HLA in juvenile liver disease with alpha-one-antitrypsin deficiency

The possible role of HLA phenotypes was investigated in the development of juvenile liver disease in persons with alpha 1-antitrypsin deficiency. Seventeen patients were investigated between the ages of 3-25 years. All of them had alpha 1-antitrypsin phenotype PiZ. After a longer follow-up a clinical diagnosis was established by the help of physical status, biochemical liver function tests and--in the cases of suspected liver disease--liver biopsy. The clinical course was correlated to the HLA phenotypes of the patients. In 2 cases all first degree relatives were investigated, as well. Our studies on the 17 unrelated patients indicated no correlation between HLA and juvenile liver disease in alpha 1-antitrypsin deficiency. The family studies confirmed these findings.