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. 2022 Aug 15;56(9):740–747. doi: 10.1097/MCG.0000000000001744

TABLE 3.

Factors for Consideration in Recognizing Patients With HAE

Parameter Key Factors
Patient history Recurrent unexplained gastrointestinal painUse of estrogen-containing contraceptivesPatient has recently reached puberty, is pregnant, or has recently entered menopausePrevious abdominal diagnoses and/or surgical interventions
Family history Family history of unexplained swelling or HAE
Signs and symptoms at presentation No fever, peritoneal signs, or elevated white blood cell countSevere abdominal pain without a history of laryngeal or cutaneous swelling
Clinical assessments Test for low serum level of C4 and low C1-INH level or function*Use CT or ultrasonography of the abdomen or pelvis
Action Refer patients to a physician experienced in managing HAE for confirmation of diagnosis
*

Both C1-INH antigenic level and functional level should be tested to distinguish between HAE type I (low antigen and low function) and HAE type II (normal antigen and low function).

C1-INH indicates C1 inhibitor; CT, contrast-enhanced computed tomography; HAE, hereditary angioedema.