What is hereditary angioedema?

➢ Hereditary Angioedema (HAE) is a very rare and potentially life-threatening genetic condition, autosomal dominant disorder (AD) of C1 inhibitor (C1-INH), that occurs in about 1 in 10,000 to 1 in 50,000 people ➢ Although named after its complement inhibitory activity, C1-inhibitor is the most important physiological inhibitor of plasma kallikrein ➢ People who have HAE have low levels of C1 esterase inhibitor in their body. ➢ HAE results from excessive production of bradykinin due to deficiency of C1 esterase inhibitor Sx (edema of body, excruciating abdominal pain, nausea, vomiting, intestinal wall swelling, airthroat swelling dangerous)