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Treatment of steroid-resistant nephrotic syndrome in children: new guidelines from KDIGO

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Abstract

Kidney Disease: Improving Global Outcomes (KDIGO) recently published the clinical practice guideline on glomerulonephritis (GN) to assist the practitioner caring for patients with GN. Chapter 4 of the guideline focuses on managing children aged 1–18 years with steroid-resistant nephrotic syndrome (SRNS), defined by an inability to achieve complete remission with corticosteroid therapy. Guideline development followed a thorough evidence review, and management recommendations and suggestions were based on the best available evidence. Limitations of the evidence, including the paucity of large-scale randomized controlled trials, are discussed. This article provides both the guideline recommendations and a brief review of relevant treatment trials related to each recommendation. This précis serves as a summary of the complete guidelines recently published.

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Author information

Authors and Affiliations

  1. Division of Nephrology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, MI, USA

    Rebecca M. Lombel & Debbie S. Gipson

  2. Centre for Kidney Research, The Children’s Hospital at Westmead, Westmead, Sydney, NSW, Australia

    Elisabeth M. Hodson

  3. Sydney School of Public Health, University of Sydney, Sydney, NSW, Australia

    Elisabeth M. Hodson

  4. Michigan Institute for Clinical and Health Research, University of Michigan, Ann Arbor, MI, USA

    Debbie S. Gipson

  5. Pediatric Nephrology, C.S. Mott Children’s Hospital, 1540 E. Hospital Drive, SPC 4297, Ann Arbor, MI, 48109-4297, USA

    Rebecca M. Lombel

Authors
  1. Rebecca M. Lombel
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  2. Elisabeth M. Hodson
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  3. Debbie S. Gipson
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Corresponding author

Correspondence to Rebecca M. Lombel.

Additional information

Educational review questions (answers are provided following the reference list)

1. For patients with steroid-resistant nephrotic syndrome, what agents may be considered for therapy?

A. Cyclosporine

B. Tacrolimus

C. Mycophenolate mofetil

D. High-dose corticosteroids

E. All of the above

2. The risk of end-stage renal disease within 5 years of diagnosis for patients with steroid-resistant nephrotic syndrome who do not achieve a partial or complete remission is:

A. <5%

B. 10%

C. 25%

D. 50%

E. 75%

3. Which steroid-sparing agent has been shown to induce at least partial remission in some patients with steroid-resistant nephrotic syndrome with podocin mutations?

A. Cyclosporine

B. Cyclophosphamide

C. Mycophenolate mofetil

D. Levamisole

E. Rituximab

4. Cosmetic changes including gingival hyperplasia and hypertrichosis are side effects of which steroid-sparing agent?

A. Cyclophosphamide

B. Chlorambucil

C. Cyclosporine

D. Tacrolimus

E. Mycophenolate mofetil

5. To be considered an adequate tissue sample, what is the minimum number of glomeruli a biopsy sample should have to detect or exclude glomerular lesions that may be focal or segmental?

A. 5

B. 10

C. 20

D. 30

E. 50

Answers:

1. E

2. D

3. A

4. C

5. C (KDIGO Guidelines, Chap. 2)

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Lombel, R.M., Hodson, E.M. & Gipson, D.S. Treatment of steroid-resistant nephrotic syndrome in children: new guidelines from KDIGO. Pediatr Nephrol 28, 409–414 (2013). https://doi.org/10.1007/s00467-012-2304-8

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  • DOI: https://doi.org/10.1007/s00467-012-2304-8

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