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Abstract
Until recently it was assumed that hereditary angioedema is a disease that results exclusively from a genetic deficiency of the C1 inhibitor. In 2000, families with hereditary angioedema, normal C1 inhibitor activity and protein in plasma were described. Since then numerous patients and families with that condition have been reported. Most of the patients by far were women. In many of the affected women, oral contraceptives, hormone replacement therapy containing estrogens, and pregnancies triggered the clinical symptoms. Recently, in some families mutations in the coagulation factor XII (Hageman factor) gene were detected in the affected persons.
Highlights
Angioedema is clinically characterized by self-limiting episodes of marked edema involving the skin, gastrointestinal (GI) tract and other organs
Almost identical numbers were observed with respect to women diagnosed with hereditary angioedema (HAE)-C1 esterase inhibitor (C1-INH). These results show that estrogens play a role in both conditions and that the negative influence of estrogens is not a specific sign for HAE type III [14]
Consider the following facts: (a) there are many similarities concerning clinical symptoms of hereditary angioedema types I and III; (b) the percentages of women whose disease is negatively affected by estrogen-containing medications is similar in both conditions; (c) angiotensin-converting enzyme inhibitors and angiotensin II type 1 receptor antagonists may lead to an increase in frequency and severity of attacks in HAE type III similar to HAE due to C1 inhibitor deficiency (HAE type I and II); and (d) the response to antihistamines and corticosteroids is lacking, at least in the patients reported up to now
Summary
Angioedema is clinically characterized by self-limiting episodes of marked edema involving the skin, gastrointestinal (GI) tract and other organs. Consider the following facts: (a) there are many similarities concerning clinical symptoms of hereditary angioedema types I and III; (b) the percentages of women whose disease is negatively affected by estrogen-containing medications is similar in both conditions; (c) angiotensin-converting enzyme inhibitors and angiotensin II type 1 receptor antagonists may lead to an increase in frequency and severity of attacks in HAE type III (according to the observations mentioned above) similar to HAE due to C1 inhibitor deficiency (HAE type I and II); and (d) the response to antihistamines and corticosteroids is lacking, at least in the patients reported up to now These facts permit the speculation that edema formation in HAE type III may be related to the kinin pathway. One woman with HAE-FXII who started tranexamic acid therapy (4 g/d) has had no attacks with this treatment regimen [14]
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