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"Kidneys" Том 9, №3, 2020

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

Authors: Agnes Trautmann, Marina Vivarelli, Susan Samuel, Debbie Gipson, Aditi Sinha, Franz Schaefer, Ng Kar Hui, Olivia Boyer, Moin A. Saleem, Luciana Feltran, Janina Müller-Deile, Jan Ulrich Becker, Francisco Cano, Hong Xu, Yam Ngo Lim, William Smoyer, Ifeoma Anochie, Koichi Nakanishi, Elisabeth Hodson, Dieter Haffner on behalf of the International Pediatric Nephrology Association

Categories: Nephrology

Sections: Official information

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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 ...

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