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HLA in Juvenile Liver Disease with Alpha‐one‐Antitrypsin Deficiency
Author(s) -
NEMETH ANTAL,
MÖLLER ERNA
Publication year - 1987
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1987.tb10528.x
Subject(s) - medicine , alpha 1 antitrypsin deficiency , liver disease , liver biopsy , phenotype , juvenile , human leukocyte antigen , disease , gastroenterology , liver function tests , clinical phenotype , pathology , biopsy , immunology , antigen , genetics , gene , biology
. The possible role of HLA phenotypes was investigated in the development of juvenile liver disease in persons with α 1 ‐antitrypsin deficiency. Seventeen patients were investigated between the ages of 3–25 years. All of them had α 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 α 1 ‐antitrypsin deficiency. The family studies confirmed these findings.