ArticlePediatric nephrology (Berlin, Germany)2009
Partial remission with cyclosporine A in a patient with nephrotic syndrome due to NPHS2 mutation.
Article in Pediatric nephrology (Berlin, Germany), 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 38 citations in OpenAlex.
- Treatment of steroid-resistant nephrotic syndrome in children: new guidelines from KDIGO.Pediatric nephrology (Berlin, Germany) · 2013Guideline
- How immunosuppressive drugs may directly target podocytes in glomerular diseases.Pediatric nephrology (Berlin, Germany) · 2022Review
- Current understandings in treating children with steroid-resistant nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2021Review
- Treatment of steroid-resistant nephrotic syndrome in the genomic era.Pediatric nephrology (Berlin, Germany) · 2019Review
- Genetic diagnosis of steroid-resistant nephrotic syndrome in a longitudinal collection of Czech and Slovak patients: a high proportion of causative variants in NUP93.Pediatric nephrology (Berlin, Germany) · 2018Article
- Application of next-generation sequencing technology to diagnosis and treatment of focal segmental glomerulosclerosis.Clinical and experimental nephrology · 2018Review
- 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
- Podocyte directed therapy of nephrotic syndrome-can we bring the inside out?Pediatric nephrology (Berlin, Germany) · 2016Review
- Rapid Response to Cyclosporin A and Favorable Renal Outcome in Nongenetic Versus Genetic Steroid-Resistant Nephrotic Syndrome.Clinical journal of the American Society of Nephrology : CJASN · 2016Article
- Nonimmunologic targets of immunosuppressive agents in podocytes.Kidney research and clinical practice · 2015Review
- Heterogeneous genetic alterations in sporadic nephrotic syndrome associate with resistance to immunosuppression.Journal of the American Society of Nephrology : JASN · 2015Article
- Actin-associated Proteins in the Pathogenesis of Podocyte Injury.Current genomics · 2013Article
- The podocyte as a target: cyclosporin A in the management of the nephrotic syndrome caused by WT1 mutations.European journal of pediatrics · 2011Review
- Effect of cyclosporin A on proteinuria in the course of glomerulopathy associated with WT1 mutations.European journal of pediatrics · 2011Article
- Immunosuppression and renal outcome in congenital and pediatric steroid-resistant nephrotic syndrome.Clinical journal of the American Society of Nephrology : CJASN · 2010Article
- Frasier syndrome: early gonadoblastoma and cyclosporine responsiveness.Pediatric nephrology (Berlin, Germany) · 2010Article
- Glomerular diseases: genetic causes and future therapeutics.Nature reviews. Nephrology · 2010Review
- Hereditary nephrotic syndrome: a systematic approach for genetic testing and a review of associated podocyte gene mutations.Pediatric nephrology (Berlin, Germany) · 2010Review
- Non-immunologic mechanisms of calcineurin inhibitors explain its antiproteinuric effects in genetic glomerulopathies.Pediatric nephrology (Berlin, Germany) · 2010Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Autosomal recessive steroid-resistant nephrotic syndrome (NS) is a rare, genetically determined nephropathy caused mainly by a mutation in the NPHS2 gene. This type of NS is usually resistant to other immunosuppressive therapy as well, but a few cases of cyclosporine A-induced partial remission of inherited NS have been reported. We present a boy that developed NS at the age of 18 months. There was no decrease of proteinuria on standard prednisolone therapy, and a diagnosis of steroid-resistant NS was established. However, the proteinuria decreased significantly following the initiation of cyclosporine A therapy (from 1280 to 380 mg/m(2) per day) without any negative effects on renal function (stable glomerular filtration rate 130-150 ml/min per 1.73 m(2)). The molecular genetic test revealed a homozygous R138Q mutation in the NPHS2 gene. Our case demonstrates that cyclosporine A can induce partial remission in patients with genetic forms of NS without influencing the glomerular filtration rate. However, its long-term effect and safety in children with hereditary forms of nephrotic syndrome have yet to be investigated.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.