[Angioneurotic hereditary edema. 3 faimilial cases].
The study involved three families with hane. This disorder differs from the simple condition by its preferential visceral localisation (digestive and, above all, the glottis), its hereditary nature and the existence of a biological abnormality affecting the serum complement system (absence of enzyme activity of C1 esterase inhibitor in the serum of sufferers). The cases reported illustrate the serious prognosis of this condition (four deaths by asphyxia in one family) in the absence of specific therapy. This disorder, although rare, represents a fundamental problem in general pathology by virtue of the enzymatic, immunological and genetic processes which it involves.
D000293:Adolescent; D000799:Angioedema / Q000175:diagnosis / Q000235:genetics*; D003172:Complement C1 / Q000172:deficiency*; D003175:Complement C2 / Q000032:analysis; D003176:Complement C3 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D003165:Complement System Proteins / Q000172:deficiency*; D004064:Digestive System; D004950:Esterases / Q000037:antagonists & inhibitors; D005260:Female; D005931:Glottis; D006801:Humans; D008297:Male; D008875:Middle Aged; D014666:Vasomotor System / Q000503:physiopathology
D003172:Complement C1; D003175:Complement C2; D003176:Complement C3; D003181:Complement C4; D003165:Complement System Proteins; D004950:Esterases
L'oedème angioneurotique héréditaire. Trois observations familiales