Клинические практические рекомендации IPNA по диагностике и лечению детей со стероид-резистентным нефротическим синдромом

Trautmann A, Vivarelli M, Samuel S, Gipson D, Sinha A, Schaefer F, Hui NK, Boyer O, Saleem MA, Feltran L, Müller-Deile J, Becker JU, Cano F, Xu H, Lim YN, Smoyer W, Anochie I, Nakanishi K, Hodson E, Haffner D (2021)

Publication Type: Journal article

Publication year: 2021

Journal

Book Volume: 22

Pages Range: 435-473

Journal Issue: 4

DOI: 10.28996/2618-9801-2020-4-435-473

Abstract

Idiopathic nephrotic syndrome newly affects 1-3 per 100,000 children per year. Approximately 85% of cases show complete remission of proteinuria following glucocorticoid treatment. Patients who do not achieve complete remission within 4-6 weeks of glucocorticoid treatment have steroid-resistant nephrotic syndrome (SRNS). In 10-30% of steroid resistant patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor of immune origin is assumed in the remaining ones. Diagnosis and management of SRNS is a great challenge due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation. A team of experts including pediatric nephrologists and renal geneticists from the International Pediatric Nephrology Association (IPNA), a renal pathologist, and an adult nephrologist have now developed comprehensive clinical practice recommendations on the diagnosis and management of SRNS in children. The team performed a systematic literature review on 9 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, formulated recommendations and formally graded them at a consensus meeting, with input from patient representatives and a dietician acting as external advisors and a voting panel of pediatric nephrologists. Research recommendations are also given.

Authors with CRIS profile

Involved external institutions

Universitätsklinikum Heidelberg Germany (DE) University of Michigan - Dearborn United States (USA) (US) University of Sydney (USYD) Australia (AU) University of Port Harcourt Nigeria (NG) Medizinische Hochschule Hannover (MHH) / Hannover Medical School Germany (DE) Universitätsklinikum Köln Germany (DE) University of the Ryukyus / 琉球大学 Japan (JP) Ohio State University United States (USA) (US) Fudan University / 复旦大学 China (CN) Universidad de Chile Chile (CL) University of Calgary Canada (CA) NUS Yong Loo Lin School of Medicine Singapore (SG) Federal University of São Paulo / Universidade Federal de São Paulo (UNIFESP) Brazil (BR) University of Bristol United Kingdom (GB) All India Institute of Medical Sciences India (IN) Institut des maladies génétiques Imagine France (FR) Ospedale Pediatrico Bambino Gesu Italy (IT)

How to cite

APA:

Trautmann, A., Vivarelli, M., Samuel, S., Gipson, D., Sinha, A., Schaefer, F.,... Haffner, D. (2021). Клинические практические рекомендации IPNA по диагностике и лечению детей со стероид-резистентным нефротическим синдромом. Нефрология и диализ, 22(4), 435-473. https://doi.org/10.28996/2618-9801-2020-4-435-473

MLA:

Trautmann, Agnes, et al. "Клинические практические рекомендации IPNA по диагностике и лечению детей со стероид-резистентным нефротическим синдромом." Нефрология и диализ 22.4 (2021): 435-473.

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