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  • Title: Effect of cyclosporin A on proteinuria in the course of glomerulopathy associated with WT1 mutations.
    Author: Wasilewska AM, Kuroczycka-Saniutycz E, Zoch-Zwierz W.
    Journal: Eur J Pediatr; 2011 Mar; 170(3):389-91. PubMed ID: 20848129.
    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.
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