Abstract
Hereditary angioedema (HAE) is clinically characterized by recurrent and self-limiting
skin, intestinal, and life-threatening laryngeal edema. This study describes the age
at which laryngeal edema first occurred, the time between onset and full development,
and the effectiveness of therapy and prophylaxis in 123 HAE patients. 61 (49.7%) patients
experienced a total of 596 laryngeal edema episodes. The ratio of laryngeal edema
episodes to skin swellings and abdominal pain attacks was approximately 1:70:54 in
patients who had laryngeal edema. The mean (SD) age at the first laryngeal edema was
26.2 (15.3) years. Nearly 80% of the laryngeal edemas occurred between age 11 and
45. The mean interval between onset and maximum development of laryngeal edema was
8.3 hours. A total of 354 laryngeal edemas cleared spontaneously without treatment
and 208 laryngeal edemas were successfully treated with C1 inhibitor concentrate.
Despite long-term prophylactic treatment with danazol, 6 patients developed subsequent
laryngeal edemas. Laryngeal edema may occur at any age, although young adults are
at greatest risk. In adults, the interval between onset of symptoms and acute risk
of asphyxiation is usually long enough to allow for use of appropriate emergency procedures.
It is essential to instruct patients and their relatives about the first signs of
laryngeal edemas and the necessary procedures to follow.
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© 2003 Published by Elsevier Inc.
