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Pediatric Nephrology
Erschienen in: Pediatric Nephrology 11/2019

02.10.2018 | Review

Treatment of steroid-resistant nephrotic syndrome in the genomic era

verfasst von: Adam R. Bensimhon, Anna E. Williams, Rasheed A. Gbadegesin

Erschienen in: Pediatric Nephrology | Ausgabe 11/2019

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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.
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Metadaten
Titel
Treatment of steroid-resistant nephrotic syndrome in the genomic era
verfasst von
Adam R. Bensimhon
Anna E. Williams
Rasheed A. Gbadegesin
Publikationsdatum
02.10.2018
Verlag
Springer Berlin Heidelberg
Erschienen in
Pediatric Nephrology / Ausgabe 11/2019
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-018-4093-1

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