Hereditary Angioedema (HAE) is a rare inherited disorder characterized by recurrent and self-limiting episodes of edema (swelling) of the skin, larynx (voice box), and gastrointestinal tract. There are two separate types of HAE (Type I and Type II). Depending on type, individuals have either mutant forms or low functional serum (blood) concentration of C1 esterase inhibitor (C1-INH).
Frequency of attacks varies among individuals and is not uniform within families. HAE attacks generally last 1-4 days. Therapies for HAE in the United States are currently limited. Medical management of HAE involves treatment of symptoms in acute attacks and maintenance therapy. Maintenance therapy consists of treatment with androgens (steroids that acts as a male sex hormone). However, their use is accompanied by significant side effects and long term safety has not been assessed.
Additional information about HAE is available at: www.haea.org
This is what an HAE attack looks like:
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