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.