1. IPNA Clinical Practice Recommendations for the Diagnosis and Management of Children with Steroid-resistant Nephrotic Syndrome
- Author
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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, and International Pediatric Nephrology Association
- Subjects
Male ,Nephrology ,Pediatrics ,medicine.medical_specialty ,Nephrotic Syndrome ,Adolescent ,Population ,Drug Resistance ,Guidelines ,Prednisone ,Internal medicine ,Chronic kidney disease ,Genetics ,medicine ,Humans ,ddc:610 ,Child ,education ,Glucocorticoids ,Congenital nephrotic syndrome ,Children ,Steroid-resistant nephrotic syndrome ,Outcome ,education.field_of_study ,business.industry ,Remission Induction ,Infant, Newborn ,Infant ,medicine.disease ,Immunosuppressive treatment ,Transplantation ,Clinical Practice ,Proteinuria ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Female ,business ,Nephrotic syndrome ,Immunosuppressive Agents ,medicine.drug ,Kidney disease - 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., 論文
- Published
- 2020
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