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

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.