{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T18:50:18Z","timestamp":1788288618443,"version":"build-2803163510"},"reference-count":44,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2021,12,7]],"date-time":"2021-12-07T00:00:00Z","timestamp":1638835200000},"content-version":"vor","delay-in-days":6,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical &amp; Translational All"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Angioedemas localized in the upper airway are potentially life threatening, and without proper treatment, they may lead to death by suffocation. Upper airway edemas (UAE) in bradykinin\u2010mediated angioedemas can even be the first symptoms of the disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Our survey was performed with a retrospective long\u2010term follow\u2010up method from the medical history of 197 hereditary (C1\u2010INH\u2010HAE) and 20 acquired C1\u2010inhibitor deficiency (C1\u2010INH\u2010AAE), 3 factor XII and 3 plasminogen gene mutation (FXII\u2010HAE, PLG\u2010HAE) patients treated at our center between 1990 and 2020. The UAE group included edemas localized to the mesopharynx, hypopharynx, and larynx, as narrowing of these anatomical regions can lead to suffocation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>98\/197 C1\u2010INH\u2010HAE (47 families) and 13\/20 C1\u2010INH\u2010AAE, 1\/3 PLG\u2010HAE, 1\/3 FXII\u2010HAE patients had experienced UAE at least once according to their medical history. In case of C1\u2010INH\u2010HAE patients, in 6\/47 families who had undiagnosed ancestors had 13 members who died of suffocation. After the diagnosis, 1\u20101 member of two families died of UAE. 44\/64 C1\u2010INH\u2010HAE patients did not smoke, 20\/64 did. The occurrence of UAE was significantly higher in smoker patients. We analyzed 7607 HAE attacks of 56\/98 patients. Out of all attacks, the incidence of UAE in the C1\u2010INH\u2010HAE group was 4%, and 9.5% in the C1\u2010INH\u2010AAE group, respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Early diagnosis is key in bradykinin\u2010mediated angioedemas cases, since the patient must be provided with adequate treatment; and also it is essential to inform patients about the importance of avoiding the trigger factors and the early symptoms of UAE, as these measures could significantly decrease the incidence of lethal UAEs.<\/jats:p><\/jats:sec>","DOI":"10.1002\/clt2.12083","type":"journal-article","created":{"date-parts":[[2021,12,7]],"date-time":"2021-12-07T14:03:43Z","timestamp":1638885823000},"source":"Crossref","is-referenced-by-count":12,"title":["The characteristics of upper airway edema in hereditary and acquired angioedema with C1\u2010inhibitor deficiency"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9041-5036","authenticated-orcid":false,"given":"Zsuzsanna","family":"Balla","sequence":"first","affiliation":[{"name":"Department of Internal Medicine and Haematology Hungarian Angioedema Center of Reference and Excellence Semmelweis University  Budapest Hungary"},{"name":"School of PhD Studies Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6104-5446","authenticated-orcid":false,"given":"No\u00e9mi","family":"Andr\u00e1si","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Haematology Hungarian Angioedema Center of Reference and Excellence Semmelweis University  Budapest Hungary"},{"name":"School of PhD Studies Semmelweis University  Budapest Hungary"},{"name":"2nd Department of Pediatrics Semmelweis University  Budapest 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