Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium.
BACKGROUND: Idiopathic nephrotic syndrome (NS) in children is classified as steroid sensitive or steroid resistant. Steroid sensitivity typically portends a low risk of permanent renal failure. However, some initially steroid-sensitive patients later develop steroid resistance. These patients with late steroid resistance (LSR) are often treated with immunosuppressant medications, but the effect of these additional drugs on the long-term prognosis of LSR is still unknown.
METHODS: A retrospective chart review was performed on patients diagnosed with idiopathic NS and subsequent LSR during the 8-year study period from 2002 up to and including 2009, with a minimum of 2 years of follow-up. Primary outcome measures were proteinuria and renal function.
RESULTS: A total of 29 patients were classified as having LSRNS. The majority of patients received treatment with calcineurin inhibitors and/or mycophenolate mofetil. Seven patients received three or more non-steroid immunosuppressant medications. Sustained complete or partial remission was achieved in 69 % of patients. Three developed end-stage renal disease, and all others maintained normal renal function. There were 13 episodes of serious adverse events, none of which were fatal or irreversible.
CONCLUSION: Most patients with LSRNS responded to immunosuppressive therapy by reduction or resolution of proteinuria and preservation of renal function. The results suggest that immunosuppressive treatment is a viable option in NS patients who develop LSR.
Pediatric nephrology (Berlin, Germany)
Analysis of Variance; Child; Child, Preschool; Disease Progression; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Infant; Kidney; Kidney Failure, Chronic; Male; Midwestern United States; Nephrotic Syndrome; Proteinuria; Remission Induction; Retrospective Studies; Time Factors; Treatment Outcome
Straatmann, Caroline; Ayoob, Rose; Gbadegesin, Rasheed; Gibson, Keisha; Rheault, Michelle N.; Srivastava, Tarak; Tran, Cheryl L.; Gipson, Debbie S.; Greenbaum, Larry A.; Smoyer, William E.; and Vehaskari, V Matti, "Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium." (2013). Manuscripts, Articles, Book Chapters and Other Papers. 1188.