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査読

1 被引用数 (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.

本文言語English
ページ(範囲)1778-1783
ページ数6
ジャーナルHuman Pathology
45
8
DOI
出版ステータスPublished - 08-2014
外部発表はい

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine

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