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steroid‑resistant nephrotic syndrome (SRNS)

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"last update: 17 September 2026"                                                                           Download Guideline

- References

1.     Mickinney PA, Feltbower RG, Brocklebank JT et al (2001) Time trends and ethnics pattern of childhood nephrotic syndrome in Yoorkshire, UK. Pediatr Nephrol 16:1040–1044.

2.     International Study Kidney Disease in children (1981) primary nephrotic syndrome in children; clinical significance of histopathologic variant of minimal change and diffuse mesangial hypercellularity: a report of the International Study Kidney Disease in Children. Kidney int 20:765–771.

3.     Trautmann A, Vivarelli M, Samuel S et al. IPNA 2020 clinical practice recommendations for the diagnosis and management of steroid–resistant nephrotic syndrome. Pediatr Nephrol 2020; 35: 1529–1561. Guideline URL:https://link.springer.com/article/https:// doi. org/ 10. 1007/s00467-​020-​04519-1.

4.     KDIGO 21 d-kidney disease: Improving Global Outcomes (KDIGO) Glomerular Disease Work Group (2021) KDIGO 2021 Clinical Practice Guideline for the management of glomerular diseases. Kidney Int. 100(45):51-S276 (https:// kidigo. org/ guide lines/ gd/ (acess ed29/ 09/ 2021) (KDIGO 2021)

5.     Sadowski CE, Lovric S, Ashraf S, Pabst WL, Gee HY, Kohl S, Engelmann S, Vega-Warner V, Fang H, Halbritter J, Somers MJ, Tan W, Shril S, Fessi I, Lifton RP, Bockenhauer D, El-Desoky S, Kari JA, Zenker M, Kemper MJ, Mueller D, Fathy HM, Soliman NA (2015) SRNS Study Group, Hildebrandt F. A single-gene cause in 29.5% of cases of steroid-resistant nephrotic syndrome. J Am Soc Nephrol. 26(6):1279–89

6.     Lovric S, Ashraf S, Tan W, Hildebrandt F (2016) Genetic testing in steroid resistant nephrotic syndrome: when and how? Nephrol Dial Transplant 31(11):1802–1813. https:// doi. org/ 10. 1093/ ndt/ gfv355

7.     Warejko JK, Tan W, Daga A, Schapiro D, Lawson JA, Shril S, Lovric S, Ashraf S, Rao J, Hermle T, Jobst-Schwan T, Widmeier E, Majmundar AJ, Schneider R, Gee HY, Schmidt JM, Vivante A, van der Ven AT, Ityel H, Chen J, Sadowski CE, Kohl S, Pabst WL, Nakayama M, Somers MJG, Rodig NM, Daouk G, Baum M, Stein DR, Ferguson MA, Traum AZ, Soliman NA, Kari JA, El Desoky S, Fathy H, Zenker M, Bakkaloglu SA, Müller D, Noyan A, Ozaltin F, Cadnapaphornchai MA, Hashmi S, Hopcian J, Kopp JB, Benador N, Bockenhauer D, Bogdanovic R, Stajić N, Chernin G, Ettenger R, Fehrenbach H, Kemper M, Munarriz RL, Podracka L, Büscher R, Serdaroglu E, Tasic V, Mane S, Lifton RP, Braun DA, Hildebrandt F (2018) Whole exome sequencing of patients with steroid-resistant nephrotic syndrome. Clin J Am Soc Nephrol 13(1):53–62

8.     Lipska-Ziętkiewicz BS. Genetic Steroid-Resistant Nephrotic Syndrome Overview.2021 Aug 26. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. Gene Reviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2021 .

9.     Hommos MS, Zeng C, Liu Z, Troost JP, Rosenberg AZ, Palmer M, Kremers WK, Cornell LD, Fervenza FC, Barisoni L, Rule AD (2018) Global glomerulosclerosis with nephrotic syndrome; the clinical importance of age adjustment. Kidney Int 93(5):1175–1182

10.  Tinawi M (2020) Update on the etiology, classification, and management of glomerular diseases. Avicenna J Med 10:61–67

11.  Buscher AK, Kranz B, Buscher R et al (2010) Immunosuppression and renal outcome in congenital and pediatric steroid resistant nephrotic syndrome. Clin J Am Soc Nephrol 5:2075–2084

