**Short‐Term Prophylaxis in Odontostomatological, Maxillofacial and ENT Procedures in Patients with Hereditary Angioedema Due to C1‐Inhibitor Deficiency**

Jesús Jurado‐Palomo and Teresa Caballero

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/67713

#### Abstract

C1 inhibitor deficiency: consensus report of an International Working Group. Allergy.

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Oestrogens, trauma, infections or stress has been described as triggers for angioedema (AE) attacks in patients with hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE). Microtrauma can precipitate the onset of acute AE attacks, and thus, dental-oral procedures carry a high risk of triggering them and also an increased risk of death from asphyxiation due to the AE location. In the past, without proper specific treatment, the overall mortality after dental surgery in patients with C1-INH-HAE was up to 30–40%. Some dental-oral, medical and/or surgical procedures are susceptible to receive "short-term prophylaxis" (STP) in order to reduce the risk of AE. We describe the published case reports of dental-oral, maxillofacial and ear, nose and throat (ENT) procedures in patients with C1-INH-HAE. Different consensus algorithms and clinical guidelines have been published for managing dental-oral, maxillofacial and otolaryngological procedures (DOMFOPs) and will be reviewed below. Based on the clinical experience of the Department of Allergology of the University Hospital La Paz (Madrid) and the University General Hospital Nuestra Señora del Prado (Talavera de la Reina), these algorithms have been updated and modified. We advise to classify procedures according to the risk of producing AE as minor, intermediate and major risks.

Keywords: algorithm, angioedema, antifibrinolytic agents, attenuated androgens, bradykinin, C1 inhibitor, dental-oral procedures, dental surgery, ecallantide, hereditary angioedema, icatibant acetate, recombinant human C1 inhibitor, plasma-derived human C1-inhibitor concentrate, short-term prophylaxis, solvent/detergent-treated plasma, treatment

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