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

IF 2.4 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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紫变肾病患者浆乳囊泡相关蛋白-1过表达1例

背景：紫绀型肾病（CN）是一种已知的紫绀型先天性心脏病（CCHD）的并发症。然而，其病理生理的许多方面仍不清楚。病例介绍：我们报告一例29岁男性法洛四联症病史。肾活检显示肾小球肥大和局灶节段性肾小球硬化。电镜显示广泛的内皮细胞损伤。为了研究内皮细胞损伤的病因，我们进行了PAL-E染色，显示肾小球毛细血管壁的染色。结论：这是CN中首次报道PAL-E染色，提示PV-1可能过表达。PV-1表达与内皮细胞损伤的关联表明其在CN发病机制中的作用。

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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.