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

Mekahli, Djalila; Liutkus, Aurelia; Ranchin, Bruno; Yu, Anchalee; Bessenay, Lucie; Girardin, Eric; Van Damme-Lombaerts, Rita; Palcoux, Jean-Bernard; Cachat, François; Lavocat, Marie-Pierre; Bourdat-Michel, Guylhene; Nobili, François; Cochat, Pierre

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Title		Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study
Authors		Mekahli, Djalila Liutkus, Aurelia Ranchin, Bruno Yu, Anchalee Bessenay, Lucie Girardin, Eric Van Damme-Lombaerts, Rita Palcoux, Jean-Bernard Cachat, François Lavocat, Marie-Pierre Bourdat-Michel, Guylhene Nobili, François Cochat, Pierre show all authors [1 - 13]
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		Adolescent — Child — Child, Preschool — Cyclophosphamide/therapeutic use — Cyclosporine/therapeutic use — Drug Resistance — Female — Humans — Immunosuppressive Agents/therapeutic use — Infant — Male — Mycophenolic Acid/analogs & derivatives/therapeutic use — Nephrotic Syndrome/*drug therapy — Retrospective Studies — Steroids/therapeutic use — Time Factors — Treatment Outcome
Identifiers		DOI: 10.1007/s00467-009-1138-5 PMID: 19280229
Full text		Article - Limited access to UNIGE Other version: http://www.springerlink.com/content/yvm0777p44030614/fulltext.pdf
Structures		Faculté de médecine / Section de médecine clinique / Département de pédiatrie
Research group		Pathogénèse du syndrome néphrotique idiopathique de l'enfant (180)
Citation (ISO format)		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. https://archive-ouverte.unige.ch/unige:19951