[Decreased plasma alpha 1-proteinase inhibitor activity in idiopathic nephrotic syndrome].
We measured plasma alpha 1-proteinase inhibitor (alpha 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 alpha 1-PI concentrations were significantly decreased in patients with INS at relapses and remissions of INS. The alpha 1-PI activities, anti-trypsin and anti-elastase activities, were also decreased in relapses of INS. However, the values revealed no statistically significant difference in remissions of INS. Significant correlations between the PI activities and alpha 2-macroglobulin levels were revealed, which suggested a possible contribution of alpha 2-macroglobulin to the plasma PI activities in INS. From these results we conclude that the decrease in plasma alpha 1-PI activities may be responsible for the reduction of glomerular negative charge, possibly caused by some unknown proteinase(s), and the resultant development of proteinuria.
Asami
T
T
Nishihara
T
T
Fujii
M
M
Sakai
K
K
D000293:Adolescent; D000328:Adult; D001798:Blood Proteins / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D009404:Nephrotic Syndrome / Q000097:blood / Q000209:etiology*; D011507:Proteinuria / Q000209:etiology; D000515:alpha 1-Antitrypsin
D001798:Blood Proteins; D000515:alpha 1-Antitrypsin