title: Recombinant human C1-inhibitor in the treatment of acute angioedema attacks.
abstract: BACKGROUND
  Patients with hereditary C1-inhibitor deficiency have recurrent attacks of angioedema, preferably treated with C1-inhibitor concentrate. A recombinant human C1-inhibitor (rHuC1INH) was developed, derived from milk from transgenic rabbits. This study was undertaken to investigate the effects of rHuC1INH in the treatment of acute angioedema attacks in patients with a hereditary C1-inhibitor deficiency.
  
  
  METHODS
  Patients with hereditary C1-inhibitor deficiency were treated with rHuC1INH (at a dose of 100 U/kg) within 8 hours after onset of an acute attack. Time to initiation of relief and time to minimal symptoms were reported by both the patient and the treating physician.
  
  
  RESULTS
  Thirteen severe angioedema attacks in 9 patients with hereditary C1-inhibitor deficiency were treated with rHuC1INH. There was rapid improvement of clinical conditions for all attacks, with approximately 80 percent of treated patients experiencing symptom relief within 2 hours and minimal symptoms within 12 hours. There were no drug-related adverse events or immunogenic reactions to C1-inhibitor or rabbit proteins.
  
  
  CONCLUSIONS
  The transgenically produced rHuC1INH was successfully used in the treatment of acute angioedema attacks in patients with hereditary C1-inhibitor deficiency.
authors.affiliation: Department of Internal Medicine, Academic Medical Center, Free University Medical Center, Amsterdam, The Netherlands.
authors.forename: Goda
authors.initials: G
authors.lastname: Choi
authors.forename: Maarten R
authors.initials: MR
authors.lastname: Soeters
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
authors.forename: Lilian
authors.initials: L
authors.lastname: Varga
authors.forename: Krystyna
authors.initials: K
authors.lastname: Obtulowicz
authors.forename: Barbara
authors.initials: B
authors.lastname: Bilo
authors.forename: Greg
authors.initials: G
authors.lastname: Porebski
authors.forename: C Erik
authors.initials: CE
authors.lastname: Hack
authors.forename: René
authors.initials: R
authors.lastname: Verdonk
authors.forename: Jan
authors.initials: J
authors.lastname: Nuijens
authors.forename: Marcel
authors.initials: M
authors.lastname: Levi
mesh_terms: D000208:Acute Disease; D000328:Adult; D000799:Angioedema / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000235:genetics; D000818:Animals; D050718:Complement C1 Inhibitor Protein / Q000008:administration & dosage* / Q000009:adverse effects / Q000235:genetics; D005260:Female; D030342:Genetic Diseases, Inborn / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D011817:Rabbits; D011859:Radiography; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000009:adverse effects / Q000235:genetics; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D050718:Complement C1 Inhibitor Protein; D011994:Recombinant Proteins