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Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium

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Abstract

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.

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Acknowledgments

We are grateful to Corinna Bowers and the MWPNC for their help and participation in this study.

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Authors and Affiliations

  1. Department of Pediatrics, Louisiana State University Health Sciences Center, 200 Henry Clay Avenue, New Orleans, LA, 70118, USA

    Caroline Straatmann & V. Matti Vehaskari

  2. Nationwide Children’s Hospital, Columbus, OH, USA

    Rose Ayoob

  3. Department of Pediatrics, Duke University, Durham, NC, USA

    Rasheed Gbadegesin

  4. Department of Medicine and Pediatrics, University of North Carolina, Chapel Hill, NC, USA

    Keisha Gibson

  5. University of Minnesota Amplatz Children’s Hospital, Minneapolis, MN, USA

    Michelle N. Rheault

  6. Children’s Mercy Hospital and University of Missouri at Kansas City, Kansas City, MO, USA

    Tarak Srivastava

  7. Department of Pediatrics, Mayo Clinic, Rochester, MN, USA

    Cheryl L. Tran

  8. Department of Pediatrics, University of Michigan School of Medicine, Ann Arbor, MI, USA

    Debbie S. Gipson

  9. Emory University and Children’s Healthcare of Atlanta, Atlanta, GA, USA

    Larry A. Greenbaum

  10. The Research Institute at Nationwide Children’s Hospital and Department of Pediatrics, The Ohio State University, Columbus, OH, USA

    William E. Smoyer

Authors
  1. Caroline Straatmann
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  2. Rose Ayoob
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  3. Rasheed Gbadegesin
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  4. Keisha Gibson
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  5. Michelle N. Rheault
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  6. Tarak Srivastava
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  7. Cheryl L. Tran
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  8. Debbie S. Gipson
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  9. Larry A. Greenbaum
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  10. William E. Smoyer
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  11. V. Matti Vehaskari
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Corresponding author

Correspondence to Caroline Straatmann.

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Straatmann, C., Ayoob, R., Gbadegesin, R. et al. Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium. Pediatr Nephrol 28, 1235–1241 (2013). https://doi.org/10.1007/s00467-013-2483-y

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  • DOI: https://doi.org/10.1007/s00467-013-2483-y

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