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

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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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Acknowledgments

This article was presented in abstract form at the American Society of Nephrology meeting in November 2007.

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Authors and Affiliations

  1. Service de Pédiatrie, Centre de référence des maladies rénales rares, Hôpital Femme Mère Enfant and Université de Lyon, Lyon, France

    Djalila Mekahli, Aurelia Liutkus, Bruno Ranchin & Pierre Cochat

  2. Pediatrics, University Hospital of Gasthuisberg, Leuven, Belgium

    Djalila Mekahli, Anchalee Yu & Rita Van Damme-Lombaerts

  3. Pediatrics, University Hospital, Clermont-Ferrand, France

    Lucie Bessenay & Jean-Bernard Palcoux

  4. Pediatrics, University Hospital, Geneva, Switzerland

    Eric Girardin

  5. Pediatrics, University Hospital, Lausanne, Switzerland

    François Cachat

  6. Pediatrics, University Hospital, Saint-Etienne, France

    Marie-Pierre Lavocat

  7. Pediatrics, University Hospital, Grenoble, France

    Guylhène Bourdat-Michel

  8. Pediatrics, University Hospital, Besançon, France

    François Nobili

  9. Service de Pédiatrie, Hôpital Femme Mère Enfant, 59 boulevard Pinel, 69677, Bron cedex, France

    Pierre Cochat

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  1. Djalila Mekahli
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  13. Pierre Cochat
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Correspondence to Pierre Cochat.

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Mekahli, D., Liutkus, A., Ranchin, B. et al. Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study. Pediatr Nephrol 24, 1525–1532 (2009). https://doi.org/10.1007/s00467-009-1138-5

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  • DOI: https://doi.org/10.1007/s00467-009-1138-5

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