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Table 2 Main clinical and laboratory findings in Z AAT patients

From: Mineralization of alpha-1-antitrypsin inclusion bodies in Mmalton alpha-1-antitrypsin deficiency

Case Age Sex AAT level Liver histology Calcification Genotype
1 22 M 28a cirrhosis Homozygous
2 44 M 82a cirrhosis Homozygous
3 45 M 75a cirrhosis Homozygous
4 56 F 102a Cirrhosis and HCC Compound heterozygous (S/Z)
5c 11 F 125b PSC Heterozygous
6d 17 M 196b Mild CAH Heterozygous
7d 13 M 222b NSRH Heterozygous
  1. aRadial immunodiffusion (n.v. 200–400 mg/dL); bNephelometry (n.v.90–200 mg/dL); cPSC: Primary sclerosing cholangitis; dLiver biopsy obtained during a staging for Hodgkin’s lymphoma; CAH = Chronic Active Hepatitis; NSRH = Non Specific Reactive Hepatitis