Effect of cyclosporin A on proteinuria in the course of glomerulopathy associated with WT1 mutations

✍ Scribed by Anna Maria Wasilewska; Elżbieta Kuroczycka-Saniutycz; Walentyna Zoch-Zwierz

• Publisher: Springer
• Year: 2010
• Tongue: English
• Weight: 84 KB
• Volume: 170
• Category: Article
• ISSN: 0340-6997
• DOI: 10.1007/s00431-010-1278-4

✦ Synopsis

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.