Hereditary Angioedema — NDEB AFK MCQ
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Correct answer: E — C1 esterase inhibitor (C1-INH)
Hereditary angioedema is caused by deficiency or dysfunction of C1 esterase inhibitor (C1-INH), an autosomal dominant condition. Uncontrolled activation of the complement and kinin pathways leads to recurrent episodes of subcutaneous and submucosal swelling (angioedema) without urticaria. Dental significance: laryngeal oedema during dental procedures can be life-threatening. Epinephrine is NOT effective — treatment requires C1-INH concentrate, icatibant (bradykinin B2 receptor antagonist), or ecallantide (kallikrein inhibitor). Pre-procedural prophylaxis with C1-INH concentrate is recommended.
Reference: Abbas – Cellular and Molecular Immunology, 10th ed., 2021