Hereditary angioedema attacks, regardless of their site, do not respond to antihistamines, corticosteroids or epinephrine. In most instances, the attacks can be prevented with attenuated androgens (danazol, stanozolol), or, somtimes with antiﬁbrinolytic agents (tranexamic acid). Their administration is recomanded previous to dental extractions, tonsilectomies and adenoidectomies. Their use is not alowed before puberty and during premenopause.
The treatments avialable today for hereditary angioedema attacks include: C1-INH concentrate, recombinant human C1-inhibitor, a bradykinin and a kallikrein receptor antagonist. When these are not available, fresh frosen plasma can be effectively used.
Hereditary angioedema patients need to be reffered to a specialised centre. Patient education and communication between the refference center and medical profesionals in the teritory (family practitioners, NTE specialists, alergollogists, dermatologists etc) is crucialy important for the successful management and survival of patients suffering from this rare genetic disorder.
Last update: 11.05:2016