COAGULOPATHY OF CHILDHOOD NEPHROTIC SYNDROME - A REAPPRAISAL OF THE ROLE OF NATURAL ANTICOAGULANTS AND FIBRINOLYSIS

In an attempt to characterise further the coagulopathy of childhood ne phrotic syndrome, this study concentrates on simultaneous measurements of the natural anticoagulants [antithrombin III (ATIII), proteins C a nd S] and the fibrinolytic factors, tissue plasminogen activator (tPA) and plasminogen activator inhibitor (PAI). The study groups consisted of 41. children (ages ranging from 2 to 14 years; median 7.1) in the relapse of nephrosis and 48 children (ages ranging from 3 to 14 years; median 7.6) in remission. The results obtained were compared with nor mal values obtained in healthy age- and sex-matched controls (n = 103) . During relapse, there was a marked increase in the plasma level of f ibrinogen, protein C, and protein S and reduced plasma ATIII level; tP A level was similar to control but PAI level exhibited a significant r eduction. During remission, the protein C level either remained elevat ed or increased further, but some decreased. Protein S and plasma ATII I level normalised. The fibrinolytic activator tPA dropped slightly bu t the PAI level remained significantly below control levels. We conclu de that in the relapse of childhood nephrosis, despite the existence o f a significant prothrombotic tendency as featured by hyperfibrinogena emia and markedly reduced ATIII level, the simultaneous elevation of t he natural anticoagulant, protein C level and enhanced fibrinolysis th at persist until the remission phase, seem to be major preventive mech anisms guarding nephrotic children against thromboembolic phenomena.