title: Management of dental-oral procedures in patients with hereditary angioedema due to C1 inhibitor deficiency.
abstract: 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.
  
  
  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.
authors: Department of Allergology, Hospital La Paz Health Research Institute (IdiPAZ), Madrid, Spain. h72jupaj@yahoo.es
authors: J
authors: J
authors: Jurado-Palomo
authors: J M
authors: JM
authors: Muñoz-Caro
authors: M C
authors: MC
authors: López-Serrano
authors: N
authors: N
authors: Prior
authors: R
authors: R
authors: Cabañas
authors: M
authors: M
authors: Pedrosa
authors: M
authors: M
authors: Burgueño
authors: T
authors: T
authors: Caballero
mesh_terms: D000328:Adult; D000728:Androgens / Q000009:adverse effects / Q000627:therapeutic use*; D050718:Complement C1 Inhibitor Protein / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D056829:Hereditary Angioedema Types I and II / Q000188:drug therapy* / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications; D011292:Premedication; D009057:Stomatognathic Diseases / Q000150:complications* / Q000601:surgery; D055815:Young Adult
chemical_list: D000728:Androgens; D050718:Complement C1 Inhibitor Protein