Hereditary angioedema (HAE) comprises a heterogeneous group of diseases with similar phenotypes but different underlying genotypes. Specific clinical signs may point to HAE as opposed to histaminergic angioedema: the typical prolonged development of angioedema over time, positive family history, a lack of response to antihistamines and steroids and response to bradykinin antagonists are typical signs of HAE. The different types of HAE may be associated with a severe clinical course. They are life-long conditions and are still potentially life-threatening. The quality of life of patients with HAE may be considerably impaired. Management plans should be individualized, which is facilitated by the variety of specific medicastions available.
机构:
Icahn Sch Med Mt Sinai, Div Clin Immunol & Allergy, New York, NY 10029 USAIcahn Sch Med Mt Sinai, Div Clin Immunol & Allergy, New York, NY 10029 USA
Busse, Paula J.
Christiansen, Sandra C.
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机构:
Univ Calif San Diego, Dept Med, San Diego, CA 92103 USAIcahn Sch Med Mt Sinai, Div Clin Immunol & Allergy, New York, NY 10029 USA
Christiansen, Sandra C.
NEW ENGLAND JOURNAL OF MEDICINE,
2020,
382
(12):
: 1136
-
1148