IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome
Agnes Trautmann, Marina Vivarelli, Susan Samuel, Debbie S. Gipson, Aditi Sinha, Franz Schaefer, Ng Kar Hui, Olivia Boyer, Moin A. Saleem, Luciana de Santis Feltran, Janina Müller‐Deile, Jan U. Becker, Francisco Cano, Hong Xu, Yam Ngo Lim, William E. Smoyer, Ifeoma Anochie, Koichi Nakanishi, Elisabeth M Hodson, Dieter Haffner
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.