[Hereditary angioedema: description of a sibship and review].
Report on a newly recognized sibship with hereditary angioedema (HAE). The 27 persons investigated include 8 with decreased concentrations of C-1INH and C4 in the Serum. Three of these are children without symptoms of HAE. Five patients have characteristic attacks of HAE, some of which are predominantly abdominal. The effects of treatment with danazol in 4 patients are described. HAE is briefly reviewed with special reference to pathogenesis and treatment.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000799:Angioedema / Q000235:genetics* / Q000276:immunology; D002648:Child; D003166:Complement Activating Enzymes / Q000032:analysis; D003174:Complement C1 Inactivator Proteins / Q000032:analysis; D015922:Complement C1q; D003176:Complement C3 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D003165:Complement System Proteins / Q000032:analysis*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree
D003174:Complement C1 Inactivator Proteins; D003176:Complement C3; D003181:Complement C4; D015922:Complement C1q; D003165:Complement System Proteins; D003166:Complement Activating Enzymes
Das hereditäre Angioödem: Beschreibung einer Sippe und Ubersicht.