IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome
Author
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Trautmann, Agnes
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Vivarelli, Marina
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Samuel, Susan
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Gipson, Debbie
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Sinha, Aditi
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Schaefer, Franz
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Hui, Ng Kar
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Boyer, Olivia
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Saleem, Moin A.
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Feltran, Luciana
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Müller-Deile, Janina
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Becker, Jan Ulrich
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Cano Schuffeneger, Francisco
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Xu, Hong
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Lim, Yam Ngo
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Smoyer, William
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Anochie, Ifeoma
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Nakanishi, Koichi
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Hodson, Elisabeth
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Haffner, Dieter
Admission date
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2020-06-16T22:11:33Z
Available date
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2020-06-16T22:11:33Z
Publication date
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2020
Cita de ítem
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Pediatr Nephrol (2020)
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Identifier
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10.1007/s00467-020-04519-1
Identifier
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https://repositorio.uchile.cl/handle/2250/175520
Abstract
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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.