ArticlePediatric nephrology (Berlin, Germany)2004
NPHS2 mutation associated with recurrence of proteinuria after transplantation.
Article in Pediatric nephrology (Berlin, Germany), 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers, 1 of them a synthesis that pooled it.
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Who cites it
16 citing papers in PubMed, 1 synthesis or guideline pooled it, 49 citations in OpenAlex.
- Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group.Nature reviews. Nephrology · 2021Guideline
- Monogenic variants drive low recurrence risk in pediatric steroid-resistant nephrotic syndrome after kidney transplantation: a high-consanguinity cohort.Pediatric nephrology (Berlin, Germany) · 2026Article
- Steroid-Resistant Nephrotic Syndrome due toKidney international reports · 2024Article
- A critical re-analysis of cases of post-transplantation recurrence in genetic nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2021Article
- Case Report: The Monogenic Familial Steroid-Resistant Nephrotic Syndrome Caused by a Novel Missense Mutation ofFrontiers in pediatrics · 2021Article
- Post-transplant recurrence of steroid resistant nephrotic syndrome in children: the Italian experience.Journal of nephrology · 2020Article
- Molecular stratification of idiopathic nephrotic syndrome.Nature reviews. Nephrology · 2019Review
- Deriving and understanding the risk of post-transplant recurrence of nephrotic syndrome in the light of current molecular and genetic advances.Pediatric nephrology (Berlin, Germany) · 2018Review
- Glycogen synthase kinase‑3β is required for epithelial‑mesenchymal transition and barrier dysfunction in mouse podocytes under high glucose conditions.Molecular medicine reports · 2016Article
- Congenital nephrotic syndrome and recurrence of proteinuria after renal transplantation.Pediatric nephrology (Berlin, Germany) · 2014Review
- Circulating suPAR in two cohorts of primary FSGS.Journal of the American Society of Nephrology : JASN · 2012Article
- Living donor kidney transplantation in patients with hereditary nephropathies.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
- Familial forms of nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2010Review
- Susceptibility genes in common complex kidney disease.Current opinion in nephrology and hypertension · 2010Review
- Genetic forms of nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2004Review
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mutations in the NPHS2 gene encoding podocin are associated with steroid-resistant nephrotic syndrome (SRNS) in childhood. Patients usually present with focal segmental glomerulosclerosis (FSGS). It is unclear to what extent SRNS due to NPHS2 mutations predisposes to recurrence of proteinuria/FSGS after renal transplantation (RTx). A 4-year-old girl with infantile SRNS was started on peritoneal dialysis because of end-stage renal disease due to FSGS. Mutational screening of the patient and her parents revealed a novel single nucleotide deletion in exon 8 of the NHPS2 gene (948delT), for which the patient was homozygous and her parents confirmed heterozygous asymptomatic carriers. At the age of 4.5 years the patient received a renal graft from her mother. On day 7 after RTx, the patient developed progressive proteinuria (urine protein/creatinine ratio 2.4 g/g), which responded within 1 week to prednisone pulse therapy, an increased cyclosporin A dosage, and ramipril therapy. The patient has maintained stable graft function and no further recurrence of proteinuria has been observed. In conclusion, patients with SRNS due to NPHS2 mutations are not protected from recurrence of proteinuria after RTx. The quick response to increased immunosuppression in our patient suggests an immune-mediated pathomechanism for recurrence of proteinuria.
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