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Original Article


Management of Dental-Oral Procedures in Patients With Hereditary Angioedema due to C1 Inhibitor Deficiency


J Jurado-Palomo,1 JM Muñoz-Caro,2 MC López-Serrano,1 N Prior,1 R Cabañas,1 M Pedrosa,1 M Burgueño,2 T Caballero1,3

1Department of Allergology, Hospital La Paz Health Research Institute (IdiPAZ), Madrid, Spain
2Department of Oral and Maxillofacial Surgery, Hospital La Paz Health Research Institute (IdiPAZ), Madrid, Spain
3Biomedical Research Network on Rare Diseases U754 (CIBERER)

J Investig Allergol Clin Immunol 2013; Vol. 23(1): 1-6



Background: Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) has considerable implications for dental health care providers, since dental procedures may trigger severe and even life-threatening episodes. The aim of the present study was to analyze the efficacy and safety of premedication with attenuated androgens (AAs), plasma-derived human C1 esterase inhibitor concentrate (pdhC1INH), or both to prevent the development of upper airway angioedema after dental-oral procedures in patients with HAE-C1-INH.

Material and Methods: All dental-oral procedures performed on patients with HAE-C1-INH who were followed up at La Paz University Hospital, Madrid, Spain were reviewed. Demographic data, maintenance treatment, preprocedure prophylaxis, disease severity, and occurrence of upper airway angioedema were recorded.

Results: Twenty-four patients (14 male/10 female; mean age, 42.6 years) underwent 66 procedures. Most procedures were performed on patients with severe HAE-C1-INH (20 procedures) or moderate HAE-C1-INH (26 procedures). Only 9 procedures were performed without short-term prophylaxis. Mild upper airway angioedema developed after 3 procedures performed without short-term prophylaxis in patients with minimal or asymptomatic HAE-C1-INH. A statistically significant association was found between development of mild postprocedure upper airway angioedema and lack of maintenance treatment with AA, lack of increased dose of preprocedure AA, and failure to administer preprocedure pdhC1INH (P=.002, Fisher exact test).

Conclusions: Increased doses of prophylactic AA, administration of pdhC1INH, or both were good options for ambulatory management of dental-oral procedures in patients with HAE-C1-INH. Prophylaxis with pdC1INH or increased doses of AA is advisable before dental-oral procedures, even in patients with low disease severity.

Key words: Hereditary angioedema. C1 esterase inhibitor. Acute pharyngeal-laryngeal attack. Dental-oral procedures. Dental surgery. Plasma-derived human C1 inhibitor concentrate. Bradykinin. Attenuated androgen.