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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
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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