Hereditary angioneurotic edema (HANE): Lack of close linkage between HLA haplotypes and C1 esterase inhibitor deficiency.
The HLA haplotypes were defined in a series including 11 patients with hereditary angioneurotic edema (HANE), five symptom-free subjects with pathological laboratory findings characteristic of HANE, and their 33 healthy kinsmen. The subjects belonged to two Finnish families representing the more common form of HANE which is recognized by the immunochemically observed decrease of the inhibitor (C1-INH) blocking the esterase activity of the first complement component. No linkage between HANE and the HLA system was found.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000799:Angioedema / Q000235:genetics* / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D003172:Complement C1; D003165:Complement System Proteins; D004950:Esterases / Q000037:antagonists & inhibitors / Q000172:deficiency; D005260:Female; D008040:Genetic Linkage; D006680:HLA Antigens; D006238:Haploidy; D006649:Histocompatibility Antigens; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010375:Pedigree
D003172:Complement C1; D006680:HLA Antigens; D006649:Histocompatibility Antigens; D003165:Complement System Proteins; D004950:Esterases