Published in:
Open Access
01-03-2011 | Short Report
Effect of cyclosporin A on proteinuria in the course of glomerulopathy associated with WT1 mutations
Authors:
Anna Maria Wasilewska, Elżbieta Kuroczycka-Saniutycz, Walentyna Zoch-Zwierz
Published in:
European Journal of Pediatrics
|
Issue 3/2011
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Abstract
Denys–Drash syndrome (DDS) is characterized by progressive glomerulopathy caused by diffuse mesangial sclerosis (DMS), genitourinary defects, and a higher risk of developing Wilms’ tumor. It is commonly assumed that the DMS is unresponsive to any medications. In this report, we present a patient with Denys–Drash syndrome, in whom the cyclosporine A (CsA) was found to induce total remission. This observation and observations of other authors confirm that in genetic forms of nephrotic syndrome, the proteinuric effect of CsA may be due to a non-immunologic mechanism. We confirm the beneficial effect of CsA treatment in DDS; however, the potential nephrotoxicity of this drug will probably not allow long-term use.