| Abstract: | In order to examine the frequency of alpha-1-antitrypsin (AAT) deficiency of phenotype Pi-Z in a consecutive liver biopsy material, PAS/diastase resistent globules with positive immunohistochemical reaction for AAT (AAT globules) were used as a marker of the Pi-Z gene. 34 (4%) of 850 liver biopsies contained AAT globules. More than half of the biopsies with globules had chief histological diagnoses within the groups fibrosis, suspicion of cirrhosis and cirrhosis. Micronodular cirrhosis was significantly more frequent in biopsies with AAT globules. The results support the assumption that AAT deficiency of phenotype Pi-Z as well in homozygous as heterozygous form is associated with development of liver cirrhosis. |
