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. © 2020, The Author(s).
Original language | English |
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Pages (from-to) | 1529-1561 |
Number of pages | 33 |
Journal | Pediatr. Nephrol. |
Volume | 35 |
Issue number | 8 |
DOIs | |
Publication status | Published - 2020 |
Keywords
- 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