Abstract
Examination of hepatic tissue obtained at autopsy (13 cases) and by biopsy (one case) from patients with homozygous alpha1-antitrypsin deficiency showed that the hepatocytes contained periodic-acid-Schiff-positive globular material similar to that seen in infantile cirrhosis with alpha1-antitrypsin deficiency. Five of the adult patients had cirrhosis, and three fibrosis of the liver; of these eight, all over 50 years of age, three also had hepatomas. Twelve of the 13 autopsy cases had either bullous or panacinar emphysema. The findings suggest that in alpha1-antitrypsin deficiency liver disease and emphysema are linked to the same genetic defect.
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