Current management includes on-demand therapy, short-term prophylaxis before procedures, and long-term prophylaxis for severe or recurrent cases.3 Most cases of HAE-C1-INH are caused by mutations in the SERPING1 gene, which encodes C1-inhibitor (C1-INH), resulting in reduced or dysfunctional C1-INH and uncontrolled vascular permeability.4 Other genetic variants, including mutations in F12, PLG, and ANGPT1, have been linked to HAE-nC1-INH.1 The relationship between genetic variants and treatment response remains an area of ongoing research, particularly in pediatric patients. This evidence concerns the gene COL18A1 and hereditary angioedema.