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

A. Trautmann, M. Vivarelli, S. Samuel, D. Gipson, A. Sinha, F. Schaefer, N.K. Hui, O. Boyer, M.A. Saleem, L. Feltran, J. Müller-Deile, J.U. Becker, F. Cano, H. Xu, Y.N. Lim, W. Smoyer, I. Anochie, K. Nakanishi, E. Hodson, D. Haffneron behalf of the International Pediatric Nephrology Association

Research output: Contribution to journalArticlepeer-review


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. © 2020, The Author(s).
Original languageEnglish
Pages (from-to)1529-1561
Number of pages33
JournalPediatr. Nephrol.
Issue number8
Publication statusPublished - 2020


  • Children
  • Chronic kidney disease
  • Genetics
  • Immunosuppressive treatment
  • Outcome
  • Pediatrics
  • Steroid-resistant nephrotic syndrome
  • angiotensin receptor antagonist
  • calcineurin inhibitor
  • cyclosporine
  • dipeptidyl carboxypeptidase inhibitor
  • glucocorticoid
  • mycophenolate mofetil
  • prednisolone
  • prednisone
  • rituximab
  • tacrolimus
  • abdominal pain
  • angioneurotic edema
  • Article
  • behavior disorder
  • bronchospasm
  • cataract
  • clinical feature
  • clinical practice
  • congenital nephrotic syndrome
  • Cushing syndrome
  • diabetes mellitus
  • diarrhea
  • disease classification
  • disease severity assessment
  • drug tolerability
  • evidence based medicine
  • gene identification
  • genetic counseling
  • genetic screening
  • glaucoma
  • glucose intolerance
  • growth retardation
  • headache
  • hepatitis
  • hepatitis B
  • high throughput sequencing
  • human
  • hypertension
  • hypertrichosis
  • hypoalbuminemia
  • hypomagnesemia
  • immunoglobulin deficiency
  • infection prevention
  • kidney biopsy
  • leg cramp
  • leukopenia
  • lifestyle modification
  • nausea
  • nephrotoxicity
  • neutropenia
  • pancytopenia
  • paresthesia
  • pediatrics
  • practice guideline
  • priority journal
  • progressive multifocal leukoencephalopathy
  • proteinuria
  • skin cancer
  • treatment duration
  • treatment outcome
  • treatment planning
  • treatment response
  • treatment withdrawal
  • urinalysis
  • urticaria
  • verruca vulgaris
  • vitamin supplementation
  • vomiting


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