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Title | : | IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome |
Authors | : | Trautmann, Agnes Vivarelli, Marina Samuel, Susan Gipson, Debbie Sinha, Aditi Schaefer, Franz Hui, Ng Kar Boyer, Olivia Saleem, Moin A Feltran, Luciana Müller-Deile, Janina Becker, Jan Ulrich Cano, Francisco Xu, Hong Lim, Yam Ngo Smoyer, William Anochie, Ifeoma Nakanishi, Koichi Hodson, Elisabeth Haffner, Dieter International Pediatric Nephrology Association |
Issue Date | : | 7-May-2020 |
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. |
URL | : | https://doi.org/10.1007/s00467-020-04519-1 |
Type Local | : | 雑誌掲載論文 |
ISSN | : | 0931-041X 1432-198X |
Publisher | : | Springer |
URI | : | http://hdl.handle.net/20.500.12000/46947 |
Citation | : | Pediatric Nephrology Vol.35 no.8 p.1529 -1561 |
Appears in Collections | : | Peer-reviewed Journal Articles (Faculty of Medicine)
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Trautmann2020_Article_IPNAClinicalPracticeRecommenda.pdf | | 2130Kb | Adobe PDF | View/Open |
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