Prospective study of C1 esterase inhibitor in the treatment of successive acute abdominal and facial hereditary angioedema attacks

Richard L. Wasserman, Robyn J. Levy, Againdra K. Bewtra, David Hurewitz, Timothy J. Craig, Peter C. Kiessling, Heinz Otto Keinecke, Jonathan A. Bernstein

Research output: Contribution to journalArticle

18 Citations (Scopus)

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. Conclusion The C1-INH concentrate dose of 20 U/kg provides rapid, effective, and safe treatment for successive HAE attacks at abdominal and facial locations.

Original languageEnglish
Pages (from-to)62-68
Number of pages7
JournalAnnals of Allergy, Asthma and Immunology
Volume106
Issue number1
DOIs
StatePublished - Jan 2011

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Complement C1 Inhibitor Protein
Hereditary Angioedemas
Prospective Studies
Hereditary Angioedema Types I and II
Parvoviridae Infections
Therapeutics
Safety
Hepatitis Viruses
Virus Diseases
Edema
Body Weight
HIV
Weights and Measures

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Pulmonary and Respiratory Medicine

Cite this

Prospective study of C1 esterase inhibitor in the treatment of successive acute abdominal and facial hereditary angioedema attacks. / Wasserman, Richard L.; Levy, Robyn J.; Bewtra, Againdra K.; Hurewitz, David; Craig, Timothy J.; Kiessling, Peter C.; Keinecke, Heinz Otto; Bernstein, Jonathan A.

In: Annals of Allergy, Asthma and Immunology, Vol. 106, No. 1, 01.2011, p. 62-68.

Research output: Contribution to journalArticle

Wasserman, Richard L. ; Levy, Robyn J. ; Bewtra, Againdra K. ; Hurewitz, David ; Craig, Timothy J. ; Kiessling, Peter C. ; Keinecke, Heinz Otto ; Bernstein, Jonathan A. / Prospective study of C1 esterase inhibitor in the treatment of successive acute abdominal and facial hereditary angioedema attacks. In: Annals of Allergy, Asthma and Immunology. 2011 ; Vol. 106, No. 1. pp. 62-68.
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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. Conclusion The C1-INH concentrate dose of 20 U/kg provides rapid, effective, and safe treatment for successive HAE attacks at abdominal and facial locations.",
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