Abstract Hereditary angioedema (HAE) is a rare autosomal dominant disorder due to deficiency or dysfunction of the complement 1 esterase inhibitor (C1-INH) and leading to bradykinin-mediated angioedema. It commonly affects the skin, gastrointestinal tract, and upper airway and may be life-threatening. Conventional therapies such as corticosteroids, antihistamines, and epinephrine are usually ineffective. 1,2 We report a case of HAE type I with recurrent exacerbations and progressive laryngeal edema causing respiratory distress, unresponsive to standard therapy. The patient was electively intubated for airway protection. Due to nonavailability of C1-INH concentrate and kallikrein inhibitors in India, fresh frozen plasma (FFP) was administered. The patient showed marked clinical improvement and was successfully extubated within 48 h. Steroids were tapered, and the patient was discharged in stable condition. This case highlights the importance of early diagnosis and the role of FFP as an effective alternative therapy in resource-limited settings.
Verma et al. (Thu,) studied this question.