Update on the Use of C1-Esterase Inhibitor Replacement Therapy in the Acute and Prophylactic Treatment of Hereditary Angioedema

@article{HenryLi2018UpdateOT,
  title={Update on the Use of C1-Esterase Inhibitor Replacement Therapy in the Acute and Prophylactic Treatment of Hereditary Angioedema},
  author={H. Henry Li and Marc A. Riedl and Jay M Kashkin},
  journal={Clinical Reviews in Allergy \& Immunology},
  year={2018},
  volume={56},
  pages={207-218}
}
In the vast majority of patients with hereditary angioedema (HAE), angioedema attacks are due to the quantitative or functional deficiency of C1-esterase inhibitor (C1-INH), which leads to increased vascular permeability and unregulated release of bradykinin. Exogenous administration of C1-INH is a rational way to restore the concentration and functional activity of this protein, regulate the release of bradykinin, and attenuate or prevent subcutaneous and submucosal edema associated with HAE… 
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DDDT_A_192475 3635..3646
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References

SHOWING 1-10 OF 60 REFERENCES
Self-administered C1 esterase inhibitor concentrates for the management of hereditary angioedema: usability and patient acceptance
  • H. Li
  • Medicine
    Patient preference and adherence
  • 2016
TLDR
The role of self-administered plasma-derived C1-INH concentrate therapy with Cinryze at home in the prophylaxis of HAE is summarized.
Safety of C1-esterase inhibitor in acute and prophylactic therapy of hereditary angioedema: findings from the ongoing international Berinert patient registry.
TLDR
This large pool of real-world clinical usage data in HAE further supports the extensive safety profile of 2 Berinert formulations when used on demand and/or for prophylaxis in both home and health care settings.
Cinryze™ as the first approved C1 inhibitor in the USA for the treatment of hereditary angioedema: approval, efficacy and safety
TLDR
The human plasma-derived C1 esterase inhibitor (Cinryze™), distributed by Lev Pharmaceuticals, was approved in October 2008 for the prevention of HAE attacks based on the results of a phase III clinical trial, and is described as the first approved chronic replacement treatment for the prophylaxis of H AE attacks.
Nanofiltered C1-esterase inhibitor for the acute management and prevention of hereditary angioedema attacks due to C1-inhibitor deficiency in children.
TLDR
In children, C1 INH-nf was well tolerated, provided relief from symptoms of hereditary angioedema attacks, and reduced the rate of attacks.
Recombinant human C1-inhibitor for the treatment of acute angioedema attacks in patients with hereditary angioedema.
TLDR
Administration of rhC1INH at 100 or 50 U/kg was highly effective as a treatment of acute attacks in patients with HAE and appeared to be safe and well tolerated.
Efficacy of human C1 esterase inhibitor concentrate compared with placebo in acute hereditary angioedema attacks.
TLDR
C1 esterase inhibitor concentrate given intravenously at a dose of 20 U/kg is an effective and safe treatment for acute abdominal and facial attacks in patients with hereditary angioedema, with a rapid onset of relief.
PSY27 A Cost-Effectiveness Comparison of Icatibant and C1-Esterase Inhibitor Concentrate for the Symptomatic Treatment of Acute Attacks of Types I and II Hereditary Angioedema in the UK Setting
TLDR
A cost-effectiveness model was performed to compare icatibant and C1-INH 20 IU/kg in a UK clinical perspective setting and is the first comparative health economic model presented for HAE.
Escalating doses of C1 esterase inhibitor (CINRYZE) for prophylaxis in patients with hereditary angioedema.
TLDR
Dose escalation of nanofiltered C1 inhibitor (human) up to 2500 U was well tolerated and reduced attack frequency in the majority of patients.
Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency.
TLDR
Intravenous self-administration of C1-inhibitor concentrate is a feasible and safe option and results in more rapid and more effective treatment or prevention of severe angioedema attacks in patients with C1 inhibitor deficiency.
Short-term prophylactic use of C1-inhibitor concentrate in hereditary angioedema: Findings from an international patient registry.
  • M. Magerl, M. Frank, +11 authors M. Rojavin
  • Medicine
    Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology
  • 2017
TLDR
The Berinert Registry, conducted between 2010 and 2014 at 30 US and 7 European sites, represents the largest clinical research evaluation of pnfC1-INH use and one of few data sets to describe its use for STP.
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1
2
3
4
5
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