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Hum Pathol. 2014 Aug;45(8):1778-83. doi: 10.1016/j.humpath.2014.03.017. Epub 2014 Apr 18.

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

Author information

1
Department of Pediatrics, Niigata University Medical and Dental Hospital, 1-784 Asahimachi-dori, Cyuo-ku, Niigata, 951-8520, Japan. Electronic address: ikezumi@med.niigata-u.ac.jp.
2
Department of Pediatrics, Niigata University Medical and Dental Hospital, 1-784 Asahimachi-dori, Cyuo-ku, Niigata, 951-8520, Japan.
3
Department of Kidney and Vascular Pathology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, 305-8575, Japan.

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.

KEYWORDS:

Denys-Drash syndrome; Diffuse mesangial sclerosis; Epithelial hyperplasia; Parietal epithelial cell; Podocyte; Urinary podocyte

PMID:
24856573
DOI:
10.1016/j.humpath.2014.03.017
[Indexed for MEDLINE]

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