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

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.

• Authors: Anna Maria Wasilewska, Elżbieta Kuroczycka-Saniutycz, Walentyna Zoch-Zwierz
• Journal: European journal of pediatrics (Eur J Pediatr) Vol. 170 Issue 3 Pg. 389-91 (Mar 2011) ISSN: 1432-1076 [Electronic] Germany
• PMID: 20848129 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunosuppressive Agents
• Cyclosporine

Topics

• Cyclosporine (therapeutic use)
• Denys-Drash Syndrome (complications, drug therapy, genetics)
• Female
• Genes, Wilms Tumor
• Humans
• Immunosuppressive Agents (therapeutic use)
• Infant
• Mutation
• Nephrotic Syndrome (drug therapy, etiology)
• Remission Induction