Human C1-Esterase Inhibitor Concentrate (Berinert®)
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▴ Human C1-esterase inhibitor concentrate (C1-INH concentrate) is derived from human plasma and is indicated for the treatment of acute episodes of hereditary angioedema.
▴ Intravenous C1-INH concentrate provided rapid symptom relief in patients with acute abdominal or facial episodes associated with type I or II hereditary angioedema, according to the results of the randomized, double-blind, multicenter, placebo-controlled IMPACT-1 trial. The median time to the onset of symptom relief was significantly shorter with C1-INH concentrate 20 U/kg than with placebo (0.5 vs 1.5 hours). A subsequent noncomparative extension trial (IMPACT-2) showed the ongoing efficacy of ‘on-demand’ treatment with C1-INH concentrate; overall, the onset of symptom relief occurred in a median 30 minutes.
▴ Several additional studies demonstrated the efficacy of intravenous C1-INH concentrate in patients with laryngeal edema, skin swellings, or abdominal episodes associated with type I or II hereditary angioedema.
▴ Self-administered home therapy with intravenous C1-INH concentrate significantly improved health-related quality of life in patients with hereditary angioedema. Dermatology Life Quality Index and Short Form 36-item Health Survey scores were significantly improved from baseline by self-administered home therapy with C1-INH concentrate.
▴ Intravenous C1-INH concentrate was well tolerated in patients with hereditary angioedema, with no confirmed cases of viral transmission.
KeywordsSymptom Relief Danazol Acute Episode Dermatology Life Quality Index Laryngeal Edema
Acknowledgments and Disclosures
The manuscript was reviewed by: M. Cicardi, Universitià degli Studi di Milan, Dipartimento di Scienze Cliniche “Luigi Sacco”, Ospedale Luigi Sacco, Milan, Italy; H. Farkas, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
The preparation of this review was not supported by any external funding. During the peer review process, the manufacturer of the agent under review was offered an opportunity to comment on this article. Changes resulting from comments received were made on the basis of scientific and editorial merit.
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