ArticleJournal of the American Society of Nephrology : JASN2017
Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.
Article in Journal of the American Society of Nephrology : JASN, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 125 papers, 6 of them syntheses that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
125 citing papers in PubMed, 6 syntheses or guidelines pooled it, 212 citations in OpenAlex.
- Interventions for idiopathic steroid-resistant nephrotic syndrome in children.The Cochrane database of systematic reviews · 2025Pooled it
- Biomarkers to predict or measure steroid resistance in idiopathic nephrotic syndrome: A systematic review.PloS one · 2025Pooled it
- NPHS Mutations in Pediatric Patients with Congenital and Steroid-Resistant Nephrotic Syndrome.International journal of molecular sciences · 2024Pooled it
- Calcineurin inhibitors in nephrotic syndrome secondary to podocyte gene mutations: a systematic review.Pediatric nephrology (Berlin, Germany) · 2021Pooled it
- IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2020Guideline
- Interventions for idiopathic steroid-resistant nephrotic syndrome in children.The Cochrane database of systematic reviews · 2019Pooled it
- Comparing Kidney Health Outcomes in Children, Adolescents, and Adults With Focal Segmental Glomerulosclerosis.JAMA network open · 2022Trial
- Immunological Impact of a Gluten-Free Dairy-Free Diet in Children With Kidney Disease: A Feasibility Study.Frontiers in immunology · 2021Trial
- Treatment outcomes and ongoing pediatric trials in steroid-resistant nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2026Review
- Rituximab in Pediatric Steroid-Resistant Nephrotic Syndrome: How Early is Early Enough?Kidney international reports · 2026Article
- Proteinuria in adolescence.Pediatric nephrology (Berlin, Germany) · 2026Review
- Kidney Survival in Children With Steroid-Resistant Nephrotic Syndrome Treated by Rituximab.Kidney international reports · 2026Article
- Serum IL-38 and the rs7599662 polymorphism: novel biomarkers for pediatric idiopathic nephrotic syndrome.Italian journal of pediatrics · 2026Article
- Article
- Gene-disease relationships for glomerular phenotypes: expert recommendations from ClinGen.Nature reviews. Nephrology · 2026Review
- Persistent Haematuria Is Associated With Reduced Kidney Survival in Primary Podocytopathies.Nephrology (Carlton, Vic.) · 2026Article
- Predictors of Heart Failure in Pediatric Patients with End-Stage Kidney Disease Secondary to Nephrotic Syndrome.Medicina (Kaunas, Lithuania) · 2026Article
- Prediction Models and Risk Factors for Steroid Resistance in Children with Nephrotic Syndrome: A Systematic Review and Meta-Analysis.Journal of clinical medicine · 2026Review
- Article
- KDOQI US Commentary on the KDIGO 2025 Clinical Practice Guideline for the Management of Nephrotic Syndrome in Children.American journal of kidney diseases : the official journal of the National Kidney Foundation · 2026Review
65 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
43 authors at 20 institutions in 13 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models. Complete and partial remission of proteinuria within 12 months of disease onset occurred in 24.5% and 16.5% of children, respectively, with the highest remission rates achieved with calcineurin inhibitor-based protocols. Ten-year ESRD-free survival rates were 43%, 94%, and 72% in children with IIS resistance, complete remission, and partial remission, respectively; 27% in children with a genetic diagnosis; and 79% and 52% in children with histopathologic findings of minimal change glomerulopathy and FSGS, respectively. Five-year ESRD-free survival rate was 21% for diffuse mesangial sclerosis. IIS responsiveness, presence of a genetic diagnosis, and FSGS or diffuse mesangial sclerosis on initial biopsy as well as age, serum albumin concentration, and CKD stage at onset affected ESRD risk. Our findings suggest that responsiveness to initial IIS and detection of a hereditary podocytopathy are prognostic indicators of favorable and poor long-term outcome, respectively, in children with steroid-resistant nephrotic syndrome. Children with multidrug-resistant sporadic disease show better renal survival than those with genetic disease. Furthermore, histopathologic findings may retain prognostic relevance when a genetic diagnosis is established.
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Registered trials
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