Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Comment
. 2023 May;103(5):839-841.
doi: 10.1016/j.kint.2023.02.017.

The case for treatment of monogenic SRNS with calcineurin inhibitors

Brandon M Lane  1 , Rasheed A Gbadegesin  2
Affiliations
Comment

The case for treatment of monogenic SRNS with calcineurin inhibitors

Brandon M Lane et al. Kidney Int. 2023 May.

Abstract

Currently, no evidence-based guidelines exist for treatment of children with monogenic steroid-resistant nephrotic syndrome. A retrospective study on 141 patients from Malakasioti et al. revealed that 27.6% responded to calcineurin inhibitor (CNI) treatment, and 75% of responders maintained stable kidney function. Virtually all CNI nonresponders developed progressive loss of kidney function. This study emphasized roles for CNIs in patients with monogenic steroid-resistant nephrotic syndrome, and the need for future studies to identify CNI response biomarkers.

PubMed Disclaimer

Figures

Figure 1:
Figure 1:
The study by Malakasioti et al revealed that 27.6% of patients with known monogenic SRNS achieved complete or partial remission to CNI therapy. The >70% that did not respond were unnecessarily exposed to the toxic side effects of CNI treatment. The findings highlight the need for the discovery of biomarkers of CNI therapy response. These biomarkers could be variants in gene that directly or indirectly affect CN signaling such as variants in RCAN genes, urine, and plasma biomarkers.
See this image and copyright information in PMC

Comment on

  • A multicenter retrospective study of calcineurin inhibitors in nephrotic syndrome secondary to podocyte gene variants.
    Malakasioti G, Iancu D, Milovanova A, Tsygin A, Horinouchi T, Nagano C, Nozu K, Kamei K, Fujinaga S, Iijima K, Sinha R, Basu B, Morello W, Montini G, Waters A, Boyer O, Yıldırım ZY, Yel S, Dursun İ, McCarthy HJ, Vivarelli M, Prikhodina L, Besouw MTP, Chan EY, Huang W, Kemper MJ, Loos S, Prestidge C, Wong W, Zlatanova G, Ehren R, Weber LT, Chehade H, Hooman N, Tkaczyk M, Stańczyk M, Miligkos M, Tullus K; CNI in Monogenic SRNS Study Investigators. Malakasioti G, et al. Kidney Int. 2023 May;103(5):962-972. doi: 10.1016/j.kint.2023.02.022. Epub 2023 Mar 8. Kidney Int. 2023. PMID: 36898413

References

    1. Trautmann A, Vivarelli M, Samuel S, et al. IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2020;35(8):1529–1561 - PMC - PubMed
    1. Choudhry S, Bagga A, Hari P, Sharma S, Kalaivani M, Dinda A. Efficacy and safety of tacrolimus versus cyclosporine in children with steroid-resistant nephrotic syndrome: a randomized controlled trial. Am J Kidney Dis. 2009;53(5):760–769. - PubMed
    1. Buscher AK, Beck BB, Melk A, et al. Rapid Response to Cyclosporin A and Favorable Renal Outcome in Nongenetic Versus Genetic Steroid-Resistant Nephrotic Syndrome. Clin J Am Soc Nephrol. 2016;11(2):245–253.3. - PMC - PubMed
    1. Trautmann A, Schnaidt S, Lipska-Zietkiewicz BS, et al. Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children. J Am Soc Nephrol. 2017;28(10):3055–3065. - PMC - PubMed
    1. Malakasioti G, Iancu D, Tullus K. Calcineurin inhibitors in nephrotic syndrome secondary to podocyte gene mutations: a systematic review. Pediatr Nephrol. 2021;36(6):1353–1364. - PubMed

Publication types

MeSH terms

LinkOut - more resources