Table 2.
Main clinical and laboratory findings in Z AAT patients
| 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 |
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