Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study.
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.
Mekahli
Djalila
D
Service de Pédiatrie, Centre de référence des maladies rénales rares, Hôpital Femme Mère Enfant and Université de Lyon, Lyon, France.
Liutkus
Aurelia
A
Ranchin
Bruno
B
Yu
Anchalee
A
Bessenay
Lucie
L
Girardin
Eric
E
Van Damme-Lombaerts
Rita
R
Palcoux
Jean-Bernard
JB
Cachat
François
F
Lavocat
Marie-Pierre
MP
Bourdat-Michel
Guylhène
G
Nobili
François
F
Cochat
Pierre
P
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use*; D016572:Cyclosporine / Q000627:therapeutic use*; D004351:Drug Resistance; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D009404:Nephrotic Syndrome / Q000188:drug therapy*; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use; D013997:Time Factors; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D013256:Steroids; D016572:Cyclosporine; D003520:Cyclophosphamide; D009173:Mycophenolic Acid