Hereditary Angioedema: Three Cases Report, Members of the Same Family

Background: This current clinical case report highlights three cases of Hereditary angioedema (HAE) patients who are all members of the same family (father and his two daughters). The father has C1–INH deficiency, while his daughters have low C1–INH levels: the first possesses only 10% function and...

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Main Authors: Alexandros Kolokotronis, Paraskevi Tsirevelou, Apostolos Matiakis, Mattheos Papamanthos, Haralambos Skoulakis
Format: Article
Language:English
Published: Lithuanian University of Health Sciences, Faculty of Odontology 2010-01-01
Series:eJournal of Oral Maxillofacial Research
Subjects:
Online Access:http://www.ejomr.org/JOMR/archives/2010/1/e9/e9ht.pdf
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author Alexandros Kolokotronis
Paraskevi Tsirevelou
Apostolos Matiakis
Mattheos Papamanthos
Haralambos Skoulakis
author_facet Alexandros Kolokotronis
Paraskevi Tsirevelou
Apostolos Matiakis
Mattheos Papamanthos
Haralambos Skoulakis
author_sort Alexandros Kolokotronis
collection DOAJ
description Background: This current clinical case report highlights three cases of Hereditary angioedema (HAE) patients who are all members of the same family (father and his two daughters). The father has C1–INH deficiency, while his daughters have low C1–INH levels: the first possesses only 10% function and the second has low C1–INH level with 0% function. Of note, the second daughter was discovered to have HAE at the age of 2, thus making her the youngest known HAE case report in the English literature.Methods: Assess the efficacy of administration of C1-INH before dental operation as regards the prevention of HAE episode, when total or partial C1-INH deficiency exists.Results: Acute angioedema leading to laryngeal oedema is a possibly fatal complication for HAE patients undergoing dental procedures. Use of both short-term and long-term HAE prophylaxis prior to dental operations might be life saving for those patients.Conclusions: Prevention and early recognition of potential laryngeal oedema that can occur as a complication of dental procedures may be lifesaving for HAE patients.
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publisher Lithuanian University of Health Sciences, Faculty of Odontology
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series eJournal of Oral Maxillofacial Research
spelling doaj-art-baa7ac9d563b4562b1d881dff6b47cf02025-08-20T02:51:24ZengLithuanian University of Health Sciences, Faculty of OdontologyeJournal of Oral Maxillofacial Research2029-283X2010-01-0111e9Hereditary Angioedema: Three Cases Report, Members of the Same FamilyAlexandros KolokotronisParaskevi TsirevelouApostolos MatiakisMattheos PapamanthosHaralambos SkoulakisBackground: This current clinical case report highlights three cases of Hereditary angioedema (HAE) patients who are all members of the same family (father and his two daughters). The father has C1–INH deficiency, while his daughters have low C1–INH levels: the first possesses only 10% function and the second has low C1–INH level with 0% function. Of note, the second daughter was discovered to have HAE at the age of 2, thus making her the youngest known HAE case report in the English literature.Methods: Assess the efficacy of administration of C1-INH before dental operation as regards the prevention of HAE episode, when total or partial C1-INH deficiency exists.Results: Acute angioedema leading to laryngeal oedema is a possibly fatal complication for HAE patients undergoing dental procedures. Use of both short-term and long-term HAE prophylaxis prior to dental operations might be life saving for those patients.Conclusions: Prevention and early recognition of potential laryngeal oedema that can occur as a complication of dental procedures may be lifesaving for HAE patients.http://www.ejomr.org/JOMR/archives/2010/1/e9/e9ht.pdfhereditary angioedemalaryngeal edemaoral surgical procedureoperative dentistrytooth extraction
spellingShingle Alexandros Kolokotronis
Paraskevi Tsirevelou
Apostolos Matiakis
Mattheos Papamanthos
Haralambos Skoulakis
Hereditary Angioedema: Three Cases Report, Members of the Same Family
eJournal of Oral Maxillofacial Research
hereditary angioedema
laryngeal edema
oral surgical procedure
operative dentistry
tooth extraction
title Hereditary Angioedema: Three Cases Report, Members of the Same Family
title_full Hereditary Angioedema: Three Cases Report, Members of the Same Family
title_fullStr Hereditary Angioedema: Three Cases Report, Members of the Same Family
title_full_unstemmed Hereditary Angioedema: Three Cases Report, Members of the Same Family
title_short Hereditary Angioedema: Three Cases Report, Members of the Same Family
title_sort hereditary angioedema three cases report members of the same family
topic hereditary angioedema
laryngeal edema
oral surgical procedure
operative dentistry
tooth extraction
url http://www.ejomr.org/JOMR/archives/2010/1/e9/e9ht.pdf
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AT mattheospapamanthos hereditaryangioedemathreecasesreportmembersofthesamefamily
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