ArticlePediatric nephrology (Berlin, Germany)2010
Successful treatment of steroid-resistant nephrotic syndrome associated with WT1 mutations.
Article in Pediatric nephrology (Berlin, Germany), 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers, 1 of them a synthesis that pooled it.
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38 citing papers in PubMed, 1 synthesis or guideline pooled it, 78 citations in OpenAlex.
- Treatment of steroid-resistant nephrotic syndrome in children: new guidelines from KDIGO.Pediatric nephrology (Berlin, Germany) · 2013Guideline
- Gene-disease relationships for glomerular phenotypes: expert recommendations from ClinGen.Nature reviews. Nephrology · 2026Review
- A NovelCase reports in genetics · 2026Article
- A case of Frasier syndrome achieved complete remission by cyclosporine A for steroid-resistant nephrotic syndrome.CEN case reports · 2025Article
- Patient-specific hiPSC-Podocytes as an in vitro model of genetic FSGS.Scientific reports · 2025Article
- The CaPediatric nephrology (Berlin, Germany) · 2025Review
- Outcomes and prognostic factors in childhood-onset steroid-resistant nephrotic syndrome: a retrospective single-center study.Pediatric nephrology (Berlin, Germany) · 2025Article
- An updated view of the pathogenesis of steroid-sensitive nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2022Review
- Multicentric Carpotarsal Osteolysis Syndrome Associated Nephropathy: Novel Variants ofJournal of clinical medicine · 2022Article
- How immunosuppressive drugs may directly target podocytes in glomerular diseases.Pediatric nephrology (Berlin, Germany) · 2022Review
- Isolated steroid-resistant nephrotic syndrome in a Chinese child carrying a de novo mutation in WT1 gene:a case report and literature review.BMC pediatrics · 2022Review
- Systematic Review of Genotype-Phenotype Correlations in Frasier Syndrome.Kidney international reports · 2021Article
- Novel insights in the genetics of steroid-sensitive nephrotic syndrome in childhood.Pediatric nephrology (Berlin, Germany) · 2021Review
- Immune-complex glomerulonephritis with a membranoproliferative pattern in Frasier syndrome: a case report and review of the literature.BMC nephrology · 2020Review
- Treatment of steroid-resistant nephrotic syndrome in the genomic era.Pediatric nephrology (Berlin, Germany) · 2019Review
- Genetic testing in steroid-resistant nephrotic syndrome: why, who, when and how?Pediatric nephrology (Berlin, Germany) · 2019Review
- Treatment of Genetic Forms of Nephrotic Syndrome.Frontiers in pediatrics · 2018Review
- Clinical genetic testing using a custom-designed steroid-resistant nephrotic syndrome gene panel: analysis and recommendations.Journal of medical genetics · 2017Article
- Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.Journal of the American Society of Nephrology : JASN · 2017Article
- Podocytes from the diagnostic and therapeutic point of view.Pflugers Archiv : European journal of physiology · 2017Review
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4 authors at 2 institutions in 2 countries.
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No grant is acknowledged in the PubMed record.
Abstract
The Wilms' tumor suppressor gene 1 (WT1) encodes a transcription factor involved in kidney and gonadal development. WT1 is also a key regulator of podocyte functions and mutations have been found in a small percentage of children with isolated or syndromal steroid-resistant nephrotic syndrome. It is commonly assumed that the nephrotic syndrome (NS) in patients with WT1 mutations is unresponsive to therapy and characterized by rapid progression to end-stage renal disease. We report long-term observations in 3 children with focal-segmental glomerulosclerosis associated with WT1 mutations and NS (2 cases) or nephrotic range proteinuria (1 case). All patients showed a favorable response to an intensified therapy consisting of cyclosporin A (CyA) in combination with induction therapy with intravenous and oral prednisone. Treatment with angiotensin-converting enzyme inhibitors and angiotensin receptor blockers was added to the regimen at various times. As shown both by the short-term response and during long-term follow-up, this treatment resulted in clinical remission of the NS and/or significant reduction of proteinuria, while normal renal function could be maintained over many years. Thus, glomerular diseases in selected patients with mutations in genes regulating renal development and podocyte function may respond to combination therapy with CyA and corticosteroids.
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