Hereditary angioneurotic edema
Definition
Hereditary angioneurotic edema (HANE) is a nonsex-linked (autosomal) dominant disease that results from mutations in a gene responsible for producing one of the proteins responsible for human immunity. This disease is also known as hereditary angioedema (HAE) or hereditary C1 inhibitor deficiency because it is a deficiency of the protein (C1-INH) that inhibits the action of the enzyme known as C1 which causes this disease.
Description
There are two recognized forms of HANE. Type I represents approximately 80-85% of the cases of hereditary angioneurotic edema. In this type, the protein C1-INH is not produced in sufficient quantities. Type II HANE represents the remaining 15-20% of cases. In this type, C1-INH...
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