MANAGEMENT OF RECURRENT NEPHROTIC SYNDROME AFTER KIDNEY-TRANSPLANTATION IN CHILDREN

Steroid-resistant nephrotic syndrome (NS) with focal glomerulosclerosi s and its recurrence after transplantation (Tx) are mainly seen in chi ldren. The average recurrence rate is 30% and the graft loss is half t his; the risk of recurrent NS in subsequent Tx is 50 to 80% according to the fate of the primary allograft. The immediate appearance of prot einuria after Tx suggests that circulating factor(s) might be present which alter the glomerular permeability. Several therapeutic schedules have been proposed and give conflicting results. However, from the cu rrent literature, a 3-step management should reasonably be settled: 1) preventive measures in patients at risk include bilateral nephrectomy prior to Tx and introduction of intravenous cyclosporine A (target Cy A whole blood level 200 to 250 ng/ml) as early as possible in associat ion with prednisone and azathioprine (+/- anti-thymocyte globulin), 2) in recurrent patients who were not under such a CyA preventive regime , high dose intravenous CyA should be started as soon as possible (tar get CyA whole blood level 250-350 ng/ml), 3) in children who fail to r espond To the above therapeutic proposals, a combination of plasmapher esis followed by substitutive immunoglobulins in association with meth ylprednisolone pulses and cyclophosphamide instead of azathioprine for 2 months should be proposed early.