Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
In vitro studies showed that C1-INH inhibits prekallikrein activation and bradykinin formation in a dose-dependent manner when added to the plasma of patients with HAE.
|
31655295 |
2020 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
Despite the efficacy of the on-demand treatment for the control of acute attacks of Hereditary Angioedema due to C1-Inhibitor Deficiency (C1-INH-HAE), the number and severity of attacks and the impairment in the quality of life of the affected patients have led to the development of a new monoclonal antibody, lanadelumab, directly addressed to the blockage of bradykinin, the principal mediator of vasodilation during angioedema attacks.
|
31695331 |
2019 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
Hereditary angioedema: Looking for bradykinin production and triggers of vascular permeability.
|
31574187 |
2019 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
Unregulated pKal activity can lead to hereditary angioedema (HAE) following excess bradykinin release.
|
30876891 |
2019 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
Neutrophils impact on processes preceding the formation of bradykinin, a major swelling mediator in hereditary angioedema (HAE), yet their potential role in HAE pathogenesis has not been sufficiently studied.
|
31236065 |
2019 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
Uncontrolled production of BK due to inefficient regulation of the plasma contact system, increased activity of contact and coagulation factors or a deficient regulation of BK receptor-triggered intracellular signalling are on the basis of HAE pathology.
|
31279849 |
2019 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
These results highlight a novel and specific approach to target PKK for the treatment of HAE and other diseases involving contact system activation and overproduction of bradykinin.
|
30817230 |
2019 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
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.
|
29909591 |
2019 |
Angioedemas, Hereditary
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
One of these conditions is hereditary angioedema (HAE), a rare disease with characteristic attacks of aggressive tissue swelling due to unregulated production and activity of the inflammatory mediator bradykinin.
|
31507620 |
2019 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
The Icatibant Outcome Survey (IOS; NCT01034969) is a Shire-sponsored, international, observational study monitoring the safety and effectiveness of icatibant, a bradykinin B2 receptor antagonist approved for the acute treatment of adults with hereditary angioedema with C1 inhibitor deficiency (HAE-C1-INH).
|
30176179 |
2019 |
Angioedemas, Hereditary
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
HAE is caused by SERPING1 gene mutations resulting in decreased or dysfunctional plasma protease C1 inhibitor (C1-INH) leading to a loss of inhibition of plasma kallikrein activity with subsequent cleavage of high-molecular weight kininogen and release of bradykinin.
|
31347612 |
2019 |
Angioedemas, Hereditary
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Reduced plasma levels of C1INH lead to enhanced activation of the contact system, triggering high levels of bradykinin and increased vascular permeability, but the cellular mechanisms leading to low C1INH levels (20%-30% of normal) in heterozygous HAE type I patients remain obscure.
|
30398465 |
2019 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
Recurrent angioedema (AE) is an important clinical problem in the context of chronic urticaria (mast cell mediator-induced), ACE-inhibitor intake and hereditary angioedema (both bradykinin-mediated).
|
31815297 |
2019 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
Bradykinin increases vascular permeability and is ultimately responsible for the episodes of swelling characteristic of HAE.
|
30463937 |
2019 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
Bradykinin, which is considered to be a byproduct of the plasma contact system during in vitro coagulation, is the main disease mediator in HAE.
|
29920929 |
2018 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
The swelling in C1-INH-HAE is a result of impaired regulation of bradykinin production.
|
30479631 |
2018 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
Comparing Pathways of Bradykinin Formation in Whole Blood From Healthy Volunteers and Patients With Hereditary Angioedema Due to C1 Inhibitor Deficiency.
|
30333824 |
2018 |
Angioedemas, Hereditary
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
These results confirm and extend our previous findings indicating that in HAE there is operating an inflammatory activation process, which involves also T helper 17 (Th17) cytokines and TGF-β isoforms, associated with localized angioedema attacks and characterized by elevated bradykinin levels.
|
29623491 |
2018 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
Hereditary angioedema (HAE) is a rare genetic disease resulting in unpredictable and potentially life-threatening subcutaneous and submucosal attacks mediated by the vasoactive peptide, bradykinin.
|
29569504 |
2018 |
Angioedemas, Hereditary
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is a form of bradykinin-mediated angioedema.
|
29214395 |
2018 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
Icatibant is a bradykinin B2-receptor antagonist used for the treatment of hereditary angioedema attacks resulting from C1-inhibitor deficiency.
|
29599966 |
2018 |
Angioedemas, Hereditary
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Activation of the kallikrein/bradykinin pathway at the endothelial cell level has a relevant pathogenetic role in acute HAE attacks.
|
29729940 |
2018 |
Angioedemas, Hereditary
|
0.400 |
Biomarker
|
disease |
BEFREE |
The agents used so far for the acute management of HAE attacks act by blocking the release of bradykinin, or its binding to its receptor.
|
29226721 |
2018 |
Angioedemas, Hereditary
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
To characterize the clinical profile, management and outcomes of patients with HAE type I and II from three specialist centres in the UK using data from the Icatibant Outcome Survey (IOS; Shire, Zug, Switzerland), an international observational study monitoring safety and effectiveness of icatibant, a selective bradykinin B2 receptor antagonist.
|
30127805 |
2018 |
Angioedemas, Hereditary
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Cytokine and estrogen stimulation of endothelial cells augments activation of the prekallikrein-high molecular weight kininogen complex: Implications for hereditary angioedema.
|
27826093 |
2017 |