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Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium |
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| Authors: | Caroline Straatmann Rose Ayoob Rasheed Gbadegesin Keisha Gibson Michelle N Rheault Tarak Srivastava Cheryl L Tran Debbie S Gipson Larry A Greenbaum William E Smoyer V Matti Vehaskari |
| Institution: | 1. Department of Pediatrics, Louisiana State University Health Sciences Center, 200 Henry Clay Avenue, New Orleans, LA, 70118, USA 2. Nationwide Children’s Hospital, Columbus, OH, USA 3. Department of Pediatrics, Duke University, Durham, NC, USA 4. Department of Medicine and Pediatrics, University of North Carolina, Chapel Hill, NC, USA 5. University of Minnesota Amplatz Children’s Hospital, Minneapolis, MN, USA 6. Children’s Mercy Hospital and University of Missouri at Kansas City, Kansas City, MO, USA 7. Department of Pediatrics, Mayo Clinic, Rochester, MN, USA 8. Department of Pediatrics, University of Michigan School of Medicine, Ann Arbor, MI, USA 9. Emory University and Children’s Healthcare of Atlanta, Atlanta, GA, USA 10. The Research Institute at Nationwide Children’s Hospital and Department of Pediatrics, The Ohio State University, Columbus, OH, USA |
| Abstract: | BackgroundIdiopathic 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.MethodsA 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.ResultsA 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.ConclusionMost 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. |
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