[Study of a large family with hereditary angioneurotic edema].
A large kindred (156 members) with hereditary angioneurotic edema is reported. 44 members of the family exhibit typical symptoms of the disease: recurring edemas of the skin and episodes of abdominal pain accompanied by vomiting due to mucosal edema in the stomach and intestine. In 32 patients complement studies were performed which in  28 cases revealed decreased levels of C1-inhibitor and almost normal values for C3 concentration. Among 21 members of the family who had never had symptoms of the disease, 7 also had low levels of C1-inhibitor. In 6 of 16 women, attacks often occur in conjunction with menstruation. In 9 of 10 women the symptoms of the disease had worsened during their first pregnancy. 8 patients have been successfully treated with tranexamic acid for 7-20 months.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000799:Angioedema / Q000188:drug therapy / Q000235:genetics*; D003172:Complement C1; D003176:Complement C3; D003169:Complement Inactivator Proteins; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D014148:Tranexamic Acid / Q000627:therapeutic use
D003172:Complement C1; D003176:Complement C3; D003169:Complement Inactivator Proteins; D014148:Tranexamic Acid
Untersuchung einer grossen Sippe mit hereditären angioneurotischem Odem