{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,4,27]],"date-time":"2025-04-27T15:10:03Z","timestamp":1745766603482,"version":"3.40.4"},"reference-count":8,"publisher":"BMJ","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["BMJ Case Reports"],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.1136\/bcr-2013-009616","type":"journal-article","created":{"date-parts":[[2013,5,9]],"date-time":"2013-05-09T03:55:02Z","timestamp":1368071702000},"page":"bcr2013009616","source":"Crossref","is-referenced-by-count":1,"title":["Critical upper airway obstruction in sporadic angioedema responding to C1-esterase inhibitor"],"prefix":"10.1136","volume":"2013","author":[{"given":"Andrew W","family":"O'Keefe","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christine","family":"McCusker","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Moshe","family":"Ben-Shoshan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2013,5,8]]},"reference":[{"key":"2025042707514705000_2013.may08_1.bcr2013009616.1","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1097\/MOP.0b013e328357b25e","article-title":"An update on hereditary angioedema","volume":"2013","author":"Hsu","year":"2012","journal-title":"Curr Opin Pediatr"},{"key":"2025042707514705000_2013.may08_1.bcr2013009616.2","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1186\/1710-1492-6-15","article-title":"Diagnosis and treatment of hereditary angioedema with normal C1 inhibitor","volume":"2013","author":"Bork","year":"2010","journal-title":"Allergy Asthma Clin Immunol"},{"key":"2025042707514705000_2013.may08_1.bcr2013009616.3","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1586\/eci.12.67","article-title":"Clinical efficacy of icatibant in the treatment of acute hereditary angioedema during the FAST-3 trial","volume":"2013","author":"Bas","year":"2012","journal-title":"Expert Rev Clin Immunol"},{"key":"2025042707514705000_2013.may08_1.bcr2013009616.4","doi-asserted-by":"crossref","first-page":"1331","DOI":"10.1111\/j.1398-9995.2010.02368.x","article-title":"Type III hereditary angio-oedema: clinical and biological features in a French cohort","volume":"2013","author":"Vitrat-Hincky","year":"2010","journal-title":"Allergy"},{"key":"2025042707514705000_2013.may08_1.bcr2013009616.5","first-page":"333","article-title":"Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. 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Classification, epidemiology, pathophysiology, genetics, clinical symptoms, and diagnosis","volume":"2013","author":"Caballero","year":"2011","journal-title":"J Invest Allergol Clin Immunol"},{"key":"2025042707514705000_2013.may08_1.bcr2013009616.6","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1007\/s11882-012-0273-4","article-title":"Current management options for hereditary angioedema","volume":"2013","author":"Bork","year":"2012","journal-title":"Curr Allergy Asthma Rep"},{"key":"2025042707514705000_2013.may08_1.bcr2013009616.7","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1177\/0310057X1204000320","article-title":"Successful perioperative management of a patient with C1 esterase inhibitor deficiency with a novel bradykinin receptor B2 antagonist","volume":"2013","author":"Senaratne","year":"2012","journal-title":"Anaesth Intensive Care"},{"key":"2025042707514705000_2013.may08_1.bcr2013009616.8","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1016\/j.anai.2012.04.001","article-title":"Successful C1 esterase inhibitor therapy in a child with a novel type 3 hereditary angioedema-like syndrome","volume":"2013","author":"Rasmussen","year":"2012","journal-title":"Ann Allergy Asthma Immunol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2013-009616","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,27]],"date-time":"2025-04-27T14:51:52Z","timestamp":1745765512000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2013-009616"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,5,8]]},"references-count":8,"alternative-id":["10.1136\/bcr-2013-009616"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2013-009616","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2013,5,8]]}}}