Immunosuppression and Renal Outcome in Congenital and Pediatric Steroid-Resistant Nephrotic Syndrome
Author(s) -
Anja Büscher,
Birgitta Kranz,
Rainer Büscher,
Friedhelm Hildebrandt,
Bernd Dworniczak,
Petra Pennekamp,
Eberhard Kuwertz-Bröking,
AnneMargret Wingen,
Ulrike John,
Markus J. Kemper,
L.A.H. Monnens,
Peter F. Hoyer,
Stefanie Weber,
Martin Konrad
Publication year - 2010
Publication title -
clinical journal of the american society of nephrology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.755
H-Index - 151
eISSN - 1555-905X
pISSN - 1555-9041
DOI - 10.2215/cjn.01190210
Subject(s) - medicine , nephrotic syndrome , immunosuppression , renal function , population , genotyping , focal segmental glomerulosclerosis , congenital nephrotic syndrome , pediatrics , gastroenterology , glomerulonephritis , kidney , genotype , proteinuria , gene , genetics , biology , environmental health
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.
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