| Abstract text: |
HAE is an autosomal-dominant disorder transmitted. The most common form of the disease (type I, 85% of patients) is characterized by a reduced synthesis of C1-INH. C1 esterase inhibitor (C1-INH) is a highly polymorphic glycosylated protein that belongs to serine protease inhibitors called serpine. The disease begins in childhood or adolescence with recurrent episodes of swelling and subcutaneous or submucosal, painless, and uncompressed, non-pruritic. Episodes are self-limited, usually resolves within 2-5 days. Attacks vary in frequency, severity, duration, location, insufficient triggers are known. There is usually a family history of the condition. However, relatives may be unaware of previous cases, which may have just been reported as an unexpected, sudden, and premature death of a parent, aunt, uncle, or grandparent. Dental procedures, sickness (including colds and the flu), and surgery have been shown to trigger certain attacks of hereditary angioedema. Intestinal problems are often associated with vomiting and diarrhea, the larynx can lead to potentially fatal respiratory obstruction. Long-term therapy of hereditary angioedema includes administration of attenuated androgens such as danazol and stanozolului that increase serum concentrations of C1-INH in both types of disease normally. |
