HETEROZYGOUS MZ ALPHA-1-ANTITRYPSIN DEFICIENCY IN ADULTS WITH CHRONIC LIVER-DISEASE

Pi phenotype was determined in 335 patients with liver diseases and compared with the results in 2830 healthy blood donors. Eleven of 335 patients had phenotype MZ (3.3% compared with 2.9% in healthy blood donors (NS)). None of 53 patients with autoimmune chronic active hepatitis had the MZ phenotype, but it was found in 2 of 18 patients (11.1% with cryptogenic cirrhosis, 3 of 78 (3.8% with alcoholic liver cirrhosis, 2 of 36 (5.6% with primary sclerosing cholangitis, and 1 of 26 (3.9% with primary biliary cirrhosis. Altogether, 3 of 335 patients were homozygous for Pi ZZ and had cirrhosis. One of them (a male) developed a hepatoma and died. We conclude that the reported association between Pi MZ phenotype and chronic non-B active hepatitis does not seem to include patients with autoimmune chronic active hepatitis, whereas the possibility of an association between cryptogenic cirrhosis and the MZ phenotype cannot be excluded. © 1990 Informa UK Ltd All rights reserved: reproduction in whole or part not permitted.