Characteristics of Patients Who Underwent a Diagnostic Test for Hereditary Angioedema Admitted Due to Angioedema

Hereditary angioedema (HAE) is clinically characterized by recurrent attacks of angioedema. This study evaluated the clinical findings and examination results of patients admitted due to angioedema who then underwent a diagnostic test for HAE. The study aimed to assess the contribution of laboratory findings to the diagnostic process and to determine clinicians' level of awareness regarding the differential diagnosis of angioedema and the appropriate laboratory tests.

Pediatric patients suspected to have HAE based on the presence of angioedema and screened for C1 esterase inhibitor levels and/or function were included in the study.

A total of 136 patients were evaluated for a preliminary diagnosis of HAE in the presence of angioedema. Angioedema was accompanied by urticaria in 65 patients (47.7%) and itching in 24 patients (17.6%). Patients were evaluated using laboratory tests, C4 levels were studied in 124 patients (91.1%) and were found to be within normal reference limits. C1 esterase inhibitor levels were studied in all patients and were found to be within normal limits. C1 esterase inhibitor function was also studied in 101 patients (74.2%) and was found to be within normal limits.

It was concluded that clinicians keep HAE in mind when encountering angioedema, but that increasing their knowledge of clinical findings that assist in differential diagnosis among angioedema types would be useful. The study authors would like to emphasize that this topic should be included in the specialty training curriculum to raise the awareness of clinicians, especially pediatricians, about clinical HAE findings and the algorithmic approach to the differential diagnosis of angioedema.