Alpha-1 Antitrypsin Deficiency, Emphysema, and Cirrhosis in an Adult

  1. KENNETH L. COHEN, M.D.;
  2. PETER E. RUBIN, M.D.;
  3. RENE A. ECHEVARRIA, M.D.;
  4. HARVEY L. SHARP, M.D.; and
  5. PERRY O. TEAGUE, Ph.D.
  1. Gainesville, Florida, and Minneapolis, Minnesota

    Abstract

    The association between alpha-1 antitrypsin deficiency and cirrhosis has been described in children but not in adults. The liver from an adult with homozygous alpha-1 antitrypsin deficiency, emphysema, and cirrhosis was studied with fluorescent antibody specific for alpha-1 antitrypsin. The morphological findings from electron microscopy, fluorescent staining, and staining with periodic acid-Schiff reagent were similar to those found in the livers from children with alpha-1 antitrypsin deficiency and cirrhosis. The areas of greatest cellular damage contained the most alpha-1 antitrypsin. A deficiency of alpha-1 antitrypsin was associated with significant liver disease in this patient. More extensive evaluation of liver function and structure in adult patients deficient in alpha-1 antitrypsin is clearly indicated.

    Article and Author Information

    • ▸From the Departments of Medicine and Pathology, J. Hillis Miller Health Center, College of Medicine, University of Florida, Gainesville, Fla.; and the Department of Pediatrics, University of Minnesota, Minneapolis, Minn.

    • ▸Requests for reprints should be addressed to Perry O. Teague, Ph.D., Department of Pathology, College of Medicine, University of Florida, Gainesville, Fla. 32601.

      • Received April 10, 1972.
      • Accepted August 7, 1972.
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    1. Ann Intern Med February 1, 1973 vol. 78 no. 2 227-232
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