Review Article
Hereditary Angioedema: A Narrative Review
Author Details
1. National Institute of Cancer Research and Hospital, Dhaka
2. Manipal Hospital, Bengaluru, India
3. Matador Diagnostic and Wellness Center
Abstract Hereditary angioedema (HAE) is mostly undiagnosed in most part of the world specially in developing countries like Bangladesh due to lack of awareness and diagnostic facilities. Swelling of face, eyes, lips, hands, feet, and genitals, abdominal pain, and life-threatening laryngeal oedema are the presenting features. HAE should be suspected in all patients who present with angioedema without wheals and who do not respond to antihistamines and/or steroids. C1 levels, C1-INH levels, and C1-INH function are the initial laboratory investigations for HAE. Management of HAE constitutes the treatment of acute attack and short-term and long-term prophylaxis. Self-administered plasma-derived C1-INH concentrate and recombinant human C1-INH (rhC1-INH), ecallantide, and icatibant is the first line treatment. Fresh frozen plasma, tranexamic acid, and attenuated androgens can be used where first line treatment is not available. Attenuated androgens have been shown to be effective in the prevention of attacks of HAE but cautions should be taken in children and in females. Hence, the treatment needs to be individualized considering the risk-benefit ratio of long-term prophylaxis. An overview of hereditary angioedema, its clinical feature, difference from histamine mediated angioedema, laboratory markers and management outline are summarized in this review. Keyword: Hereditary angioedema, Bradykinin, Histamine, Fresh frozen plasma, Tranexamic acid. |
Keywords: Hereditary Angioedema, Bradykinin, Histamine, Fresh Frozen Plasma, Tranexamic Acid