Effect of cyclosporin A on proteinuria in the course of glomerulopathy associated with WT1 mutations
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Anna Maria Wasilewska & El؟bieta Kuroczycka-Saniutycz & Walentyna Zoch-Zwierz
- چاپ و سال / کشور: 2011
Description
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.
Eur J Pediatr (2011) 170:389–391 DOI 10.1007/s00431-010-1278-4 Received: 28 June 2010 / Accepted: 11 August 2010 / Published online: 17 September 2010
