Medium-Term Outcome of Cyclosporine in Children with Steroid-Dependent Nephrotic Syndrome: Single Centre experience from Pakistan

Abstract

Objective: To determine the medium-term outcome of Cyclosporine in children with steroid-dependent nephrotic syndrome from a single centre in Pakistan Study Design: Prospective longitudinal study Place and Duration of Study: Pediatric Nephrology Department of Sindh Institute of Urology and Transplantation, Karachi Pakistan from Jan 2014 to Dec 2016. Methodology: All consecutive patients with steroid-dependent nephrotic syndrome of either gender, between 1-12 years of age, were identified and included in this study, who relapsed post-Cyclophosphamide therapy and were prescribed Cyclosporine after renal biopsy. Results: Seventy-five children with steroid-dependent nephrotic syndrome met the inclusion criteria. There were 55(74%) boys with a mean age of 4.3 ± 2.5 years, while the mean age at the start of Cyclosporine therapy was 6.5 ± 1.5 years. All patients were previously treated with Cyclophosphamide, and sustained remission was achieved for 37.6 ± 43.6 weeks. All patients underwent renal biopsy showing minimal change disease in 46(61.3%) subjects, focal segmental glomerulosclerosis in 12(16%), IgM nephropathy in 13(17.3%) and mesangioproliferative glomerulonephritis in 4(5.3%) children. At the last follow-up, 36(48%) patients were in complete remission, 26(35%) achieved partial remission, seven children (9.3%) did not achieve remission at all, 2(2.7%) were lost to follow-up, and 4(5.3%) patients expired. The mean estimated glomerular filtration rate at the start of Cyclosporine was 112.55 ml/min/1.73 m2, which declined to 107.77 ml/min/1.73 m2 at three years. Conclusion: In the medium term, Cyclosporine effectively maintains remission and reduces steroid usage with minimal nephrotoxicity in steroid-dependent nephrotic syndrome.

Keywords

• Children
• Cyclosporine
• Nephrotic syndrome
• Steroid-dependent