Clinical Course of Steroid Sensitive Nephrotic Syndrome in Children: Outcome and Outlook

ABSTRACT

Introduction: The aim of our study was to investigate the relative efficiency and adverse effects of various treatments of steroid sensitive nephrotic syndrome (SSNS) in children, and to determine factors associated with relapse risk in these patients.

Materials and Method: We retrospectively studied the data from 690 SSNS children treated in referral center over 25 years. The analyzed treatment protocols were: Prednisolone (PRED, eight weeks in a dose 1.5-2.0 mg/kg, then it tapering and given for 9-12 months), Chlorambucil (CHL, cumulative dose 28.5-30 mg/kg), Cyclophosphamide intravenously (CYC I.V., cumulative dose of 30-36 mg/kg, then supporting dose of CHL, cumulative dose of 20-25 mg/kg) and intramuscular (CYC I.M., cumulative dose of 120-150 mg/kg). The alkylating agents were used after remission induction by PRED and under its protection.

Results: Cumulative relapse-free survival was 81.9%, 69.0% and 64.5% after 12, 36 and 60 months, respectively. In multivariate analyses, relapse risk was associated with age of treatment (<6 years), and both PRED and CYC I.V. The only predictive factor for early relapse was PRED, unlike two and more relapses group where PRED and CYC I.V. as well as age from 3 to 6 years was highly prognostic. The high probability of sustained remission in combination with relatively mild adverse effects was observed for PRED used at first episode and CHL used at relapse.

Conclusion: To summarize, our protocols characterized by the prolonged PRED and CHL demonstrated promising results and should be considered as an efficient alternative strategy in SSNS management.