1. Рекомендації для клінічної практики IPNA щодо діагностики й лікування дітей із стероїд-резистентним нефротичним синдромом.
- Author
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Trautmann, Agnes, Vivarelli, Marina, Samuel, Susan, Gipson, Debbie, Sinha, Aditi, Schaefer, Franz, Ng Kar Hui, Boyer, Olivia, Saleem, Moin A., Feltran, Luciana, Deile, Janina Müller-, Becker, Jan Ulrich, Cano, Francisco, Hong Xu, Yam Ngo Lim, Smoyer, William, Anochie, Ifeoma, Koichi Nakanishi, Hodson, Elisabeth, and Haffner, Dieter
- 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. [ABSTRACT FROM AUTHOR]
- Published
- 2020
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