IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome

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 (2020)

Publication Type: Journal article, Review article

Publication year: 2020

Journal

DOI: 10.1007/s00467-020-04519-1

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.

Involved external institutions

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

How to cite

APA:

Trautmann, A., Vivarelli, M., Samuel, S., Gipson, D., Sinha, A., Schaefer, F.,... Haffner, D. (2020). IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome. Pediatric Nephrology. https://doi.org/10.1007/s00467-020-04519-1

MLA:

Trautmann, Agnes, et al. "IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome." Pediatric Nephrology (2020).

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