Post-transplantation nephrosis in congenital nephrotic syndrome of the Finnish type

摘要: Post-transplantation nephrosis in congenital nephrotic syndrome of the Finnish type. Congenital type (CNF) is an autosomal recessively inherited disease manifesting as massive proteinuria, edema and ascites neonatal period. The believed to be limited kidneys recurrences after renal transplantation have not been reported. At our center 29 transplantations performed on 28 CNF patients. One 33 months transplantation, seven grafts (24%) six patients developed a steroid-resistant syndrome. clinical data histology these were analyzed order elucidate cause proteinuria. onset episodes nephrosis, patient had evidence preceding CMV- or EBV-infection remaining sinusitis. Upon light electron microscopy examination, endothelial swelling glomerular capillaries resembling transplant glomerulopathy (TG) was seen, but unlike TG, basement membranes normal. response proteinuria steroid cyclophosphamide therapy poor, with total remission only two partial one patient, all treated methylprednisolone immediately diagnosis. Four lost. Our show that increased tendency for post-transplantation nephrosis.