IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome

摘要: Idiopathic nephrotic syndrome newly affects 1–3 per 100,000 children year. Approximately 85% of cases show complete remission proteinuria following glucocorticoid treatment. Patients who do not achieve within 4–6 weeks treatment have steroid-resistant (SRNS). In 10–30% patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor immune origin is assumed the remaining ones. Diagnosis and management SRNS a great challenge due to its heterogeneous etiology, frequent lack by further immunosuppressive treatment, severe complications including development end-stage kidney disease recurrence after renal transplantation. A team experts pediatric nephrologists geneticists from International Pediatric Nephrology Association (IPNA), pathologist, adult nephrologist now developed comprehensive clinical practice recommendations on diagnosis children. The performed systematic literature review 9 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, formulated formally graded them at consensus meeting, with input patient representatives dietician acting as external advisors voting panel nephrologists. Research are also given.