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Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study
Djalila Mekahli1, Aurelia Liutkus, Bruno Ranchin
1Service de Pédiatrie, Centre de référence des maladies rénales rares, Hôpital Femme Mère Enfant and Université de Lyon, Lyon, France.
Insights
Long-term outcomes for pediatric idiopathic steroid-resistant nephrotic syndrome (SRNS) show improved kidney survival due to better management. Age at onset over 10 years predicts end-stage renal disease, highlighting the need for further research.
Area of Science:
- Pediatric Nephrology
- Immunology
- Renal Medicine
Background:
- Idiopathic steroid-resistant nephrotic syndrome (SRNS) presents a significant challenge in pediatric nephrology.
- Long-term outcomes and prognostic factors for SRNS remain critical areas for investigation.
Purpose of the Study:
- To retrospectively analyze the long-term outcomes of pediatric idiopathic SRNS.
- To identify predictors of end-stage renal disease (ESRD) and evaluate treatment strategies.
Main Methods:
- Retrospective study of 78 children with idiopathic SRNS across eight centers over 20 years.
- Analysis of patient demographics, treatment strategies (e.g., ciclosporine, mycophenolate mofetil), and long-term outcomes including renal survival and patient survival.
- Cox regression analysis was used to identify independent predictors of ESRD.
Main Results:
- Actuarial patient survival after 15 years was 97%, with renal survival rates of 75% (5 years), 58% (10 years), and 53% (15 years).
- Age at onset > 10 years was the sole independent predictor of ESRD (P < 0.001).
- Twenty-six percent of patients received transplants, with a 40% recurrence rate among them.
Conclusions:
- Kidney survival in pediatric idiopathic SRNS has improved over the past two decades due to enhanced management strategies.
- Early identification of risk factors, such as age at onset, is crucial for predicting disease progression.
- Further prospective trials are needed to evaluate novel immunosuppressive treatments for SRNS.
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.
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