Single-Center Experience With Cyclosporine for Treatment of Idiopathic Minimal Change Nephrotic Syndrome in Children

Abstract

Introduction. Cyclosporine A is used in the treatment of idiopathic nephrotic syndrome. We conducted this study to evaluate the effect of cyclosporine and its combination with ketoconazole in Egyptian nephrotic children with steroid-resistant and steroid-dependant minimal change.

Materials and Methods. Forty-eight children with minimal change lesions who received cyclosporine with or without ketoconazole were studied. Their mean age was 5.17 ± 1.59 years, and they were 31 boys and 17 girls. The mean duration of the disease was 6.22 ± 3.16 years. Thirty-one of the children were steroid dependent and 17 were steroid resistant. Cyclosporine treatment was commenced after remission was attained and adjusted to a target trough level of 100 ng/mL. The mean cyclosporine therapy at a dose of 2.07 ± 0.91 mg/kg was administered for a mean of 25.75 ± 1.95 months. Thirty-three patients received adjunctive ketoconazole therapy.

Results. Thirty-eight patients (79.2%) responded well to cyclosporine. Steroid therapy could be discontinued in 43 patients (89.6%), but 9 experienced relapse. Ten patients (20.8%) were resistant to cyclosporine therapy. Fifteen patients received cyclosporine alone, while 33 received concomitant cyclosporine and ketoconazole. The response to cyclosporine was significantly better in those on ketoconazole. The economic effect of ketoconazole therapy was a reduction in the costs of cyclosporine treatment by 47.4% at 1 year of treatment.

Conclusions. Cyclosporine treatment in children with minimal change nephrotic syndrome is effective in preventing relapse and decreasing steroid toxicity. Its combination with low-dose ketoconazole is safe, reduces treatment costs, and improves the response to cyclosporine.