title: Clinical Pattern and Acute and Long-term Management of Hereditary Angioedema Due to C1-Esterase Inhibitor Deficiency.
abstract: BACKGROUND
  Hereditary angioedema due to C1-esterase inhibitor deficiency (HAE-C1-INH) is a life-threatening disease.
  
  
  OBJECTIVE
  To describe the clinical characteristics and management of patients with HAE-C1-INH during routine clinical practice.
  
  
  METHODS
  An observational, retrospective study was performed in patients with HAE-C1-INH. Demographic, clinical, and analytical data were collected from 2 periods: period A (October 2009-September 2010) and period B (October 2007-September 2009).
  
  
  RESULTS
  We studied 112 patients with HAE-C1-INH (57.1% females). Age at onset of symptoms was 14.4 years (lower in patients who had experienced attacks in the previous year). In period B (n=87), 62.1% of patients presented at least 1 edema attack (median, 3.5 attacks/patient/2 years), and 19.1% of attacks were treated. In period A (n=77), 58.4% of patients were on maintenance therapy. Stanozolol was the most widely used drug (48.9%), with a mean weekly dose of 6.7 mg. At least 1 attack was recorded in 72.7% of patients (median, 3.0 attacks/patient/year), and 31.5% of the attacks were treated. Treatment of acute attacks increased by 12.4%.
  
  
  CONCLUSIONS
  Age at onset of symptoms is associated with clinical expression of disease. The higher age at onset of symptoms, the fewer number of attacks per patient and year, and the lower dose of attenuated androgens necessary to control the disease than in other series lead us to hypothesize that HAE-C1-INH could have a less severe expression in Spain. Acute attacks seem to be treated increasingly often.
authors.forename: C
authors.initials: C
authors.lastname: Gómez-Traseira
authors.forename: E
authors.initials: E
authors.lastname: Pérez-Fernández
authors.forename: M C
authors.initials: MC
authors.lastname: López-Serrano
authors.forename: M C
authors.initials: MC
authors.lastname: García-Ara
authors.forename: M
authors.initials: M
authors.lastname: Pedrosa
authors.forename: M
authors.initials: M
authors.lastname: López-Trascasa
authors.forename: T
authors.initials: T
authors.lastname: Caballero
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000728:Androgens / Q000627:therapeutic use*; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D000933:Antifibrinolytic Agents / Q000627:therapeutic use*; D001920:Bradykinin / Q000031:analogs & derivatives* / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use*; D019468:Disease Management; D005260:Female; D056829:Hereditary Angioedema Types I and II / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D000728:Androgens; D000894:Anti-Inflammatory Agents, Non-Steroidal; D000933:Antifibrinolytic Agents; D050718:Complement C1 Inhibitor Protein; C065679:icatibant; D001920:Bradykinin