ReviewPediatric nephrology (Berlin, Germany)2022
Clinicopathologic features of non-lupus membranous nephropathy in a pediatric population.
Review in Pediatric nephrology (Berlin, Germany), 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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15 citing papers in PubMed, 21 citations in OpenAlex.
- Risk stratification guided treatment and outcomes of pediatric membranous nephropathy.Pediatric nephrology (Berlin, Germany) · 2026Observational
- Semaphorin 3B-Membranous Nephropathy in Adults.Kidney international reports · 2026Article
- EXT2 transition in adolescent membranous nephritis.Pediatric nephrology (Berlin, Germany) · 2026Article
- Antigens in membranous nephropathy: discovery and clinical implications.Nature reviews. Nephrology · 2025Review
- Questions and Caveats in Antigen-Defined Membranous Nephropathy.Journal of the American Society of Nephrology : JASN · 2025Review
- A rare case of late-onset immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome confused with IgA vasculitis nephropathy.Pediatric nephrology (Berlin, Germany) · 2025Article
- The Prevalence, Characteristics, and Putative Mechanisms of Dual Antigen-Positive Membranous Nephropathy: The Underestimated Condition.International journal of molecular sciences · 2024Review
- NELL1 membranous nephropathy: clinical associations provide mechanistic clues.Frontiers in nephrology · 2024Review
- How to Choose the Right Treatment for Membranous Nephropathy.Medicina (Kaunas, Lithuania) · 2023Review
- Clinical and Histopathologic Characteristics of Pediatric Patients With Primary Membranous Nephropathy.Kidney international reports · 2023Article
- The Antigenic Landscape of Pediatric Membranous Nephropathy.Kidney international reports · 2023Article
- Delayed skeletal development and IGF-1 deficiency in a mouse model of lysinuric protein intolerance.Disease models & mechanisms · 2023Article
- The Many Faces of NELL1 MN.Clinical kidney journal · 2023Review
- A girl with membranous nephropathy associated with ventriculoperitoneal shunt infection.CEN case reports · 2023Article
- Pediatric membranous nephropathy: In the novel antigens era.Frontiers in immunology · 2022Review
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Authors and funding
6 authors at 2 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundMembranous nephropathy is an uncommon cause of nephrotic syndrome in pediatrics.
methodsWe reviewed our kidney biopsy records for patients ≤ 20 years of age with membranous nephropathy without evidence of systemic lupus erythematosus within 6 months of biopsy (January 1995-September 2020). Staining for PLA2R, NELL1, THSD7A, SEMA3B, EXT2 (3 biopsies), and IgG-subclass were performed.
resultsSixteen children (≤ 12 years) and 25 adolescents (13-20 years) were identified. Four children and 15 adolescents showed autoantigen positivity: PLA2R+/SEMA3B- (13), SEMA3B+/PLA2R+ (2), SEMA3B+/PLA2R- (1), NELL1 (1), EXT2+ (2), and THSD7A (0). Co-morbidities associated with PLA2R positivity included IPEX syndrome, active hepatitis B, Von Hippel Lindau syndrome, solitary kidney, type 1 diabetes, hyperuricemia, pregnancy (1), obesity (3), type II diabetes, H. pylori, viral prodrome, and nephrolithiasis. The SEMA3B+/PLA2R- adolescent was pregnant, the NELL1+ adolescent was obese, and the two EXT2+ adolescents eventually met the clinical criteria for lupus (4, 9 years post-biopsy). Co-morbidities among the remaining 24 patients included remote hepatitis B (2), Down's syndrome, lysinuric protein intolerance, recurrent UTIs, hypothyroidism, pregnancy (3), and obesity (2). Follow-up data was available for 12 children and 16 adolescents. Of the 12 children, 6 achieved complete remission, 4 achieved partial remission, and 2 had no response to treatment (1 transplant). Of the 16 adolescents, 4 achieved complete remission, 4 achieved partial remission, and 8 had no response to treatment (3 transplants). A child with "full-house" immunofluorescence staining achieved spontaneous disease remission.
conclusionOur non-lupus membranous nephropathy cohort represents one of the largest pediatric studies to date. A higher resolution version of the Graphical abstract is available as Supplementary information.
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