associated with familial alphal antitrypsin deficiency, and the evidence that pulmonary tissue injury may be related to the deficiency of these inhibitors. PROTEINASE INWBITORS Many naturally occurring proteinase inhibitors in plants and animals have been identified and recently reviewed (2). In some instances such as the soybean, the function of the trypsin inhibitor appears to be incidental; in others, such as the nematode, A sca ris lumbricoides, the inhibitor proper ties may be essential for
Annual Review of Medicine – Annual Reviews
Published: Feb 1, 1971
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