title: Prospective study of rapid relief provided by C1 esterase inhibitor in emergency treatment of acute laryngeal attacks in hereditary angioedema.
abstract: BACKGROUND
  Hereditary angioedema (HAE) is a rare disorder characterized by C1 esterase inhibitor (C1-INH) deficiency, resulting in periodic attacks of acute edema that can be life-threatening if they occur in the laryngeal region. We assessed the efficacy of C1-INH concentrate in the emergency treatment of rarely occurring acute laryngeal HAE attacks in a prospective, open-label clinical study.
  
  
  METHODS
  Acute laryngeal attacks were each treated with C1-INH concentrate (Berinert) at a single dose of 20 U/kg body weight. Efficacy endpoints included time to onset of symptom relief and time to complete resolution of all symptoms, each based on the patient's assessment.
  
  
  RESULTS
  All 39 laryngeal attacks in 16 patients were treated successfully. The median time to onset of symptom relief was 15 min. The median time to complete resolution of all symptoms was 8.25 h. No treatment-related serious adverse events occurred, and the treatment was well tolerated. The administration of C1-INH concentrate was not associated with any viral infections.
  
  
  CONCLUSIONS
  C1-INH concentrate is an effective and safe emergency treatment for providing reliable and rapid relief from the potentially life-threatening symptoms of laryngeal HAE attacks.
authors.affiliation: Asthma and Respiratory Research, Penn State University, 500 University Drive, Hershey, PA 17033, USA. tcraig@hmc.psu.edu
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Craig
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: Lynda
authors.initials: L
authors.lastname: Schneider
authors.forename: Flint
authors.initials: F
authors.lastname: Packer
authors.forename: William H
authors.initials: WH
authors.lastname: Yang
authors.forename: Heinz-Otto
authors.initials: HO
authors.lastname: Keinecke
authors.forename: Peter C
authors.initials: PC
authors.lastname: Kiessling
mesh_terms: D000293:Adolescent; D000328:Adult; D054179:Angioedemas, Hereditary / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology / Q000503:physiopathology; D050718:Complement C1 Inhibitor Protein / Q000008:administration & dosage* / Q000009:adverse effects / Q000235:genetics; D018572:Disease-Free Survival; D004487:Edema; D004632:Emergency Medical Services; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D007818:Laryngeal Diseases / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011795:Surveys and Questionnaires
chemical_list: D050718:Complement C1 Inhibitor Protein