ArticleKidney international2023
A multicenter retrospective study of calcineurin inhibitors in nephrotic syndrome secondary to podocyte gene variants.
Article in Kidney international, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 30 citations in OpenAlex.
- Nanotechnology-mediated podocyte injury repair: mechanistic exploration and therapeutic prospects.Renal failure · 2026Review
- Immune dysregulation in pediatric cancer-associated nephrotic syndrome: current insights.Pediatric nephrology (Berlin, Germany) · 2026Review
- Genetic insights into congenital and infantile nephrotic syndrome: predicting severity and informing care.Pediatric nephrology (Berlin, Germany) · 2026Article
- Clinicopathological characteristics and cyclosporine-responsive proteinuria in nail-patella syndrome with a novel LMX1B mutation.Pediatric nephrology (Berlin, Germany) · 2026Article
- 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
- Aligning Practice With Guidelines: A Canadian National Survey and Canadian Society of Nephrology Commentary on the 2025 KDIGO Pediatric Nephrotic Syndrome Recommendations.Canadian journal of kidney health and disease · 2026Article
- A case of Frasier syndrome achieved complete remission by cyclosporine A for steroid-resistant nephrotic syndrome.CEN case reports · 2025Article
- Vincristine in FSGS: Rediscovering an Old Therapeutic for FSGS.Kidney international reports · 2025Article
- Efficacy of Calcineurin Inhibition in Children With Steroid-Resistant Nephrotic Syndrome.Kidney international reports · 2025Article
- Childhood idiopathic nephrotic syndrome: recent advancements shaping future guidelines.Pediatric nephrology (Berlin, Germany) · 2025Review
- Childhood-onset lupus nephritis: long-term outcomes and their predictors.Pediatric nephrology (Berlin, Germany) · 2025Article
- Two brothers presented with rare clinical characteristics with a novel LAGE3 variant: a case report and literature review.BMC pediatrics · 2025Review
- Mechanisms of podocyte injury in genetic kidney disease.Pediatric nephrology (Berlin, Germany) · 2025Review
- In steroid-resistant nephrotic syndrome that meets the strict definition, monogenic variants are less common than expected.Pediatric nephrology (Berlin, Germany) · 2024Article
- Managing Lupus Nephritis in Children and Adolescents.Paediatric drugs · 2024Review
- Article
- The case for treatment of monogenic SRNS with calcineurin inhibitors.Kidney international · 2023Article
- State of the art in childhood nephrotic syndrome: concrete discoveries and unmet needs.Frontiers in immunology · 2023Review
Corrections and comments
- Commented on by
- Erratum issued
Authors and funding
39 authors at 20 institutions in 18 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
While 44-83% of children with steroid-resistant nephrotic syndrome (SRNS) without a proven genetic cause respond to treatment with a calcineurin inhibitor (CNI), current guidelines recommend against the use of immunosuppression in monogenic SRNS. This is despite existing evidence suggesting that remission with CNI treatment is possible and can improve prognosis in some cases of monogenic SRNS. Herein, our retrospective study assessed response frequency, predictors of response and kidney function outcomes among children with monogenic SRNS treated with a CNI for at least three months. Data from 203 cases (age 0-18 years) were collected from 37 pediatric nephrology centers. Variant pathogenicity was reviewed by a geneticist, and 122 patients with a pathogenic and 19 with a possible pathogenic genotype were included in the analysis. After six months of treatment and at last visit, 27.6% and 22.5% of all patients respectively, demonstrated partial or full response. Achievement of at least partial response at six months of treatment conferred a significant reduction in kidney failure risk at last follow-up compared to no response (hazard ratio [95% confidence interval] 0.25, [0.10-0.62]). Moreover, risk of kidney failure was significantly lower when only those with a follow-up longer than two years were considered (hazard ratio 0.35, [0.14-0.91]). Higher serum albumin level at CNI initiation was the only factor related to increased likelihood of significant remission at six months (odds ratio [95% confidence interval] 1.16, [1.08-1.24]). Thus, our findings justify a treatment trial with a CNI also in children with monogenic SRNS.
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