ArticlePediatric nephrology (Berlin, Germany)2017
IgA nephropathy with presentation of nephrotic syndrome at onset in children.
Article in Pediatric nephrology (Berlin, Germany), 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers, 1 of them a synthesis that pooled it.
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Who cites it
19 citing papers in PubMed, 1 synthesis or guideline pooled it, 32 citations in OpenAlex.
- IPNA clinical practice recommendations for the diagnosis and management of children with IgA nephropathy and IgA vasculitis nephritis.Pediatric nephrology (Berlin, Germany) · 2025Guideline
- Promoting clinical trials in pediatric IgA nephropathy through extrapolation: an artificial intelligence-enhanced literature review of the disease in pediatrics sponsored by the Kidney Health Initiative.Pediatric nephrology (Berlin, Germany) · 2026Review
- Analysis of changes in glomerular lesions in children diagnosed with IgA nephropathy following immunosuppressive therapy.BMC nephrology · 2026Article
- Successful Remission of Nephrotic Syndrome Caused by IgA Nephropathy with Membranoproliferative Glomerulonephritis-pattern Lesion Treated by Cyclosporine and Oral Corticosteroids.Internal medicine (Tokyo, Japan) · 2026Article
- Comparative Analysis of Clinical and Pathological Traits in IgA Nephropathy Patients with Nephrotic Syndrome or Nephrotic Range Proteinuria.Kidney & blood pressure research · 2026Article
- Clinical features, pathological characteristics, and prognosis of patients with IgA nephropathy complicated with nephrotic syndrome.Scientific reports · 2025Article
- Is Childhood IgA Nephropathy Different From Adult IgA Nephropathy? A Narrative Review.Canadian journal of kidney health and disease · 2025Review
- Differences of clinicopathological characteristics and outcomes of IgA nephropathy patients with and without nephrotic syndrome.International urology and nephrology · 2024Article
- Are children with IgA nephropathy different from adult patients?Pediatric nephrology (Berlin, Germany) · 2024Article
- IgA nephropathy in a boy with frequently relapsing nephrotic syndrome.CEN case reports · 2024Article
- Distinct characteristics and prognosis of IgA nephropathy patients with nephrotic syndrome: a propensity score-matched cohort study.Frontiers in medicine · 2024Article
- Cell-type-specific molecular characterization of cells from circulation and kidney in IgA nephropathy with nephrotic syndrome.Frontiers in immunology · 2023Article
- Efficacy of combination therapy for childhood complicated focal IgA nephropathy.Clinical and experimental nephrology · 2022Article
- Involvement of Hemopexin in the Pathogenesis of Proteinuria in Children with Idiopathic Nephrotic Syndrome.Journal of clinical medicine · 2021Article
- IgA nephropathy in children and in adults: two separate entities or the same disease?Journal of nephrology · 2020Review
- Remission of Proteinuria May Protect against Progression to Chronic Kidney Disease in Pediatric-Onset IgA Nephropathy.Journal of clinical medicine · 2020Article
- Steroid therapy in children with IgA nephropathy.Pediatric nephrology (Berlin, Germany) · 2020Review
- Clinical and pathological features of immunoglobulin A nephropathy patients with nephrotic syndrome.Clinical and experimental medicine · 2019Observational
- Nephropathy in a Child with Severe Recessive Dystrophic Epidermolysis Bullosa Treated with Cyclophosphamide: A Case Report.Case reports in nephrology and dialysisArticle
Corrections and comments
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Authors and funding
12 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundDespite a low incidence, nephrotic syndrome (NS) can present with IgA nephropathy (IgAN). The clinical characteristics and long-term outcomes of pediatric patients with IgAN presenting with NS (NS-IgAN) at onset have not been fully elucidated.
methodsWe retrospectively analyzed 426 patients, and compared clinical and pathological (Oxford) findings between those with NS-IgAN and those with non-NS-IgAN.
resultsAmong 426 patients, 30 (7.0 %) had NS-IgAN. Logistic analyses showed that male sex (OR: 7.6, p = 0.0002), M1 (OR: 10.3, p = 0.002), and E1 (OR: 15.2, p = 0.0001) were significantly related to NS. The mean observation period was 6.2 ± 3.2 years. Although NS-IgAN was associated with significantly lower renal survival than non-NS-IgAN according to Kaplan-Meier analysis (p = 0.02), renal survival of NS-IgAN was good (92.4 % at 10 years). The most significant prognostic factor for renal survival was remission of proteinuria after treatment, and NS at onset is also a significant prognostic factor for renal survival after adjusting for remission of proteinuria. Twenty children with NS-IgAN were treated with prednisolone alone, or prednisolone and immunosuppressant. Remission of proteinuria occurred in 21 patients. Three cases of NS-IgAN progressed to stage III-V chronic kidney disease at the most recent observation. They all demonstrated heavy proteinuria after the 2-year initial treatment. The significant factor for persistent proteinuria at 5 years was S1 in NS-IgAN.
conclusionsThe most significant factor for renal survival was responsiveness to treatment, not NS itself. As modifiable acute lesions are the dominant pathological findings in NS-IgAN, histological improvements achieved by appropriate treatments can result in a favorable prognosis.
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