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… 
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22 Citations
Background Citations
4
Methods Citations
3

22 Citations

Recombinant human C1 esterase inhibitor (Conestat alfa) for prophylaxis to prevent attacks in adult and adolescent patients with hereditary angioedema
TLDR
This review will critically appraise available information about rhC1-INH (conestat alfa) prophylactic treatment in adult and adolescent patients with congenital C1-inH deficiency to facilitate tailoring HAE treatment to patients’ needs.
Ecallantide for the treatment of acute attacks of hereditary angioedema due to C1-inhibitor deficiency
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.
TLDR
The current therapies available for the treatment of HAE-C1-INH, latest treatment guidelines, results of several studies demonstrating the efficacy and safety of the plasma-derived, pasteurized and nanofiltered C1-inH concentrate Berinert(®) (CSL Behring GmBH, Marburg, Germany), and future perspectives are discussed.
Acute Management of Hereditary Angioedema Attacks.
  • C. Katelaris
  • Medicine
    Immunology and allergy clinics of North America
  • 2017
Current pharmacotherapy of bradykinin-mediated angioedema
  • H. Farkas
  • Medicine
    Expert opinion on pharmacotherapy
  • 2013
TLDR
The range of targeted therapeutic options has increased in recent years and includes measures to handle emergencies, prevent edematous episodes and manage additional types of bradykinin-mediated angioedema.
Angiotensin-converting enzyme inhibitor-induced angioedema--a dangerous new epidemic.
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.
Acquired complement C1 esterase inhibitor deficiency in a patient with a rare SERPING1 variant with unknown significance
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.
Hereditary angioedema: what the gastroenterologist needs to know
  • M. Ali, M. Borum
  • Medicine
    Clinical and experimental gastroenterology
  • 2014
TLDR
Gastroenterologists can play a critical role in identifying and treating patients with HAE, and should have a high index of suspicion when encountering patients with recurrent, unexplained bouts of abdominal pain.
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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