{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,11]],"date-time":"2026-06-11T23:23:23Z","timestamp":1781220203700,"version":"3.54.1"},"reference-count":5,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,7,29]],"date-time":"2021-07-29T00:00:00Z","timestamp":1627516800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,7,29]],"date-time":"2021-07-29T00:00:00Z","timestamp":1627516800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Int J Emerg Med"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A 14-year-old African American female presented to the emergency department with spontaneous, sudden-onset lip swelling for 1 h. On examination, there was significant water-bag edema of the upper lip extending to the philtrum and premaxilla. Nasopharyngeal laryngoscopy revealed a patent airway without edema. She was initiated on intravenous dexamethasone, famotidine, and diphenhydramine, after which her edema improved but did not resolve. She was subsequently transferred to a local pediatric hospital and upon further testing she was found to have a C1 esterase inhibitor de novo gene mutation. Angioedema causes localized, non-pitting edema of the dermis, subcutaneous and submucosal tissue, and often manifests in the lips, face, mouth, and throat. Signs of laryngeal involvement include change in voice, stridor, dysphagia, and dyspnea. When laryngeal edema is present, it may necessitate definitive airway management and patients should be monitored in the intensive care unit.<\/jats:p>","DOI":"10.1186\/s12245-021-00364-7","type":"journal-article","created":{"date-parts":[[2021,7,29]],"date-time":"2021-07-29T12:04:02Z","timestamp":1627560242000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Hereditary angioedema"],"prefix":"10.1186","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8916-1948","authenticated-orcid":false,"given":"Helen","family":"Lesser","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason E.","family":"Cohn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,7,29]]},"reference":[{"issue":"6","key":"364_CR1","doi-asserted-by":"publisher","first-page":"630","DOI":"10.1016\/j.anai.2014.09.003","volume":"113","author":"SJ Kim","year":"2014","unstructured":"Kim SJ, Brooks JC, Sheikh J, Kaplan MS, Goldberg BJ. Angioedema deaths in the United States, 1979\u20132010. Ann Allergy Asthma Immunol. 2014;113(6):630\u20134. https:\/\/doi.org\/10.1016\/j.anai.2014.09.003.","journal-title":"Ann Allergy Asthma Immunol"},{"issue":"3","key":"364_CR2","doi-asserted-by":"publisher","first-page":"692","DOI":"10.1016\/j.jaci.2012.05.055","volume":"130","author":"K Bork","year":"2012","unstructured":"Bork K, Hardt J, Witzke G. Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. J Allergy Clin Immunol. 2012;130(3):692\u20137. https:\/\/doi.org\/10.1016\/j.jaci.2012.05.055.","journal-title":"J Allergy Clin Immunol"},{"issue":"12","key":"364_CR3","doi-asserted-by":"publisher","first-page":"2016","DOI":"10.1097\/00005537-200012000-00007","volume":"110","author":"BL Bentsianov","year":"2000","unstructured":"Bentsianov BL, Parhiscar A, Azer M, Har-el G. The role of fiberoptic nasopharyngoscopy in the management of the acute airway in angioneurotic edema. Laryngoscope. 2000;110(12):2016\u20139. https:\/\/doi.org\/10.1097\/00005537-200012000-00007.","journal-title":"Laryngoscope."},{"key":"364_CR4","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1186\/1710-1492-6-24","volume":"6","author":"T Bowen","year":"2010","unstructured":"Bowen T, Cicardi M, Farkas H, et al. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy, Asthma Clin Immunol. 2010;6:24.","journal-title":"Allergy, Asthma Clin Immunol"},{"issue":"11","key":"364_CR5","first-page":"1","volume":"14","author":"RG Wilkerson","year":"2012","unstructured":"Wilkerson RG, Manini A, Scott J. Angioedema in the emergency department: an evidence-based review. Emerg Med Pract. 2012;14(11):1\u201321.","journal-title":"Emerg Med Pract"}],"container-title":["International Journal of Emergency Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12245-021-00364-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12245-021-00364-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12245-021-00364-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,7,29]],"date-time":"2021-07-29T12:04:56Z","timestamp":1627560296000},"score":1,"resource":{"primary":{"URL":"https:\/\/intjem.biomedcentral.com\/articles\/10.1186\/s12245-021-00364-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,7,29]]},"references-count":5,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["364"],"URL":"https:\/\/doi.org\/10.1186\/s12245-021-00364-7","relation":{},"ISSN":["1865-1372","1865-1380"],"issn-type":[{"value":"1865-1372","type":"print"},{"value":"1865-1380","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,7,29]]},"assertion":[{"value":"18 September 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 June 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 July 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"43"}}