The Medical Letter on Drugs and Therapeutics
A Recombinant C1 Esterase Inhibitor (Ruconest) for Hereditary Angioedema (online only)
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The FDA has approved Ruconest (Salix), a recombinant analog of human complement component 1 esterase inhibitor (C1INH), for treatment of acute attacks in patients with hereditary angioedema (HAE).

THE DISEASE — HAE, a rare autosomal dominant disorder (estimated prevalence 1:10,000-50,000), is usually caused by a mutation of the C1-inhibitor gene. C1INH suppresses the activity of the serine protease plasma kallikrein, preventing generation of bradykinin, a vasoactive substance that increases vascular permeability and causes acute angioedema attacks. Patients with HAE are deficient in endogenous or functional C1INH, which leads to an overproduction of bradykinin. They experience recurrent and frequently unpredictable attacks of angioedema lasting 2-5 days, typically involving the extremities, gastrointestinal tract, genitalia, face, oropharynx, and/or larynx.

OTHER PRODUCTS FOR HAE — ... more      

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Title: A Recombinant C1 Esterase Inhibitor (Ruconest) for Hereditary Angioedema (online only)
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