Current Management Options for Hereditary Angioedema

@article{Bork2012CurrentMO,
  title={Current Management Options for Hereditary Angioedema},
  author={Konrad Bork},
  journal={Current Allergy and Asthma Reports},
  year={2012},
  volume={12},
  pages={273-280}
}
  • K. Bork
  • Published 23 June 2012
  • Biology, Medicine
  • Current Allergy and Asthma Reports
The aim of treatment of hereditary angioedema (HAE) due to C1 esterase inhibitor deficiency (HAE-C1-INH) is either treating acute attacks or preventing attacks by using prophylactic treatment. For treating acute attacks, plasma-derived C1 inhibitor (C1-INH) concentrates, a bradykinin B2 receptor antagonist, and a recombinant human C1-INH are available in Europe. In the United States, a plasma-derived C1-INH concentrate, a bradykinin B2 receptor antagonist, and a plasma kallikrein inhibitor have… 
Recombinant human C1 esterase inhibitor (Conestat alfa) for prophylaxis to prevent attacks in adult and adolescent patients with hereditary angioedema
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TLDR
Ecallantide is a highly specific and potent plasma kallikrein inhibitor approved for the treatment of acute attacks of HAE-C1INH, and in two randomized, placebo-controlled, Phase III clinical trials, 30 mg of subcutaneously administered ecallantide demonstrated significant, rapid and durable symptom relief compared with placebo.
Pasteurized and nanofiltered, plasma-derived C1 esterase inhibitor concentrate for the treatment of hereditary angioedema.
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TLDR
Complement C1 inhibitor concentrate and bradykinin receptor antagonists, normally used to treat patients with hereditary angioedema, have shown good results when used in patients with bradyKinin-mediated angioingema.
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TLDR
A 35-year-old man presenting with upper-airway AE progressing rapidly and promptly required cricothyroidotomy is described, unusual to have this disease before the age of 40 years and antiallergic medication is not effective in BK-mediated AE.
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  • Medicine
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  • 2014
TLDR
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Critical upper airway obstruction in sporadic angioedema responding to C1-esterase inhibitor
TLDR
This case of recurrent oropharyngeal angioedema in a 16-year-old boy with a history of sickle cell disease and thrombocytopenia and with no family history of angIOedema is described, exemplifying that even in cases of sporadic angioEDema, treatment with C1-INH may be an effective and life-saving management strategy.
C1-Esterase Inhibitor: Biological Activities and Therapeutic Applications
TLDR
An overview of the structure and functions of human C1-INH, its role in HAE, summarizes published data available for recently approved C1 -INH therapeutic products, and considers possible use of C1,INH for other applications are provided.
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