Cirrhosis Associated with Partial Deficiency of Alpha-1 Antitrypsin in an Adult

A 63-year-old woman developed ascites that led to a diagnosis of cryptogenic cirrhosis. Eosinophilic cytoplasmic inclusions were found in hepatocytes on examination of liver biopsy specimens and were shown by immunofluorescent staining to be deposits of alpha-1 antitrypsin. She had a partial deficiency of serum alpha-1 antitrypsin, with a SZ phenotype for the Pi (protease inhibitor) system. Severe, homozygous alpha-1 antitrypsin deficiency is known to be associated with juvenile cirrhosis; we propose that a partial deficiency may be related to liver disease in adults.