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Review
. 2019 Nov;34(11):2279-2293.
doi: 10.1007/s00467-018-4093-1. Epub 2018 Oct 2.

Treatment of steroid-resistant nephrotic syndrome in the genomic era

Adam R Bensimhon  1 , Anna E Williams  1 , Rasheed A Gbadegesin  2   3   4
Affiliations
Review

Treatment of steroid-resistant nephrotic syndrome in the genomic era

Adam R Bensimhon et al. Pediatr Nephrol. 2019 Nov.

Abstract

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.

Keywords: Genetic SRNS; Nephrotic syndrome; SRNS; Treatment.

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Conflict of interest statement

Conflict of interest The authors declare that they have no conflicts of interest.

Figures

Fig. 1
Fig. 1
Approach to genetic testing in SRNS. a Approach to genetic testing in population with high prevalence of SRNS such as an inbred population or populations with founder mutations. b Approach to genetic testing in an outbred population
Fig. 2
Fig. 2
Approach to management of a child with non-genetic steroid-resistant nephrotic syndrome (SRNS)
Fig. 3
Fig. 3
Suggested approach to management of a child with genetic steroid-resistant nephrotic syndrome (SRNS)
See this image and copyright information in PMC

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