To define the clinico-pathogenetic importance of alpha 1-inhibitor of proteinases and alpha 2-macroglobulin of the blood in children with glomerulonephritis, a study was made of the phenotype of alpha 1-inhibitor of proteinases and its concentration in the blood serum of 156 patients with different clinical forms of glomerulonephritis. Overall 1290 practically healthy children were examined as control. The patients suffering from glomerulonephritis did not demonstrate phenotypes responsible for acute deficiency of alpha 1-inhibitor of proteinases (PISS, PISZ). A relationship was established between the amount of alpha 1-inhibitor of proteinases in the blood serum in children with different clinical forms of glomerulonephritis: the patients with the nephrotic form manifested a significant decrease of the inhibitor concentration in the blood serum, whereas in the hematuric form, a significant rise of it was recorded. All the patients suffering from glomerulonephritis showed a significant increase of the content of alpha 2-macroglobulin, particularly in the nephrotic form, which is likely to be determined by the enhanced output of the given protein and its negligible loss with urine in connection with a high molecular weight.
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