Trends in the treatment of idiopathic nephrotic syndrome in children based on a single-centre experience

Abstract

Aim: The aim of the study was to perform a retrospective analysis of treatment modalities in children with idiopathic nephrotic syndrome, types of responses to the therapies used and adverse effects induced by the drugs based on a singlecentre experience. Material and methods: Medical histories of 146 patients (82 boys and 64 girls) diagnosed with idiopathic nephrotic syndrome and hospitalised in the Clinic of Paediatric Nephrology, Medical University of Wroclaw, between 2012 and 2016 were included in the analysis. Results: Remission was achieved in 83.6% of patients treated due to the first episode of idiopathic nephrotic syndrome, whereas primary steroid resistance was diagnosed in 16.4% of cases. During the treatment of subsequent disease recurrences, 52% of children developed steroid dependence, while secondary steroid resistance occurred in 8% of patients. Cyclosporine A was the most common second-line regimen after steroids. Alkylating agents were used as second-line therapy in 1/3 of children; however, they were not used in any of the patients at the end of the follow-up period. Although mycophenolate mofetil was used as a second-line drug in only 3 patients, it was the most common therapeutic option for subsequent relapses. Adverse effects were observed in 50.5% of children receiving steroid therapy and 58.8% of patients receiving cyclosporine A. Adverse reactions were observed in less than 1% of patients receiving alkylating agents and mycophenolate mofetil. Conclusions: Cyclosporine A was the most widely used and a safe second-line drug in the treatment of paediatric idiopathic nephrotic syndrome. Mycophenolate mofetil became an important therapeutic option and an effective alternative for cyclosporine A in the therapy of steroid-dependent idiopathic nephrotic syndrome. Appropriate selection and monitoring of therapy with new generation drugs allowed to avoid irreversible complications in most cases.

Keywords

• cyclosporine A
• alkylating agents
• mycophenolate mofetil
• steroid dependence
• steroid resistance