Hereditary angioedema
Abstract 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 air...
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Online Access: | https://doi.org/10.1186/s12245-021-00364-7 |
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doaj-05e13e97a80343e98cefc098b4031c9d2021-08-01T11:14:41ZengBMCInternational Journal of Emergency Medicine1865-13721865-13802021-07-011411210.1186/s12245-021-00364-7Hereditary angioedemaHelen Lesser0Jason E. Cohn1Department of Otolaryngology-Head and Neck Surgery, Philadelphia College of Osteopathic MedicineDepartment of Otolaryngology-Head and Neck Surgery, Division of Facial Plastic Reconstructive Surgery, Louisiana State University Health Sciences CenterAbstract 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.https://doi.org/10.1186/s12245-021-00364-7AngioedemaHereditary angioedemaLip swellingPediatric otolaryngology |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Helen Lesser Jason E. Cohn |
spellingShingle |
Helen Lesser Jason E. Cohn Hereditary angioedema International Journal of Emergency Medicine Angioedema Hereditary angioedema Lip swelling Pediatric otolaryngology |
author_facet |
Helen Lesser Jason E. Cohn |
author_sort |
Helen Lesser |
title |
Hereditary angioedema |
title_short |
Hereditary angioedema |
title_full |
Hereditary angioedema |
title_fullStr |
Hereditary angioedema |
title_full_unstemmed |
Hereditary angioedema |
title_sort |
hereditary angioedema |
publisher |
BMC |
series |
International Journal of Emergency Medicine |
issn |
1865-1372 1865-1380 |
publishDate |
2021-07-01 |
description |
Abstract 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. |
topic |
Angioedema Hereditary angioedema Lip swelling Pediatric otolaryngology |
url |
https://doi.org/10.1186/s12245-021-00364-7 |
work_keys_str_mv |
AT helenlesser hereditaryangioedema AT jasonecohn hereditaryangioedema |
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