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Hereditary Angioedema

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Î Treatment with replacement plasma-derived C1INH provides effective and safe long-term HAE prophylaxis. (A) Î The novel agents for treatment of patients with C1INH deficiency syndromes are more costly than alternative treatment with attenuated androgens. Formal studies of cost utility and cost-effectiveness are required to aid providers in the management of patients with C1INH deficiency syndromes. (D) Î Treatment with low to moderate doses of anabolic androgens provides effective and relatively safe long-term HAE prophylaxis for many patients. (B) Î Treatment with antifibrinolytic agents provides somewhat effective and relatively safe long-term HAE prophylaxis but is generally less effective than androgens. (B) Î Short-term prophylaxis can be achieved with fresh frozen plasma, C1INH replacement or short-term high dose anabolic androgen therapy. (B) Î The need for long-term HAE prophylaxis must be individualized based on the patient's situation. (D) Î The dose and effectiveness of long-term prophylaxis should be based on clinical criteria, not laboratory parameters. (C) Î Adjunctive strategies such as avoidance of ACE-Is, avoidance of estrogen therapy as feasible, and stress reduction are important to decrease the frequency and severity of HAE attacks. (D) Î Mechanisms of action of 17α-alkylated androgen and antifibrinolytic drugs for HAE have not been completely elucidated. (D) Î Pregnancy might be associated with an increase in the frequency and severity of HAE episodes. For long-term prophylaxis during pregnancy, treatment with androgens is contraindicated, and plasma-derived C1INH is preferred (D). Prevention

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