KENNETH L. COHEN, M.D.; PETER E. RUBIN, M.D.; RENE A. ECHEVARRIA, M.D.; HARVEY L. SHARP, M.D.; PERRY O. TEAGUE, Ph.D.
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.
COHEN KL, RUBIN PE, ECHEVARRIA RA, et al. Alpha-1 Antitrypsin Deficiency, Emphysema, and Cirrhosis in an Adult. Ann Intern Med. 1973;78:227–232. doi: https://doi.org/10.7326/0003-4819-78-2-227
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Published: Ann Intern Med. 1973;78(2):227-232.
Gastroenterology/Hepatology, Liver Disease.
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