Overexpression of plasmalemmal vesicle-associated protein-1 in patient with cyanotic nephropathy: a case report
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
TL;DR
A case report shows possible overexpression of a protein linked to kidney damage in a patient with heart disease.
Contribution
First report of PAL-E staining in cyanotic nephropathy, suggesting PV-1 overexpression.
Findings
Renal biopsy showed glomerular hypertrophy and focal segmental glomerulosclerosis.
Electron microscopy revealed extensive endothelial cell damage.
PAL-E staining indicated possible PV-1 overexpression in the patient.
Abstract
Cyanotic nephropathy (CN) is a known complication of cyanotic congenital heart disease (CCHD). However, many aspects of its pathophysiology remain unclear. 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. 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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Taxonomy
TopicsRenal Diseases and Glomerulopathies · Neutrophil, Myeloperoxidase and Oxidative Mechanisms · Vasculitis and related conditions