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Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study

Mekahli, Djalila
Liutkus, Aurelia
Ranchin, Bruno
Yu, Anchalee
Bessenay, Lucie
Van Damme-Lombaerts, Rita
Palcoux, Jean-Bernard
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Published in Pediatric nephrology. 2009, vol. 24, no. 8, p. 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.
Keywords AdolescentChildChild, PreschoolCyclophosphamide/*therapeutic useCyclosporine/*therapeutic useDrug ResistanceFemaleHumansImmunosuppressive Agents/*therapeutic useInfantMaleMycophenolic Acid/*analogs & derivatives/therapeutic useNephrotic Syndrome/*drug therapyRetrospective StudiesSteroids/therapeutic useTime FactorsTreatment Outcome
PMID: 19280229
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Research group Pathogénèse du syndrome néphrotique idiopathique de l'enfant (180)
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MEKAHLI, Djalila et al. Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study. In: Pediatric nephrology, 2009, vol. 24, n° 8, p. 1525-1532. doi: 10.1007/s00467-009-1138-5 https://archive-ouverte.unige.ch/unige:19951

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Deposited on : 2012-04-23

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