RT Book, Section A1 Wesemann, Duane R. A1 Breslow, Rebecca A2 Murray, Michael F. A2 Babyatsky, Mark W. A2 Giovanni, Monica A. A2 Alkuraya, Fowzan S. A2 Stewart, Douglas R. SR Print(0) ID 1102702521 T1 Hereditary Angioedema T2 Clinical Genomics: Practical Applications in Adult Patient Care YR 2014 FD 2014 PB McGraw-Hill Education PP New York, NY SN 9780071622448 LK accessmedicine.mhmedical.com/content.aspx?aid=1102702521 RD 2024/03/29 AB Disease summaryHereditary angioedema (HAE) is a disorder characterized by episodic local subcutaneous and submucosal edema that typically involves the gastrointestinal and upper respiratory tracts.There are three clinically indistinguishable types of the disorder. In HAE type I, serum levels of C1 esterase inhibitor (C1NH) are less than 35% of normal. In HAE type II, the levels are normal or elevated, but the protein is nonfunctional. HAE type III occurs exclusively in women. Both concentration and function of C1 inhibitor are normal and are precipitated or worsened by high estrogen levels.The hereditary form accounts for approximately 2% of clinical angioedema cases and occurs in approximately 1 in 50,000 persons. It affects whites, African Americans, and all other ethnic groups.Hereditary basis:Inheritance: autosomal dominant