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C1 Esterase Inhibitor (Human) for the Treatment of Acute Hereditary Angioedema

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Publication Date: 25 Sep 2011

Journal: Clinical Medicine Insights: Blood Disorders

Citation: Clinical Medicine Insights: Blood Disorders 2011:4 21-30

doi: 10.4137/CMBD.S4090

CMIbd journal

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Abstract

Hereditary angioedema (HAE) is a relatively rare disease characterized by acute episodes of swelling. These swellings can be disfiguring, painful and life-threatening. Since the symptoms occur in different areas and most patients experience a delay in their diagnosis, resulting in unnecessary suffering and dangerous situations. HAE can have a tremendous impact on the quality of life.

The major genetic deficiency in this disorder is either an absent or nonfunctional C1INH which regulates the complement, fibrinolitic, kalikrein and plasmin pathways.


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