[Immune complex nephropathy and hereditary deficiency of C1 esterase inhibitor (author's transl)].
Deficiency of C1 esterase inhibitor (C1-INH) was demonstrated in 7 of 22 subjects belonging to the same French family. Immune complex glomerulonephritis without lupic symptoms was discovered in one of the C1-INH deficient subjects, and in a girl of the same family the same deficiency was associated with an insulin-dependent diabetes of sudden onset. The pathophysiological consequences of complement deficiency resulting from the lack of C1 esterase inhibitor are discussed.
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000235:genetics; D005260:Female; D005921:Glomerulonephritis / Q000235:genetics / Q000276:immunology*; D006801:Humans; D007105:Immune Complex Diseases / Q000235:genetics*; D008297:Male; D008875:Middle Aged
D003174:Complement C1 Inactivator Proteins
Néphropathie à complexes immuns et déficit héréditaire en inhibiteur de la C1 estérase.