PATHOLOGY OF RENAL-ARTERIES OF DYSLIPIDEMIC CHILDREN WITH CONGENITAL NEPHROSIS

Congenital nephrotic syndrome of the Finnish type (CNF) is well charac terized in infants and associated with major lipid risk factors for at herosclerosis. This study was undertaken to investigate if any arteria l pathology is present in children with CNF and, if so, to describe it s nature in renal arteries collected at nephrectomy at a mean age of 1 2.5 +/- 4.4 months. Denuded endothelial injury and intimal thickening were seen in 9 out of 10 patient specimens of renal arteries. Intimal thickening contained loose abundant extracellular matrix with a few sm ooth muscle or myofibroblastoid cells. Only a few Sudan black- or oil red O-positive lipid droplets were found in six and seven samples, res pectively. Areas immunoreactive with antibodies against apoprotein B w ere seen in only two specimens. Immunohistochemistry did not reveal an y activated T or B cells, or any expression of IL-1 or IL-2 receptors. Macrophages were present in only two specimens. No foam cells were se en. We conclude that the vascular pathology together with altered lipo protein metabolism indicates that children with CNF might be at risk f or early atherosclerotic arterial disease, particularly if their hyper lipidemia persists.