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


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2009-08

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

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. More... »

PAGES

1525-1532

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  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1007/s00467-009-1138-5

    DOI

    http://dx.doi.org/10.1007/s00467-009-1138-5

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1048800371

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/19280229


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