title: Critical upper airway obstruction in sporadic angioedema responding to C1-esterase inhibitor.
abstract: We describe a case of recurrent oropharyngeal angioedema in a 16-year-old boy with a history of sickle cell disease and thrombocytopenia and with no family history of angioedema. Emergency treatment of angioedema with C1-esterase inhibitor (C1-INH) provided immediate relief, avoiding the placement of a surgical airway. Further evaluation has shown C1-INH to be normal in quantity and function, with normal complement studies during acute attacks. Genetic testing revealed no abnormality in the factor XII gene. Our case exemplifies that even in cases of sporadic angioedema, treatment with C1-INH may be an effective and life-saving management strategy.
authors: Division of Paediatric Allergy and Clinical Immunology, Department of Paediatrics, Montreal Children's Hospital, McGill University Health Center, Montreal, Canada.
authors: Andrew W
authors: AW
authors: O'Keefe
authors: Christine
authors: C
authors: McCusker
authors: Moshe
authors: M
authors: Ben-Shoshan
mesh_terms: D000293:Adolescent; D000402:Airway Obstruction / Q000188:drug therapy* / Q000209:etiology; D000755:Anemia, Sickle Cell / Q000150:complications*; D000799:Angioedema / Q000150:complications / Q000188:drug therapy*; D003174:Complement C1 Inactivator Proteins / Q000378:metabolism; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use*; D004638:Emergency Treatment; D005174:Factor XII / Q000235:genetics; D006801:Humans; D008297:Male; D009960:Oropharynx / Q000473:pathology*; D013921:Thrombocytopenia / Q000150:complications*
chemical_list: D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D005174:Factor XII
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