ReviewPediatric health, medicine and therapeutics2019
Diagnostic and Management Challenges in Congenital Nephrotic Syndrome.
Review in Pediatric health, medicine and therapeutics, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 23 citations in OpenAlex.
- Nephrotic syndrome in children: A review.Medicine · 2026Review
- Clinical Presentation, Genetic Testing, and Outcome of Congenital Nephrotic Syndrome in KwaZulu-Natal, South Africa.Nephron · 2026Article
- Perinatal Stroke and Cerebral Sinovenous Thrombosis Caused by Congenital Nephrotic Syndrome NPSH1 (Finnish Type): A Case Report.Neuropediatrics · 2025Article
- Differences in kidney prognosis between congenital and infantile nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2025Article
- Spontaneous bladder perforation with urinary ascites secondary to posterior urethral valves and suspected atypical congenital nephrotic syndrome of a premature neonate: A case report.Urology case reports · 2025Article
- Urinary nephrin - An emerging novel biomarker in early detection of diabetic nephropathy: A review.Bioinformation · 2025Article
- Neonatal nephrotic syndrome: all is not gloomy.BMJ case reports · 2024Article
- Case report: Unveiling a less severe congenital nephrotic syndrome in a Rapa Nui patient with aFrontiers in nephrology · 2024Article
- Use of diuretics in the neonatal period.Pediatric nephrology (Berlin, Germany) · 2021Review
- Thromboprophylaxis in congenital nephrotic syndrome: 15-year experience from a national cohort.Pediatric nephrology (Berlin, Germany) · 2021Article
- Article
- Case Report: CMV-Associated Congenital Nephrotic Syndrome.Frontiers in pediatrics · 2020Article
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Congenital Nephrotic Syndrome (CNS) is defined as nephrotic range proteinuria, hypoalbuminaemia and edema in the first three months of life. CNS is most commonly genetic in cause, with international variance in the incidence of causative mutations. Initially defined by the histopathological appearance, increasingly sophisticated and accessible genetic analyses now provide a body of evidence to suggest that there is a disparity between the histological appearance, the genotype of individuals and the severity of the clinical disease. Through the evolution of management approaches CNS has changed from being an invariably fatal condition to one with appreciable ongoing morbidity and mortality but comparably good outcomes to other causes of paediatric end-stage renal disease, especially following transplantation. This review briefly summarises the more commonly recognised genetic mutations leading to CNS, addresses common management decisions, and concludes with potential therapies for the future.
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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.