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.

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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://dx.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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