Idiopathic nephrotic syndrome - Deflazacort, an alternative?

Background: Prednisolone has been the first-line therapy in the treatment of idiopathic nephrotic syndrome in children. Recurrences are common, and the need for repeated high doses of prednisolone alone or in combination with other immunosuppressants may induce significant side effects. Deflazacort has been used in recent years as an alternative, but there are not many studies on its effectiveness in maintenance therapy. The aim of this study was to assess the efficacy and safety of deflazacort maintenance therapy in childhood idiopathic nephrotic syndrome. Methods: Retrospective observational review of patients with steroid-dependent idiopathic nephrotic syndrome who had received previous treatment with prednisolone and were under deflazacort for at least a year. Steroid-dependent idiopathic nephrotic syndrome was defined when relapses occurred during the reduction of corticosteroid therapy or up to two weeks after its withdrawal. We compared the number of relapses, steroid dose at relapse, the period of time without recurrence and side effects in the twelve months of therapy before and after the introduction of deflazacort. Results: We included 20 patients, 75% were male. At diagnosis of idiopathic nephrotic syndrome, the median age was 3.4 years. All children had begun treatment with prednisolone, which was replaced by deflazacort on average 2.7 years after diagnosis. After the first year of therapy with deflazacort, we found a significant reduction in the number of relapses (mean 0.5 vs. 2.0; p < 0.001) and the side effects of corticosteroids (p = 0.037). There were no significant differences in other variables. Conclusions: In this sample, deflazacort was associated with a small number of side effects and relapses, as compared to prednisolone, proving to be a safe and effective therapy in the maintenance treatment of idiopathic nephrotic syndrome in children

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Bibliographic Details
Main Authors: Neves,Catarina, Cordinhã,Ana Carolina, Ferreira,Carmen, Gomes,Clara, Correia,António Jorge
Format: Digital revista
Language:English
Published: Sociedade Portuguesa de Nefrologia 2016
Online Access:http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000100005
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Summary:Background: Prednisolone has been the first-line therapy in the treatment of idiopathic nephrotic syndrome in children. Recurrences are common, and the need for repeated high doses of prednisolone alone or in combination with other immunosuppressants may induce significant side effects. Deflazacort has been used in recent years as an alternative, but there are not many studies on its effectiveness in maintenance therapy. The aim of this study was to assess the efficacy and safety of deflazacort maintenance therapy in childhood idiopathic nephrotic syndrome. Methods: Retrospective observational review of patients with steroid-dependent idiopathic nephrotic syndrome who had received previous treatment with prednisolone and were under deflazacort for at least a year. Steroid-dependent idiopathic nephrotic syndrome was defined when relapses occurred during the reduction of corticosteroid therapy or up to two weeks after its withdrawal. We compared the number of relapses, steroid dose at relapse, the period of time without recurrence and side effects in the twelve months of therapy before and after the introduction of deflazacort. Results: We included 20 patients, 75% were male. At diagnosis of idiopathic nephrotic syndrome, the median age was 3.4 years. All children had begun treatment with prednisolone, which was replaced by deflazacort on average 2.7 years after diagnosis. After the first year of therapy with deflazacort, we found a significant reduction in the number of relapses (mean 0.5 vs. 2.0; p < 0.001) and the side effects of corticosteroids (p = 0.037). There were no significant differences in other variables. Conclusions: In this sample, deflazacort was associated with a small number of side effects and relapses, as compared to prednisolone, proving to be a safe and effective therapy in the maintenance treatment of idiopathic nephrotic syndrome in children