Publication

Recombinant human C1-inhibitor in the treatment of acute angioedema attacks

Choi, G., Soeters, M. R., Farkas, H., Varga, L., Obtulowicz, K., Bilo, B., Porebski, G., Hack, C. E., Verdonk, R., Nuijens, J. & Levi, M., Jun-2007, In : Transfusion. 47, 6, p. 1028-1032 5 p.

Research output: Contribution to journalArticleAcademicpeer-review

  • Goda Choi
  • Maarten R. Soeters
  • Henriette Farkas
  • Lilian Varga
  • Krystyna Obtulowicz
  • Barbara Bilo
  • Greg Porebski
  • C. Erik Hack
  • Rene Verdonk
  • Jan Nuijens
  • Marcel Levi

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.

Study Design and 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.

Conclusion: The transgenically produced rHuC1INH was successfully used in the treatment of acute angioedema attacks in patients with hereditary C1-inhibitor deficiency.

Original languageEnglish
Pages (from-to)1028-1032
Number of pages5
JournalTransfusion
Volume47
Issue number6
Publication statusPublished - Jun-2007
Externally publishedYes

    Keywords

  • C1 INHIBITOR CONCENTRATE, HEREDITARY ANGIOEDEMA, DEFICIENCY, THERAPY

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