Hereditary Nephritis With the Nephrotic Syndrome

Abstract

The nephrotic syndrome defined as a process characterized by increased glomerular membrane permeability manifested by proteinuria of greater than 3.5 gm/24 hr/1.73 sq m body surface was found in five of 15 patients from six families with hereditary nephritis and deafness. Edema and hypercholesterolemia were rarely found perhaps because most patients were young and had sufficient hepatic synthetic capacity to compensate for urinary protein wasting. Failure to quantify daily protein excretion probably accounts for the paucity of reports of the nephrotic syndrome occurring during the course of hereditary nephritis.