HLA in Juvenile Liver Disease with Alpha‐one‐Antitrypsin Deficiency

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Abstract
The possible role of HLA phenotypes was investigated in the development of juvenile liver disease in persons with α1‐antitrypsin deficiency. Seventeen patients were investigated between the ages of 3–25 years. All of them had α1‐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 α1‐antitrypsin deficiency. The family studies confirmed these findings.