Hereditary angio-oedema: treatment with danazol. Report of a case.
An 8-year-old boy with hereditary angio-oedema was treated with danazol under close endocrinological supervision. The boy's C1 esterase inhibitor (C1inh) and C4 levels increased rapidly to near normal values under a daily dose of 400 mg and were maintained at about 50% of the normal by maintenance doses of 200 mg danazol every other day. Throughout the entire treatment period (11 months) the boy has been maintained free from attacks of angio-oedema. No hormonal imbalance was detected in the follow-up period. Our results indicate that danazol should be suitable for the treatment of HAE not only in adults but also in prepubescent children.
D000799:Angioedema / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology; D002648:Child; D003174:Complement C1 Inactivator Proteins / Q000097:blood; D003181:Complement C4 / Q000032:analysis; D003613:Danazol / Q000008:administration & dosage / Q000627:therapeutic use*; D004334:Drug Administration Schedule; D006801:Humans; D008297:Male; D010375:Pedigree; D011245:Pregnadienes / Q000627:therapeutic use*
D003174:Complement C1 Inactivator Proteins; D003181:Complement C4; D011245:Pregnadienes; D003613:Danazol