|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
As there are limited agents for prophylaxis of HAE attacks, there is a high unmet need for a therapeutic agent for regulating pKal with a high degree of specificity.
|
30876891 |
2019 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
Lanadelumab (Takhzyro™), a first-in-class fully human monoclonal antibody against plasma kallikrein, has been approved in several countries, including Australia, Canada, those of the EU, Switzerland and the USA, for the prevention of hereditary angioedema (HAE) attacks in patients aged ≥ 12 years.
|
31560114 |
2019 |
|
Angioedemas, Hereditary
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Hereditary angioedema (HAE) with C1 esterase inhibitor (C1-INH) deficiency (C1-INH-HAE) is a rare disease characterized by diminished levels or dysfunctional activity of C1-INH, leading to dysregulated plasma kallikrein activity within the kallikrein-kinin pathway.
|
30539362 |
2019 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
Lanadelumab is a human monoclonal antibody against plasma kallikrein indicated for prevention of attacks of hereditary angioedema (HAE).
|
31347612 |
2019 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
Inhibitors of plasma kallikrein have recently been approved for prophylaxis of hereditary angioedema and are under investigation for use in other indications.
|
31530337 |
2019 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
Attacks recur with unpredictable frequency and severity, laryngeal edema is potentially lethal, and the disease burden may severely disrupt patients' lives.<b>Areas covered</b>: This review provides an overview of lanadelumab, a human monoclonal antibody targeted against plasma kallikrein that was recently approved for prevention of symptoms in C1-INH-HAE.<b>Expert opinion</b>: The phase III HELP Study demonstrated the efficacy of lanadelumab in reducing HAE attacks.
|
31721602 |
2019 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
Oral plasma kallikrein inhibitor BCX7353 for treatment of hereditary angioedema.
|
31635497 |
2019 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
With the aim of improving patients' quality of life, the therapeutic scenario has expanded over the years.<b>Areas covered</b>: The focus of the present review is lanadelumab, a fully human, κ-light-chain, monoclonal immunoglobulin G1 against plasma kallikrein, currently approved for long-term prophylaxis of C1-INH-HAE attacks in the USA and Canada and designated as an orphan drug by the European Medicines Agency.<b>Expert opinion</b>: Lanadelumab is able to inhibit plasma kallikrein with high selectivity and affinity.
|
31657963 |
2019 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
In sum, prekallikrein and its enzyme PKa are major targets in HAE providing much opportunity to improve the acute and chronic management of HAE.
|
29423395 |
2018 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
Effective inhibition of plasma kallikrein may have significant benefits for patients with hereditary angioedema due to deficiency of C1 inhibitor (C1-INH-HAE) by reducing the frequency of angioedema attacks.
|
29688579 |
2018 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
Oral Plasma Kallikrein Inhibitor for Prophylaxis in Hereditary Angioedema.
|
30044938 |
2018 |
|
Angioedemas, Hereditary
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We studied 249 C1-INH-HAE patients from 114 European families, and we explored possible associations of C1-INH-HAE clinical features with carriage of KLKB1-428G/A, combined or not with that of the functional F12-46C/T polymorphism.
|
29130992 |
2017 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
Inhibiting Plasma Kallikrein for Hereditary Angioedema Prophylaxis.
|
28225674 |
2017 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
Prophylactic therapy requires extended, repeated dosing and the results of this study will provide important data on the long-term safety and efficacy of lanadelumab, a monoclonal antibody inhibitor of plasma kallikrein for subcutaneous administration for the treatment of HAE.<i>Trial registration</i> NCT02741596.
|
29043014 |
2017 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
C1-INH replacement and specific inhibition of plasma kallikrein with ecallantide have been successful in the treatment of hereditary angioedema (HAE), a more common related disorder.
|
23406939 |
2013 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
Ecallantide is a potent and specific plasma kallikrein inhibitor approved for the treatment of acute attacks of HAE affecting any anatomic site.
|
22149337 |
2012 |
|
Angioedemas, Hereditary
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
C1(-)-inhibitor (C1(-)-INH) proteins from normal persons and members of eight different kindred with dysfunctional C1(-)-INH proteins associated with hereditary angioneurotic edema (HANE) were compared with respect to their inhibitory activity against purified preparations of C1s-, plasma kallikrein, activated forms of Hageman factor, and plasmin.
|
3965500 |
1985 |
|
Angioedemas, Hereditary
|
0.100 |
Biomarker
|
disease |
BEFREE |
To establish whether plasma kallikrein could activate prorenin under physiologic or pathologic conditions, prorenin to renin conversion was assessed at neutral pH in plasma deficient in C1 inhibitor (hereditary angioedema).
|
3889200 |
1985 |