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Treatment of steroid-resistant nephrotic syndrome in the genomic era.

Publication ,  Journal Article
Bensimhon, AR; Williams, AE; Gbadegesin, RA
Published in: Pediatr Nephrol
November 2019

The pathogenesis of steroid-resistant nephrotic syndrome (SRNS) is not completely known. Recent advances in genomics have elucidated some of the molecular mechanisms and pathophysiology of the disease. More than 50 monogenic causes of SRNS have been identified; however, these genes are responsible for only a small fraction of SRNS in outbred populations. There are currently no guidelines for genetic testing in SRNS, but evidence from the literature suggests that testing should be guided by the genetic architecture of the disease in the population. Notably, most genetic forms of SRNS do not respond to current immunosuppressive therapies; however, a small subset of patients with monogenic SRNS will achieve partial or complete remission with specific immunomodulatory agents, presumably due to non-immunosuppressive effects of these agents. We suggest a pragmatic approach to the therapy of genetic SRNS, as there is no evidence-based algorithm for the management of the disease.

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Published In

Pediatr Nephrol

DOI

EISSN

1432-198X

Publication Date

November 2019

Volume

34

Issue

11

Start / End Page

2279 / 2293

Location

Germany

Related Subject Headings

  • Urology & Nephrology
  • Remission Induction
  • Precision Medicine
  • Nephrotic Syndrome
  • Mutation
  • Inheritance Patterns
  • Immunologic Factors
  • Humans
  • High-Throughput Nucleotide Sequencing
  • Glucocorticoids
 

Citation

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Bensimhon, A. R., Williams, A. E., & Gbadegesin, R. A. (2019). Treatment of steroid-resistant nephrotic syndrome in the genomic era. Pediatr Nephrol, 34(11), 2279–2293. https://doi.org/10.1007/s00467-018-4093-1
Bensimhon, Adam R., Anna E. Williams, and Rasheed A. Gbadegesin. “Treatment of steroid-resistant nephrotic syndrome in the genomic era.Pediatr Nephrol 34, no. 11 (November 2019): 2279–93. https://doi.org/10.1007/s00467-018-4093-1.
Bensimhon AR, Williams AE, Gbadegesin RA. Treatment of steroid-resistant nephrotic syndrome in the genomic era. Pediatr Nephrol. 2019 Nov;34(11):2279–93.
Bensimhon, Adam R., et al. “Treatment of steroid-resistant nephrotic syndrome in the genomic era.Pediatr Nephrol, vol. 34, no. 11, Nov. 2019, pp. 2279–93. Pubmed, doi:10.1007/s00467-018-4093-1.
Bensimhon AR, Williams AE, Gbadegesin RA. Treatment of steroid-resistant nephrotic syndrome in the genomic era. Pediatr Nephrol. 2019 Nov;34(11):2279–2293.
Journal cover image

Published In

Pediatr Nephrol

DOI

EISSN

1432-198X

Publication Date

November 2019

Volume

34

Issue

11

Start / End Page

2279 / 2293

Location

Germany

Related Subject Headings

  • Urology & Nephrology
  • Remission Induction
  • Precision Medicine
  • Nephrotic Syndrome
  • Mutation
  • Inheritance Patterns
  • Immunologic Factors
  • Humans
  • High-Throughput Nucleotide Sequencing
  • Glucocorticoids