A. Erythematous, edematous rash with diffuse pruritis B. Hives on the abdomen and lower back C. Progressive swelling of the lips, tongue, and face D. Pitting edema of the lower legs E. Unsure

A. Low C4, low C1-INH antigen, low C1-INH function B. Low C4, normal C1-INH antigen, normal C1-INH function C. Low C4, low C1-INH antigen, normal C1-INH function D. Low C4, normal C1-INH antigen, low C1-INH function E. Unsure

A. On-demand therapy is not needed unless there is oropharyngeal swelling B. Every patient should have access to 2 doses of on-demand therapy C. Subcutaneous on-demand therapies are approved for use in children D. An HAE expert should be consulted to give the “okay” to administer on-demand therapy E. Unsure

A. Therapeutic massage B. Routine dental exam C. Cosmetic facial D. Colonoscopy E. Unsure

A. All patients require long-term prophylaxis B. Disease severity and disease burden should be considered when starting long-term prophylaxis C. The goal of long-term prophylaxis is to decrease hospitalizations D. Generic medications are used for long-term prophylaxis E. Unsure

A. C1-INH products are contraindicated in patients with rabbit allergy B. Ecallantide (Kalbitor) can be given by a healthcare provider or self-administered C. Icatibant (Firazyr and generics) is a convenient option in terms of formulation, dosing, and self-administration D. A second dose is recommended in product labeling for all on-demand treatments E. Unsure

A. Both plasma-derived and recombinant C1-INH products are options B. Berotralstat (Orladeyo) could be opened and sprinkled on applesauce for a 7-year-old child who cannot swallow capsules C. Thromboembolism is a concern for all agents D. The least frequent dosing option in adolescents and adults is every 2 to 4 weeks E. Unsure

A. Icatibant (Firazyr and generics) and lanadelumab (Takhzyro) B. Ecallantide (Kalbitor) and plasma-derived C1-INH (Cinryze) C. Recombinant C1-INH (Ruconest) and plasma-derived C1-INH (Haegarda) D. Plasma-derived C1-INH (Berinert) and berotralstat (Orladeyo) E. Unsure

A. Plasma-derived C1-INH concentrate (Cinryze) B. Plasma-derived C1-INH concentrate (Haegarda) C. Lanadelumab (Takhzyro) D. Berotralstat (Orladeyo) E. Unsure

A. Prescribe 4 doses of recombinant C1-INH (Ruconest) (2 for each child) B. Prescribe 4 doses of plasma-derived C1-INH (Berinert) (2 for each child) C. Prescribe 2 doses of plasma-derived C1-INH (Berinert) (for 1 child) and 2 doses of recombinant C1-INH (Ruconest) (for 1 child) D. Prescribe ecallantide (Kalbitor) for both children so that the family has contact with a health care team during HAE attacks E. Unsure