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

研究成果: Article

1 引用 (Scopus)

抄録

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.

元の言語English
ページ(範囲)1778-1783
ページ数6
ジャーナルHuman Pathology
45
発行部数8
DOI
出版物ステータスPublished - 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

これを引用

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. :: Human Pathology. 2014 ; 巻 45, 番号 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.

:: Human Pathology, 巻 45, 番号 8, 01.01.2014, p. 1778-1783.

研究成果: Article

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