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Safety and Usage of C1-Inhibitor in Hereditary Angioedema: Berinert Registry Data.

Publication ,  Journal Article
Riedl, MA; Bygum, A; Lumry, W; Magerl, M; Bernstein, JA; Busse, P; Craig, T; Frank, MM; Edelman, J; Williams-Herman, D; Feuersenger, H ...
Published in: J Allergy Clin Immunol Pract
2016

BACKGROUND: The plasma-derived, highly purified, nanofiltered C1-inhibitor concentrate (Berinert; "pnfC1-INH") is approved in the United States for treating hereditary angioedema (HAE) attacks and in many European countries for attack treatment and short-term prophylaxis. OBJECTIVE: The objective of this study was to describe safety and usage patterns of pnfC1-INH. METHODS: A multicenter, observational, registry was conducted between 2010 and 2014 at 30 United States and 7 European sites to obtain both prospective (occurring after enrollment) and retrospective (occurring before enrollment) safety and usage data on subjects receiving pnfC1-INH for any reason. RESULTS: Of 343 enrolled patients, 318 received 1 or more doses of pnfC1-INH for HAE attacks (11,848 infusions) or for prophylaxis (3142 infusions), comprising the safety population. Median dosages per infusion were 10.8 IU/kg (attack treatment) and 16.6 IU/kg (prophylaxis). Approximately 95% of infusions were administered outside of a health care setting. No adverse events (AEs) were reported in retrospective data. Among prospective data (n = 296 subjects; 9148 infusions), 252 AEs were reported in 85 (28.7%) subjects (rate of 0.03 events/infusion); 9 events were considered related to pnfC1-INH. Two thromboembolic events were reported in subjects with thrombotic risk factors. No patient was noted to have undergone viral testing for suspected blood-borne infection during registry participation. CONCLUSIONS: The findings from this large, international patient registry documented widespread implementation of pnfC1-INH self-administration outside of a health care setting consistent with current HAE guidelines. These real-world data revealed pnfC1-INH usage for a variety of reasons in patients with HAE and showed a high level of safety regardless of administration setting or reason for use.

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Published In

J Allergy Clin Immunol Pract

DOI

EISSN

2213-2201

Publication Date

2016

Volume

4

Issue

5

Start / End Page

963 / 971

Location

United States

Related Subject Headings

  • Young Adult
  • United States
  • Registries
  • Middle Aged
  • Male
  • Humans
  • Female
  • Europe
  • Complement C1 Inhibitor Protein
  • Child, Preschool
 

Citation

APA
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ICMJE
MLA
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Riedl, M. A., Bygum, A., Lumry, W., Magerl, M., Bernstein, J. A., Busse, P., … Berinert Registry investigators, . (2016). Safety and Usage of C1-Inhibitor in Hereditary Angioedema: Berinert Registry Data. J Allergy Clin Immunol Pract, 4(5), 963–971. https://doi.org/10.1016/j.jaip.2016.04.018
Riedl, Marc A., Anette Bygum, William Lumry, Markus Magerl, Jonathan A. Bernstein, Paula Busse, Timothy Craig, et al. “Safety and Usage of C1-Inhibitor in Hereditary Angioedema: Berinert Registry Data.J Allergy Clin Immunol Pract 4, no. 5 (2016): 963–71. https://doi.org/10.1016/j.jaip.2016.04.018.
Riedl MA, Bygum A, Lumry W, Magerl M, Bernstein JA, Busse P, et al. Safety and Usage of C1-Inhibitor in Hereditary Angioedema: Berinert Registry Data. J Allergy Clin Immunol Pract. 2016;4(5):963–71.
Riedl, Marc A., et al. “Safety and Usage of C1-Inhibitor in Hereditary Angioedema: Berinert Registry Data.J Allergy Clin Immunol Pract, vol. 4, no. 5, 2016, pp. 963–71. Pubmed, doi:10.1016/j.jaip.2016.04.018.
Riedl MA, Bygum A, Lumry W, Magerl M, Bernstein JA, Busse P, Craig T, Frank MM, Edelman J, Williams-Herman D, Feuersenger H, Rojavin M, Berinert Registry investigators. Safety and Usage of C1-Inhibitor in Hereditary Angioedema: Berinert Registry Data. J Allergy Clin Immunol Pract. 2016;4(5):963–971.
Journal cover image

Published In

J Allergy Clin Immunol Pract

DOI

EISSN

2213-2201

Publication Date

2016

Volume

4

Issue

5

Start / End Page

963 / 971

Location

United States

Related Subject Headings

  • Young Adult
  • United States
  • Registries
  • Middle Aged
  • Male
  • Humans
  • Female
  • Europe
  • Complement C1 Inhibitor Protein
  • Child, Preschool