C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma. The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.
This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system. The disease is characterized by acute attacks of painful, and in some cases, fatal swelling of several soft tissues or edema, which may last up to five days when untreated.
Intravenous and subcutaneous formulations of the human C1-esterase inhibitor are indicated for routine prophylaxis against acute attacks of hereditary angioedema in patients six years of age and older. It is also used to treat these in adult and adolescent patients with hereditary angioedema.
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