Inherited complement component deficiencies in membranoproliferative glomerulonephritis.
Anecdotal reports of complement component deficiencies in patients with immune complex disease led to a systematic study of the levels of seven complement components in serum specimens from 178 patients with glomerulonephritis and 163 normal subjects. Deficiencies were found with significantly higher frequency (22.7%) among 44 patients with membranoproliferative glomerulonephritis (MPGN) types I and III, than among the normal subjects (6.7%, P less than 0.002) or among 134 patients with other glomerulonephritides (5.2%, P less than 0.001). The component deficiencies in MPGN were partial in nine patients and subtotal in one. They could not be ascribed to acquired hypocomplementemia or to a nephrotic syndrome. They were present over long periods, were found in family members, and involved C2, C3, factor B, C6, C7, and C8. Six were presumably the result of null structural genes, two were associated with a structurally abnormal component, and two were of unknown cause. The results give evidence that partial deficiency of one or more complement components is a factor predisposing to MPGN.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003165:Complement System Proteins / Q000097:blood / Q000172:deficiency*; D005260:Female; D005921:Glomerulonephritis / Q000235:genetics* / Q000276:immunology; D006801:Humans; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D010375:Pedigree
D003165:Complement System Proteins