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Hereditary angioedema: three cases report, members of the same family

Mattheos Papamanthos1, Apostolos Matiakis2, Paraskevi Tsirevelou3

  • 1Department of Dentistry, General Hospital of Volos, Volos Greece.

Journal of Oral & Maxillofacial Research
|January 15, 2014
PubMed

Insights

Hereditary angioedema (HAE) prophylaxis before dental procedures can prevent life-threatening laryngeal edema. This case report details HAE in a family, including the youngest reported patient, emphasizing preventative strategies.

Area of Science:

  • Immunology
  • Genetics
  • Medical Case Reports

Background:

  • Presents a clinical case report of three Hereditary Angioedema (HAE) patients from the same family.
  • Details varying levels of C1-INH deficiency within the family, including the father and two daughters.
  • Highlights the youngest reported HAE case in English literature, diagnosed at age 2.

Purpose of the Study:

  • To assess the efficacy of C1-INH administration prior to dental operations for preventing HAE episodes.
  • To evaluate prophylactic strategies for patients with total or partial C1-INH deficiency undergoing dental procedures.

Main Methods:

  • Clinical case review of a family with Hereditary Angioedema.
  • Assessment of C1-INH levels and function in affected individuals.
  • Evaluation of prophylactic C1-INH administration before dental procedures.

Main Results:

  • Acute angioedema, including laryngeal edema, is a critical risk for HAE patients during dental procedures.
  • Prophylaxis with C1-INH, both short-term and long-term, may be life-saving.
  • Early recognition and prevention of laryngeal edema are crucial.

Conclusions:

  • Prophylactic C1-INH administration before dental operations is vital for HAE patients.
  • Preventing laryngeal edema through early recognition and intervention can be life-saving.
  • Dental procedures require careful management in HAE patients due to potential complications.
Abstract

Keywords:
hereditary angioedemalaryngeal edemaoperative dentistryoral surgical proceduresurgical diagnostic techniquetooth extraction.

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