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Pediatric membranous nephropathy: In the novel antigens era
Guoping Huang1, Fei Liu1, Ling Yu1
1Department of Nephrology, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center For Child Health, Hangzhou, China.
Abstract:
Membranous nephropathy (MN) falls within the scope of a glomerular disease. MN exhibits subepithelial immune- complex deposition and capillary wall thickening which could occur in all age groups. In comparison with adult patients with MN, MN in pediatric population has a lower incidence and more secondary factors (e.g., systemic lupus erythematosus, infection, malignancy, or drug toxicity). Two target antigens for the immune complexes, PLA2R (identified in 2009) and THSD7A (in 2014), found in previous studies and first presented in adult MN, are found in pediatric patients suffering from MN and their antibodies are now an effective tool for diagnosis and monitoring in children and adolescents. Several novel antigens have been identified (e.g., EXT1/EXT2, NELL1, Sema3B, PCDH7, HTRA1, and NCAM1) over the past few years. Each of them represents different clinical and pathologic findings. In-depth research should be conducted to gain insights into the outcomes and pathophysiology of the above novel antigen-associated MN. Targeted treatment opinions for different novel antigen-related MN are under development both in adults and pediatric patients.
Insights
Membranous nephropathy (MN) is a glomerular disease affecting all ages. Pediatric MN is less common and often secondary, but new antigens like PLA2R and THSD7A are key diagnostic tools.
Area of Science:
- Nephrology
- Immunology
- Pediatric Nephrology
Background:
- Membranous nephropathy (MN) is a glomerular disease characterized by subepithelial immune complex deposition and capillary wall thickening.
- While MN occurs in all age groups, pediatric MN is less frequent and more often associated with secondary causes compared to adult MN.
- Recent advancements have identified specific target antigens, such as Phospholipase A2 Receptor (PLA2R) and Thrombospondin Type-1 Domain Containing 7A (THSD7A), crucial for diagnosis and monitoring.
Purpose of the Study:
- To review the current understanding of membranous nephropathy in pediatric patients.
- To highlight the role of identified antigens (PLA2R, THSD7A) and novel antigens in pediatric MN.
- To emphasize the need for further research into novel antigen-associated MN and targeted therapies.
Main Methods:
- Literature review of pediatric membranous nephropathy.
- Analysis of identified target antigens (PLA2R, THSD7A) in pediatric cases.
- Discussion of recently discovered novel antigens and their clinical implications.
Main Results:
- PLA2R and THSD7A antibodies are effective diagnostic and monitoring tools in pediatric MN.
- Several novel antigens (EXT1/EXT2, NELL1, Sema3B, PCDH7, HTRA1, NCAM1) have been identified, each associated with distinct clinical and pathological features.
- These novel antigens offer new avenues for understanding and potentially treating pediatric MN.
Conclusions:
- Pediatric MN diagnosis and monitoring are significantly aided by understanding antibodies to specific antigens like PLA2R and THSD7A.
- The identification of novel antigens in MN necessitates further investigation into their specific pathophysiology and clinical outcomes.
- Developing targeted treatment strategies for novel antigen-related MN in both pediatric and adult populations is an ongoing area of research.
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