We measured plasma α
1-proteinase inhibitor (α
1-PI) levels in patients with idiopathic nephrotic syndrome (INS), chance proteinuria and/or hematuria, and normal controls to examine the roles of proteinase inhibitors in the pathogenesis of renal diseases. Plasma α
1-PI concentrations were significantly decreased in patients with INS at relapses and remissions of INS. The α
1-PI activities, anti-trypsin and anti-elastase activities, were also decreased in relapses of INS. However, the values revealed no statistically signifiant difference in remissions of INS. Significant correlations between the PI activities and α
2-macroglobulin levels were revealed, which suggested a possible contribution of α
2-macroglobulin to the plasma PI activities in INS. From these results we conclude that the decrease in plasma α1-PI activities may be responsible for the reduction of glomerular negative charge, possiblly caused by some unknown proteinase(s), and the resultant development of proteinuria.
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