title: Prospective study of C1 esterase inhibitor in the treatment of successive acute abdominal and facial hereditary angioedema attacks.
abstract: BACKGROUND
  hereditary angioedema (HAE) is a rare disorder characterized by a quantitative or functional deficiency of C1 esterase inhibitor (C1-INH), resulting in periodic attacks of acute edema at various body locations. The symptoms of these painful attacks can be treated effectively with C1-INH concentrate.
  
  
  OBJECTIVE
  to document the efficacy and safety of a weight-based dose of C1-INH concentrate in the treatment of successive HAE attacks at abdominal and facial locations.
  
  
  METHODS
  acute facial and abdominal attacks were each treated with C1-INH concentrate using a single intravenous dose of 20 U/kg body weight. Efficacy end points included patient-reported time to onset of symptom relief and time to complete resolution of all symptoms. Safety was assessed by monitoring adverse events and assaying for markers of viral infection.
  
  
  RESULTS
  we treated 663 abdominal attacks in 50 patients and 43 facial attacks in 16 patients (a total of 706 attacks in 53 patients). The median time to onset of relief for all attacks was 19.8 minutes, with a median time to complete resolution of 11.0 hours. The median time to onset of relief was 19.8 minutes for abdominal attacks and 28.2 minutes for facial attacks, indicating efficacy for both types of attack. No treatment-related serious adverse events occurred, and C1-INH concentrate was well tolerated. No human immunodeficiency virus, hepatitis virus, or parvovirus B19 infections arose during the study.
  
  
  CONCLUSIONS
  the C1-INH concentrate dose of 20 U/kg provides rapid, effective, and safe treatment for successive HAE attacks at abdominal and facial locations.
authors.affiliation: DallasAllergyImmunology, Dallas, Texas 75230, USA. richwasserman_pdai@yahoo.com
authors.forename: Richard L
authors.initials: RL
authors.lastname: Wasserman
authors.forename: Robyn J
authors.initials: RJ
authors.lastname: Levy
authors.forename: Againdra K
authors.initials: AK
authors.lastname: Bewtra
authors.forename: David
authors.initials: D
authors.lastname: Hurewitz
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Craig
authors.forename: Peter C
authors.initials: PC
authors.lastname: Kiessling
authors.forename: Heinz-Otto
authors.initials: HO
authors.lastname: Keinecke
authors.forename: Jonathan A
authors.initials: JA
authors.lastname: Bernstein
mesh_terms: D000005:Abdomen; D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D054179:Angioedemas, Hereditary / Q000188:drug therapy*; D002648:Child; D050718:Complement C1 Inhibitor Protein / Q000009:adverse effects / Q000627:therapeutic use*; D005145:Face; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D016896:Treatment Outcome
chemical_list: D050718:Complement C1 Inhibitor Protein