Effect of cyclosporin A on proteinuria in the course of glomerulopathy associated with WT1 mutations.
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.
Wasilewska
Anna Maria
AM
Department of Paediatrics and Nephrology, Medical University of Białystok, ul. Waszyngtona 17, 15-274 Białystok, Poland. annwasil@interia.pl
Kuroczycka-Saniutycz
Elżbieta
E
Zoch-Zwierz
Walentyna
W
D016572:Cyclosporine / Q000627:therapeutic use*; D030321:Denys-Drash Syndrome / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D005260:Female; D016162:Genes, Wilms Tumor; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D009154:Mutation; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000209:etiology; D012074:Remission Induction
D007166:Immunosuppressive Agents; D016572:Cyclosporine