Clinical Studies of Sudden Upper Airway Obstruction in Patients With Hereditary Angioedema Due to C1 Esterase Inhibitor Deficiency
Open Access
- 26 May 2003
- journal article
- research article
- Published by American Medical Association (AMA) in Archives of internal medicine (1960)
- Vol. 163 (10) , 1229-1235
- https://doi.org/10.1001/archinte.163.10.1229
Abstract
HEREDITARY angioedema (HAE) was first described clinically by Quincke1 and Osler.2 Currently, 3 types of HAE have been described. Classic HAE is a well-defined autosomal dominant disease (Mendelian Inheritance in Man online database No. 106100) caused by an inherited deficiency of functional C1 esterase inhibitor (C1-INH). Donaldson and Evans3 discovered the underlying defect in 1963: the defective C1-INH gene produces either no C1-INH (type I HAE) or a dysfunctional C1-INH (type II HAE).This publication has 11 references indexed in Scilit:
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