[Hereditary angioedema].
Hereditary angio-oedema (AOH) is a familial affliction that is most often hereditary, but sometimes acquired and linked to a deficiency of C1 esterase inhibitor. 8 cases have been examined, amongst which there was one attack in a young girl, without any clinical signs. Amongst the other 7, one had acquired AOH. The diagnosis was confirmed by the deficiency of C1 esterase inhibitor, associated with a significant reduction of the other fraction of complement c3-c4 and also total haemolytic complement. Strong doses of corticosteroids were used in 4 patients for urgent treatment. Basic treatment with DANAZOL was commenced in 3 patients.
Sabbah
A
A
Laboratoire d'Immuno-Allergologie CHRU Angers.
Marsollier
I
I
Trouillard
V
V
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000799:Angioedema / Q000097:blood / Q000188:drug therapy / Q000235:genetics; D002648:Child; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000235:genetics; D003165:Complement System Proteins / Q000172:deficiency; D003613:Danazol / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male
D000305:Adrenal Cortex Hormones; D003174:Complement C1 Inactivator Proteins; D003165:Complement System Proteins; D003613:Danazol
L'angio-oedème héréditaire (AOH).