Mesenteric arterial thrombosis complicating congenital nephrotic syndrome of Finnish type: report of one case.

Abstract

A female, term neonate presented with generalized edema, heavy proteinuria, hypoalbuminemia and hyperlipidemia in the second week of life. The clinical and laboratory features were compatible with the diagnosis of congenital nephrotic syndrome. Treatment included albumin infusion, empirical penicillin, steroid and continuous arterio-venous hemofiltration. Intestinal perforation developed at the 19th day of age and led to a fatal outcome. At autopsy, thrombosis of the superior mesenteric artery and its branches was noted, and histology of the kidney was compatible with congenital nephrotic syndrome of the Finnish type. The risk of thromboembolism, arterial or venous, should be considered in patients with nephrotic syndrome, even in the neonatal period. Preventive measures, including avoiding volume depletion and femoral arterial/venous puncture, are essential in managing these patients. Prophylactic anticoagulation and infusion of fresh frozen plasma may be warranted to avoid such potentially lethal complications of thromboembolism.