Overexpression of Plasmalemmal Vesicle-Associated Protein-1 in a Case of Cyanotic Nephropathy

Author:

Ushio Yusuke1,Hirata So1,Manabe Shun1,Suyama Mayuko1,Tanaka Ayano1,Seki Momoko1,Kato Haruka1,Nomura Kana1,Nakai Anna1,Sumori Hitoko1,Kawaguchi Yuki1,Kobayashi Shizuka1,Makabe Shiho1,Kataoka Hiroshi1,Itoh Naoko1,Taneda Sekiko1,Honda Kazuho2,Hoshino Junichi1

Affiliation:

1. Tokyo Women’s Medical University

2. Showa University

Abstract

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. For 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. Previous studies have linked PV-1 expression to endothelial cell damage, implicating it in the pathogenesis of CN.

Publisher

Springer Science and Business Media LLC

Reference15 articles.

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3. Mechanisms of development and progression of cyanotic nephropathy;Inatomi J;Pediatr Nephrol,2006

4. Glomerular alterations in cyanotic congenital heart disease;Spear GS;Bull Johns Hopkins Hosp,1960

5. Pathogenesis of the glomerular abnormality in cyanotic congenital heart disease;Perloff JK;Am J Cardiol,2000

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