C1 inhibitors for hereditary angioedema

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

C1 inhibitor (C1-INH) replacement is essential to the management of the acute episodes of hereditary angioedema, which result from the deficiency of the C1-INH. Similar to the endogenous protein, C1-INH preparations achieve blockage of the mechanisms leading to edema formation at multiple target sites. The largest body of experience has been accumulated with plasma-derived C1-INH as regards both the numbers of patients and attacks, and the duration of follow-up. In addition to their efficacy, the safety of plasma-derived C1-INH preparations has increased considerably due to the introduction of nanofiltration for elimination of viruses. Subsequently, the advanced use of recombinant DNA technology has made the production of Ruconest® (recombinant human [rh] C1-INH) possible. Currently, Ruconest (Rhucin) is the only recombinant C1-INH preparation available. RhC1INH has the same inhibitory properties as pdC1INH [101]. Phase I, II and III clinical studies demonstrated that rhC1-INH is an effective and safe drug for the treatment of all localization of hereditary angioedema attacks. When administered in a dose of 50 U/kg bodyweight, it reverts clinical symptoms rapidly and without significant adverse events, relevant abnormalities, or relapses. Moreover, its efficacy does not decrease during repeated administration. Absence of evidence for immunogenicity also support the safety of rhC1-INH.

Original languageEnglish
Title of host publicationCurrent and Emerging Options for Hereditary Angioedema Management
PublisherFuture Medicine Ltd.
Pages33-45
Number of pages13
ISBN (Print)9781780840208, 9781780841106
DOIs
Publication statusPublished - Aug 1 2012

Fingerprint

Hereditary Angioedemas
Complement C1 Inhibitor Protein
Safety
Recombinant DNA
Edema
Viruses
Technology
Recurrence
Pharmaceutical Preparations
conestat alpha
Therapeutics

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Farkas, H., & Varga, L. (2012). C1 inhibitors for hereditary angioedema. In Current and Emerging Options for Hereditary Angioedema Management (pp. 33-45). Future Medicine Ltd.. https://doi.org/10.2217/EBO.11.78

C1 inhibitors for hereditary angioedema. / Farkas, H.; Varga, L.

Current and Emerging Options for Hereditary Angioedema Management. Future Medicine Ltd., 2012. p. 33-45.

Research output: Chapter in Book/Report/Conference proceedingChapter

Farkas, H & Varga, L 2012, C1 inhibitors for hereditary angioedema. in Current and Emerging Options for Hereditary Angioedema Management. Future Medicine Ltd., pp. 33-45. https://doi.org/10.2217/EBO.11.78
Farkas H, Varga L. C1 inhibitors for hereditary angioedema. In Current and Emerging Options for Hereditary Angioedema Management. Future Medicine Ltd. 2012. p. 33-45 https://doi.org/10.2217/EBO.11.78
Farkas, H. ; Varga, L. / C1 inhibitors for hereditary angioedema. Current and Emerging Options for Hereditary Angioedema Management. Future Medicine Ltd., 2012. pp. 33-45
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