We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome.
- Authors
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
- 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.
- Subjects
THERAPEUTIC use of glucocorticoids; NEPHROTIC syndrome diagnosis; CHRONIC kidney failure; DRUG resistance; IMMUNOSUPPRESSION; IMMUNOSUPPRESSIVE agents; INTERNATIONAL agencies; MEDICAL protocols; GENETIC mutation; NEPHROLOGY; NEPHROTIC syndrome; DISEASE complications; CHILDREN
- Publication
Pediatric Nephrology, 2020, Vol 35, Issue 8, p1529
- ISSN
0931-041X
- Publication type
Article
- DOI
10.1007/s00467-020-04519-1