Overexpression of plasmalemmal vesicle-associated protein-1 in patient with cyanotic nephropathy: a case report.

IF 2.2 4区医学 Q2 UROLOGY & NEPHROLOGY BMC Nephrology Pub Date : 2025-03-03 DOI:10.1186/s12882-025-04046-x

Yusuke Ushio, So Hirata, Shun Manabe, Mayuko Suyama, Ayano Tanaka, Momoko Seki, Haruka Kato, Kana Nomura, Anna Nakai, Hitoko Sumori, Yuki Kawaguchi, Shizuka Kobayashi, Shiho Makabe, Hiroshi Kataoka, Naoko Itoh, Sekiko Taneda, Kazuho Honda, Junichi Hoshino

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Abstract

Background: Cyanotic nephropathy (CN) is a known complication of cyanotic congenital heart disease (CCHD). However, many aspects of its pathophysiology remain unclear.

Case presentation: We report the case of a 29-year-old male with a history of tetralogy of Fallot. Renal biopsy revealed glomerular hypertrophy and focal segmental glomerulosclerosis. Electron microscopy revealed extensive endothelial cell damage. To investigate the etiology of endothelial cell damage, PAL-E staining was conducted, revealing staining along the glomerular capillary wall.

Conclusion: This is the first report of PAL-E staining in CN, suggesting potential overexpression of PV-1. The association of PV-1 expression with endothelial cell damage indicates its role in the pathogenesis of CN.

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来源期刊

BMC Nephrology UROLOGY & NEPHROLOGY-

CiteScore

4.30

自引率

0.00%

发文量

375

审稿时长

3-8 weeks

期刊介绍： BMC Nephrology is an open access journal publishing original peer-reviewed research articles in all aspects of the prevention, diagnosis and management of kidney and associated disorders, as well as related molecular genetics, pathophysiology, and epidemiology.