Children with Steroid-resistant Nephrotic Syndrome: a Single-Center Study

Authors

1 Antalya Research and Education Hospital, Pediatric Nephrology Department, Antalya, Turkey.

2 Ankara Dr.Sami Ulus Research and Education Hospital, Pediatric Nephrology Department, Ankara, Turkey.

Abstract

Background and Aim: Steroid-resistant nephrotic syndrome (SRNS) accounts for 10%-20% of all cases of idiopathic nephrotic syndrome. These patients are at risk of developing end-stage renal disease. The aim of this study was to determine the demographic characteristics, renal biopsy findings, response to immunosuppressive treatment, and prognosis in pediatric patients with SRNS.
Materials and Methods: This retrospective study included 31 patients diagnosed as primary SRNS. Age at first episode, gender, parental consanguinity, and familial history of nephrotic syndrome were recorded. Demographic characteristics, renal biopsy findings, response to immunosuppressive treatment, and prognosis were analyzed, as were the number of and treatment of relapses, extra-renal manifestations, and complications of disease and treatment.
Results: Mean age at first episode of nephrotic syndrome was 4,1±2,9 years. At the end of the first immunosuppressive treatment cycle, 14 (51.8%) patients achieved complete remission, 4 (14.8%) patients achieved partial remission, and 9 patients (33.3%) did not achieve remission. Analysis of the final status of the patients showed that 16 patients (51.6%) developed remission, 5 patients (16%) continued to have nephrotic range proteinuria and 10 patients (32%) developed chronic renal failure (CRF).
Conclusion: The treatment of SRNS remains controversial. Early genetic testing can help the inevitable immunosuppressive treatments which may not be effective and have several side effects. Calcineurin inhibitors and mycophenolate mofetil are known to be effective immunosuppressive drugs for treating steroid resistant nephrotic syndrome .

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