Prospective study of rapid relief provided by C1 esterase inhibitor in emergency treatment of acute laryngeal attacks in hereditary angioedema

Timothy J. Craig, Richard L. Wasserman, Robyn J. Levy, Againdra K. Bewtra, Lynda Schneider, Flint Packer, William H. Yang, Heinz Otto Keinecke, Peter C. Kiessling

Research output: Contribution to journalArticle

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Abstract

Introduction: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. Conclusion 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.

Original languageEnglish
Pages (from-to)823-829
Number of pages7
JournalJournal of Clinical Immunology
Volume30
Issue number6
DOIs
StatePublished - Nov 2010

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Complement C1 Inhibitor Protein
Hereditary Angioedemas
Emergency Treatment
Prospective Studies
Virus Diseases
Edema
Body Weight
Therapeutics

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology

Cite this

Prospective study of rapid relief provided by C1 esterase inhibitor in emergency treatment of acute laryngeal attacks in hereditary angioedema. / Craig, Timothy J.; Wasserman, Richard L.; Levy, Robyn J.; Bewtra, Againdra K.; Schneider, Lynda; Packer, Flint; Yang, William H.; Keinecke, Heinz Otto; Kiessling, Peter C.

In: Journal of Clinical Immunology, Vol. 30, No. 6, 11.2010, p. 823-829.

Research output: Contribution to journalArticle

Craig, Timothy J. ; Wasserman, Richard L. ; Levy, Robyn J. ; Bewtra, Againdra K. ; Schneider, Lynda ; Packer, Flint ; Yang, William H. ; Keinecke, Heinz Otto ; Kiessling, Peter C. / Prospective study of rapid relief provided by C1 esterase inhibitor in emergency treatment of acute laryngeal attacks in hereditary angioedema. In: Journal of Clinical Immunology. 2010 ; Vol. 30, No. 6. pp. 823-829.
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abstract = "Introduction: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. Conclusion 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.",
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