C1 Esterase Inhibitor: Managing Hereditary Angioedema
C1 Esterase Inhibitor (C1-INH) is a plasma protein that regulates the complement and kinin systems. A deficiency in this protein leads to a condition called Hereditary Angioedema (HAE), characterized by sudden, severe swelling of the skin, gastrointestinal tract, and, most dangerously, the larynx (throat).
Plasma-derived C1-INH is used in two ways:
Acute Treatment: Administered during an attack to stop the swelling quickly.
Prophylaxis: Regular infusions to prevent attacks from occurring. Before this derivative was available, HAE had a high mortality rate due to asphyxiation from laryngeal edema. Today, it is a manageable chronic condition.
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