12.  Buscher AK, Beck BB, Melk A et al (2016) Rapid response to cyclosporine A and favorable renal outcome in nongenetic vs genetic steroid-resistant nephrotic syndrome in children. Clin J Am Soc Nephrol 11:245–253

13.  Moustafa B (2006) Infection associated nephropathies IPNA Palermo Seminar Italy 2006

14.  KDIGO (2018) Kidney Disease Improving Global Outcomes Hepatitis C Work Group KDIGO 2018 Clinical Practice Guideline for the prevention, diagnosis, evaluation and treatment of hepatitis C in chronic kidney disease. Int Suppl 2018:891–165

15.  Soliman 21 Neveen a. Soliman Review article COVID-19 infection and the kidney: learning the lesson. Journal of infection and public Health journal home page: http:// www. elsev ier. com/ locate/ jiph. Neveen A Soliman Department of pediatrics, Center of Pediatric Nephrology & Transplantation Kasr Al Ainy School of Medicine, Cairo University.

16.  Ozen S (2020) Sag E Childhood vasculitis. Rheumatology (Oxford) (Suppl 3):iii 95-iii100. https:// doi. org/ 10. 1093/ rheum atolo gy/ kez599

17.  Groot N, de Graeff N, Marks SD, et al (2017) European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative. Ann Rheum Dis.

18.  Abdel Baky A, Omar TEI, Amer YS; Egyptian Pediatric Clinical Practice Guidelines Committee (EPG). Adapting global evidence-based practice guidelines to the Egyptian healthcare context: the Egyptian Pediatric Clinical Practice Guidelines Committee (EPG) initiative. Bull Natl Res Cent. 2023;47(1):88. https://doi.org/10.1186%2Fs42269-023-01059-0

19.  Alshehri A, Almazrou S, Amer Y. Methodological frameworks for adapting global practice guidelines to national context in the Eastern Mediterranean Region. Eastern Mediterranean Health Journal. 2023 Jul 1;29(7). https://www.emro.who.int/emhj-volume-29-2023/volume-29-issue-7/methodological-frameworks-for-adapting-global-practice-guidelines-to-national-context-in-the-eastern-mediterranean-region.html

20.  Schünemann H, Brozek J, Guyatt G, Oxman A (editors). GRADE handbook: handbook for grading the quality of evidence and the strength of recommendations using the GRADE approach. Grading of Recommendations Assessment, Development and Evaluation (GRADE) Working Group; 2013 (Online updated version: https://gdt.gradepro.org/app/handbook/handbook.html Accessed 16/8/2024)

21.  Klugar M, Lotfi T, Darzi AJ, et al. GRADE Guidance 39: Using GRADE-ADOLOPMENT to adopt, adapt or create contextualized recommendations from source guidelines and evidence syntheses. Journal of Clinical Epidemiology. 2024 Aug 6:111494. https://doi.org/10.1016/j.jclinepi.2024.111494 (in press)

22.  Amer YS, Elzalabany MM, Omar TI, Ibrahim AG, Dowidar NL. The ‘Adapted ADAPTE’: an approach to improve utilization of the ADAPTE guideline adaptation resource toolkit in the A lexandria C enter for E vidence‐B ased C linical P ractice G uidelines. Journal of evaluation in clinical practice. 2015 Dec;21(6):1095-106. https://doi.org/10.1111/jep.12479

23.  Brouwers MC, Kho ME, Browman GP, Burgers JS, Cluzeau F, Feder G, Fervers B, Graham ID, Grimshaw J, Hanna SE, Littlejohns P, Makarski J, Zitzelsberger L; AGREE Next Steps Consortium. AGREE II: advancing guideline development, reporting and evaluation in health care. CMAJ. 2010 Dec 14;182(18):E839-42. https://doi.org/10.1503%2Fcmaj.090449 

24.   Agree II (2022) AGREE Enterprise website. Available at: https://www.agreetrust.org/resource-centre/agree-ii/  (Accessed: 16/8/2024).

25.  Song Y, Alonso-Coello P, Ballesteros M, et al. A Reporting Tool for Adapted Guidelines in Health Care: The RIGHT-Ad@pt Checklist[J]. Annals of Internal Medicine, 2022, 175(5):710-719. https://doi.org/10.7326/M21-4352  (Official RIGHT Statement Website: http://www.right-statement.org/extensions/13 Accessed 16/8/2024).