Hereditary glomerulonephritis of non-Alport type.
In three unrelated kindreds, 21 subjects with hereditary renal disease were identified. The mode of inheritance was autosomal dominant and the main clinical features were asymptomatic proteinuria and/or haematuria. Three of the 16 females but none of the five males developed progressive renal failure. Renal biopsy carried out in six of the 21 patients showed varying degrees of mesangial change with glomerular deposition of IgM and C3 in some cases. Nerve deafness and renal ultrastructural changes typical of Alport's Syndrome were absent. The renal disease in these subjects does not conform with previously recognised types of familial nephropathy.
D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003176:Complement C3 / Q000032:analysis; D005260:Female; D005799:Genes, Dominant; D005921:Glomerulonephritis / Q000235:genetics*; D006417:Hematuria / Q000235:genetics; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis; D007676:Kidney Failure, Chronic / Q000235:genetics; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009394:Nephritis, Hereditary; D010375:Pedigree; D011507:Proteinuria / Q000235:genetics
D003176:Complement C3; D007075:Immunoglobulin M