Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study |
| |
Authors: | Djalila Mekahli Aurelia Liutkus Bruno Ranchin Anchalee Yu Lucie Bessenay Eric Girardin Rita Van Damme-Lombaerts Jean-Bernard Palcoux François Cachat Marie-Pierre Lavocat Guylhène Bourdat-Michel François Nobili Pierre Cochat |
| |
Institution: | 1. Service de Pédiatrie, Centre de référence des maladies rénales rares, H?pital Femme Mère Enfant and Université de Lyon, Lyon, France 2. Pediatrics, University Hospital of Gasthuisberg, Leuven, Belgium 3. Pediatrics, University Hospital, Clermont-Ferrand, France 4. Pediatrics, University Hospital, Geneva, Switzerland 5. Pediatrics, University Hospital, Lausanne, Switzerland 6. Pediatrics, University Hospital, Saint-Etienne, France 7. Pediatrics, University Hospital, Grenoble, France 8. Pediatrics, University Hospital, Besan?on, France 9. Service de Pédiatrie, H?pital Femme Mère Enfant, 59 boulevard Pinel, 69677, Bron cedex, France
|
| |
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. |
| |
Keywords: | Steroid-resistant nephrotic syndrome End-stage renal disease (ESRD) Child Focal segmental glomerulosclerosis |
本文献已被 SpringerLink 等数据库收录! |
|