Title: Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study
Authors: Mekahli, Djalila ×
Liutkus, Aurelia
Ranchin, Bruno
Yu, Anchalee
Bessenay, Lucie
Girardin, Eric
Lombaerts, Rita
Palcoux, Jean-Bernard
Cachat, François
Lavocat, Marie-Pierre
Bourdat-Michel, Guylhène
Nobili, François
Cochat, Pierre #
Issue Date: Aug-2009
Publisher: Springer International
Series Title: Pediatric Nephrology vol:24 issue:8 pages:1525-1532
Abstract: Long-term outcome of idiopathic steroid-resistant nephrotic syndrome was retrospectively studied in 78 children in eight centers for the past 20 years. Median age at onset was 4.4 years (1.1-15.0 years) and the gender ratio was 1.4. Median follow-up period was 7.7 years (1.0-19.7 years). The disease in 45 patients (58%) was initially not steroid-responsive and in 33 (42%) it was later non-responsive. The main therapeutic strategies included administration of ciclosporine (CsA) alone (n = 29; 37%) and CsA + mycophenolate mofetil (n = 18; 23%). Actuarial patient survival rate after 15 years was 97%. Renal survival rate after 5 years, 10 years and 15 years was 75%, 58% and 53%, respectively. An age at onset of nephrotic syndrome (NS) > 10 years was the only independent predictor of end-stage renal disease (ESRD) in a multivariate analysis using a Cox regression model (P < 0.001). Twenty patients (26%) received transplants; ten showed recurrence of the NS: seven within 2 days, one within 2 weeks, and two within 3-5 months. Seven patients lost their grafts, four from recurrence. Owing to better management, kidney survival in idiopathic steroid-resistant nephrotic syndrome (SRNS) has improved during the past 20 years. Further prospective controlled trials will delineate the potential benefit of new immunosuppressive treatment.
ISSN: 0931-041X
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Pediatric Nephrology Section (-)
Laboratory of Molecular and Cellular Signaling
× corresponding author
# (joint) last author

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