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Updated: Apr 29, 2026
Glomerular Outgrowth as an Ex Vivo Assay to Analyze Pathways Involved in Parietal Epithelial Cell Activation
Published on: August 19, 2020
Glomerular epithelial cell phenotype in diffuse mesangial sclerosis: a report of 2 cases with markedly increased
Yohei Ikezumi1, Toshiaki Suzuki1, Tamaki Karasawa1
1Department of Pediatrics, Niigata University Medical and Dental Hospital, 1-784 Asahimachi-dori, Cyuo-ku, Niigata, 951-8520, Japan.
Insights
Diffuse mesangial sclerosis (DMS) involves severe podocyte loss and parietal cell proliferation. These cellular changes are key to understanding DMS pathogenesis in pediatric kidney disease.
Area of Science:
- Nephrology
- Pediatric Pathology
- Renal Cell Biology
Background:
- Diffuse mesangial sclerosis (DMS) is a severe glomerular disease often presenting with nephrotic syndrome.
- WT1 mutations are associated with Denys-Drash syndrome, characterized by Wilms tumor and kidney abnormalities.
Observation:
- Two pediatric cases of DMS were analyzed, one with a WT1 mutation and Wilms tumor, the other with heavy proteinuria.
- Histological examination revealed significant podocyte loss in sclerotic glomeruli, with remaining podocytes in less affected areas.
Findings:
- Immunohistochemistry showed reduced synaptopodin (podocyte marker) in sclerotic glomeruli.
- Proliferating cells surrounding sclerotic lesions stained positive for claudin-1 (parietal cell marker) and negative for synaptopodin.
Implications:
- Podocyte depletion and subsequent parietal cell proliferation appear to be crucial mechanisms in the development of DMS.
- Understanding these cellular dynamics may offer new therapeutic targets for pediatric kidney diseases like DMS.
Abstract:
We report 2 cases of diffuse mesangial sclerosis (DMS) accompanied by severe podocyte excretion in urine. Patient 1 was a 9-day-old girl with a WT1 mutation who developed Wilms tumor at 6 months of age and was subsequently diagnosed with Denys-Drash syndrome. Patient 2 was a 1-year-old boy without a WT1 abnormality but presenting with heavy proteinuria. In both patients, histological examination showed findings of DMS. Immunohistochemical staining for synaptopodin (a podocyte marker) revealed a reduced number of podocytes in the glomeruli with severe sclerosis; however, podocytes persisted in the relatively intact glomeruli. Some glomeruli were accompanied by sclerotic lesions surrounded by proliferating cells; immunofluorescence staining revealed a majority of these proliferating cells to be positive for claudin-1 (a parietal cell marker) but negative for synaptopodin. These findings suggest that podocyte loss and the consequent proliferation of parietal cells are common processes in the pathogenesis of DMS.
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