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

• Main Authors: Alexandros Kolokotronis (Author), Paraskevi Tsirevelou (Author), Apostolos Matiakis (Author), Mattheos Papamanthos (Author), Haralambos Skoulakis (Author)
• Format: Book
• Published: Stilus Optimus, 2010-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 2029-283X
• Keywords: hereditary angioedema; laryngeal edema; oral surgical procedure; operative dentistry; tooth extraction; Dentistry; RK1-715
• Source: eJournal of Oral Maxillofacial Research, Vol 1, Iss 1, p e9 (2010)

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.