title: [Episodes of angioedema in children with C1 esterase inhibitor deficiency].
abstract: A 6-year-old boy and a 3.5-year-old girl presented with unexplained episodes of angioedema without urticaria. Low serum C1 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. C1 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 C1 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.
authors.affiliation: Universitair Medisch Centrum Utrecht, Wilhelmina Kinderziekenhuis.
authors.forename: M A C
authors.initials: MA
authors.lastname: Hemels
authors.forename: S P M
authors.initials: SP
authors.lastname: Geelen
authors.forename: M O
authors.initials: MO
authors.lastname: Hoekstra
authors.forename: Y
authors.initials: Y
authors.lastname: Meijer
mesh_terms: D000402:Airway Obstruction / Q000209:etiology*; D000799:Angioedema / Q000188:drug therapy / Q000209:etiology* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000235:genetics; D005260:Female; D006801:Humans; D008297:Male
chemical_list: D003174:Complement C1 Inactivator Proteins