Agnes Trautmann 1 , Marina Vivarelli 2 , Susan Samuel 3 , Debbie Gipson 4 , Aditi Sinha 5 , Franz Schaefer 1 , Ng Kar Hui 6 , Olivia Boyer 7 , 8 , Moin A Saleem 9 , Luciana Feltran 10 , Janina Müller-Deile 11 , Jan Ulrich Becker 12 , Francisco Cano 13 , Hong Xu 14 , Yam Ngo Lim 15 , William Smoyer 16 , Ifeoma Anochie 17 , Koichi Nakanishi 18 , Elisabeth Hodson 19 , Dieter Haffner , 20 , 21 , 22 , on behalf of the International Pediatric Nephrology Association
7 May 2020
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.