Glomerular epithelial cell phenotype in diffuse mesangial sclerosis: A report of 2 cases with markedly increased urinary podocyte excretion

Yohei Ikezumi, Toshiaki Suzuki, Tamaki Karasawa, Utako Kaneko, Takeshi Yamada, Hiroya Hasegawa, Michio Nagata, Akihiko Saitoh

Research output: Contribution to journalArticle

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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.

Original languageEnglish
Pages (from-to)1778-1783
Number of pages6
JournalHuman Pathology
Volume45
Issue number8
DOIs
Publication statusPublished - 01-01-2014

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Podocytes
Epithelial Cells
Phenotype
Denys-Drash Syndrome
Claudin-1
Staining and Labeling
Wilms Tumor
Sclerosis
Proteinuria
Fluorescent Antibody Technique
Cell Proliferation
Diffuse mesangial sclerosis
Urine
Mutation

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine

Cite this

Ikezumi, Yohei ; Suzuki, Toshiaki ; Karasawa, Tamaki ; Kaneko, Utako ; Yamada, Takeshi ; Hasegawa, Hiroya ; Nagata, Michio ; Saitoh, Akihiko. / Glomerular epithelial cell phenotype in diffuse mesangial sclerosis : A report of 2 cases with markedly increased urinary podocyte excretion. In: Human Pathology. 2014 ; Vol. 45, No. 8. pp. 1778-1783.
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Glomerular epithelial cell phenotype in diffuse mesangial sclerosis : A report of 2 cases with markedly increased urinary podocyte excretion. / Ikezumi, Yohei; Suzuki, Toshiaki; Karasawa, Tamaki; Kaneko, Utako; Yamada, Takeshi; Hasegawa, Hiroya; Nagata, Michio; Saitoh, Akihiko.

In: Human Pathology, Vol. 45, No. 8, 01.01.2014, p. 1778-1783.

Research output: Contribution to journalArticle

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