Episodes of angioedema in children with C1 esterase inhibitor deficiency

- A 6-year-old boy and a 3.5-year-old girl presented with unexplained episodes ofangioedema without urticaria. Low serum CI esterase inhibitor activity was found in both children. Family history revealed autosomal dominant inheritance in the girl. The boy had a negative family history for angioedema. CI esterase inhibitor deficiency is a rare but serious condition that may cause oedema of the upper respiratory tract and death by asphyxiation. Episodes of angioedema occur spontaneously, usually subsiding within 48-72 h. Between episodes, the patients are symptom free. Treatment consists of substitution of synthetic CI esterase inhibitor during episodes of edema carrying a risk of upper airway obstruction. In patients who have more than one episode of severe angioedema per month, daily treatment with tranexamic acid should be considered. Both of these patients were not receiving daily treatment.