GuidelineNature reviews. Nephrology2021
Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group.
Guideline in Nature reviews. Nephrology, 2021. 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 47 papers, 1 of them a synthesis that pooled it.
What it found
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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
47 citing papers in PubMed, 1 synthesis or guideline pooled it, 92 citations in OpenAlex.
- NPHS Mutations in Pediatric Patients with Congenital and Steroid-Resistant Nephrotic Syndrome.International journal of molecular sciences · 2024Pooled it
- Transient Nephrotic Syndrome in an Infant With Heterozygous Variants of Uncertain Significance in LMX1B and TTC21B.Cureus · 2026Article
- Genetic insights into congenital and infantile nephrotic syndrome: predicting severity and informing care.Pediatric nephrology (Berlin, Germany) · 2026Article
- Hyperkalemia in pediatric nephrectomy: a common complication.Pediatric nephrology (Berlin, Germany) · 2026Article
- European Reference Networks - a flagship activity of the EU in the field of rare and complex diseases: from 2017 to 2025.Orphanet journal of rare diseases · 2026Review
- Congenital nephrotic syndrome in a newborn with glycogen storage disease and Wilms tumor 1 (WT1) mutation.CEN case reports · 2026Article
- A Rare NPHS2 Mutation (E130K) in Hereditary Steroid-Resistant Nephrotic Syndrome: A Case Report.Case reports in nephrology · 2026Article
- Congenital Nephrotic Syndrome in Oman:Sultan Qaboos University medical journal · 2026Article
- Kidney transplantation outcomes in children with WT1-associated kidney disease: a single-center cohort study.Frontiers in pediatrics · 2026Article
- 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
- Expanded CRB2-related disease phenotype: multisystem involvement and post-transplant complications in monozygotic twins.Pediatric nephrology (Berlin, Germany) · 2025Article
- Transient Congenital Nephrotic Syndrome in Neonates: Two Case Reports and Review of Recent Literature.Cureus · 2025Article
- CRISPR and gene editing for kidney diseases: where are we?Clinical kidney journal · 2025Review
- Response to: Nephrectomy for congenital nephrotic syndrome: unanswered questions.Pediatric nephrology (Berlin, Germany) · 2025Article
- Nephrectomy for congenital nephrotic syndrome: unanswered questions.Pediatric nephrology (Berlin, Germany) · 2025Article
- Differences in kidney prognosis between congenital and infantile nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2025Article
- The clinical characteristics of patients with congenital nephrotic syndrome secondary to NPHS1 mutation: Is nephrectomy still a therapeutic option for selected cases?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
- Long-Term Outcomes in Nephrotic Syndrome by Kidney Biopsy Diagnosis and Proteinuria.Journal of the American Society of Nephrology : JASN · 2025Article
Corrections and comments
- Erratum issued
Authors and funding
12 authors at 10 institutions in 8 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Congenital nephrotic syndrome (CNS) is a heterogeneous group of disorders characterized by nephrotic-range proteinuria, hypoalbuminaemia and oedema, which manifest in utero or during the first 3 months of life. The main cause of CNS is genetic defects in podocytes; however, it can also be caused, in rare cases, by congenital infections or maternal allo-immune disease. Management of CNS is very challenging because patients are prone to severe complications, such as haemodynamic compromise, infections, thromboses, impaired growth and kidney failure. In this consensus statement, experts from the European Reference Network for Kidney Diseases (ERKNet) and the European Society for Paediatric Nephrology (ESPN) summarize the current evidence and present recommendations for the management of CNS, including the use of renin-angiotensin system inhibitors, diuretics, anticoagulation and infection prophylaxis. Therapeutic management should be adapted to the clinical severity of the condition with the aim of maintaining intravascular euvolaemia and adequate nutrition, while preventing complications and preserving central and peripheral vessels. We do not recommend performing routine early nephrectomies but suggest that they are considered in patients with severe complications despite optimal conservative treatment, and before transplantation in patients with persisting nephrotic syndrome and/or a WT1-dominant pathogenic variant.
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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 Socratic graph.