title: Successful management of hereditary angioedema during tonsillectomy:  a case report.
abstract: Hereditary angioedema (HAE) is a rare genetic disorder causing a deficiency in C1 esterase inhibitor (C1-INH) that is manifested through unpredictable oedema. We describe a case of a patient with HAE who had previously been refused surgery for tonsillitis due to the potential for oedema, in whom regular monitoring of C1-INH levels combined with intensified therapy with danazol, tranexamic acid and C1-INH concentrate enabled an uncomplicated procedure with no oedema crisis. However, clinicians should be alerted that higher overall dosages of C1-INH concentrate for perioperative prophylaxis may be required than those typically used to treat acute HAE attacks.
authors.affiliation: Hemostasis and Thrombosis Unit, Division of Adult Hematology, Belgium. cedric.hermans@uclouvain.be
authors.forename: Cedric
authors.initials: C
authors.lastname: Hermans
authors.forename: Sébastien
authors.initials: S
authors.lastname: Vander Vorst
authors.forename: Catherine
authors.initials: C
authors.lastname: Lambert
mesh_terms: D000328:Adult; D054179:Angioedemas, Hereditary / Q000150:complications* / Q000188:drug therapy*; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use; D003613:Danazol / Q000627:therapeutic use; D004487:Edema / Q000209:etiology / Q000517:prevention & control; D005260:Female; D006801:Humans; D014068:Tonsillectomy / Q000009:adverse effects / Q000379:methods*; D014069:Tonsillitis / Q000150:complications* / Q000601:surgery*; D014148:Tranexamic Acid / Q000627:therapeutic use
chemical_list: D050718:Complement C1 Inhibitor Protein; D014148:Tranexamic Acid; D003613:Danazol