Immunosuppression and renal outcome in congenital and pediatric steroid-resistant nephrotic syndrome.

  • Anja K Büscher
  • Birgitta Kranz
  • Rainer Büscher
  • Friedhelm Hildebrandt
  • Bernd Dworniczak
  • Petra Pennekamp
  • Eberhard Kuwertz-Bröking
  • Anne-Margret Wingen
  • Ulrike John
  • Markus J. Kemper
  • Leo Monnens
  • Peter F Hoyer
  • Stefanie Weber
  • Martin Konrad

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Abstract

Mutations in podocyte genes are associated with steroid-resistant nephrotic syndrome (SRNS), mostly affecting younger age groups. To date, it is unclear whether these patients benefit from intensified immunosuppression with cyclosporine A (CsA). The aim of this study was to evaluate the influence of podocyte gene defects in congenital nephrotic syndrome (CNS) and pediatric SRNS on the efficacy of CsA therapy and preservation of renal function. Design, settings, participants, ; measurements: Genotyping was performed in 91 CNS/SRNS patients, irrespective of age at manifestation or response to CsA.

Bibliographical data

Original languageGerman
Article number11
ISSN1555-9041
Publication statusPublished - 2010
pubmed 20798252