A new form of familial glomerulonephritis.
An unusual familial glomerular disease, characterized by the presence of diffuse round mesangial deposits of C3, is described in 2 siblings (1 male and 1 female) and their mother. The clinical picture in the 3 patients was a long-lasting proteinuria. An acute hemolytic uremic syndrome with malignant hypertension developed in the male at the age of 24 years, requiring bilateral nephrectomy. The glomerulonephritis recurred on a renal allograft. This disease is not HLA-linked and no characteristic abnormality of complement profile was seen in the 3 patients.
D000293:Adolescent; D000328:Adult; D003176:Complement C3 / Q000276:immunology*; D005260:Female; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000235:genetics* / Q000276:immunology; D006680:HLA Antigens / Q000276:immunology; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics; D006801:Humans; D006974:Hypertension, Malignant / Q000235:genetics; D007678:Kidney Glomerulus / Q000276:immunology*; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged
D003176:Complement C3; D006680:HLA Antigens