Hereditary angioedema

PJ Busse, SC Christiansen - New England Journal of Medicine, 2020 - Mass Medical Soc
Hereditary Angioedema Hereditary angioedema is a rare genetic disease that may include
recurrent attacks of cutaneous angioedema, severe abdominal pain, and airway …

Differences and similarities in the mechanisms and clinical expression of bradykinin-mediated vs. mast cell–mediated angioedema

M Maurer, M Magerl - Clinical Reviews in Allergy & Immunology, 2021 - Springer
Angioedema (AE), transient localized swelling due to extravasated fluid, is commonly
classified as mast cell mediator-induced, bradykinin-mediated or of unknown cause. AE …

[HTML][HTML] The international WAO/EAACI guideline for the management of hereditary angioedema–the 2021 revision and update

M Maurer, M Magerl, S Betschel, W Aberer… - World Allergy …, 2022 - Elsevier
Hereditary Angioedema (HAE) is a rare and disabling disease for which early diagnosis and
effective therapy are critical. This revision and update of the global WAO/EAACI guideline on …

The international WAO/EAACI guideline for the management of hereditary angioedema–the 2017 revision and update

M Maurer, M Magerl, I Ansotegui… - World Allergy …, 2018 - Springer
Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and
appropriate therapy are essential. This update and revision of the global guideline for HAE …

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

H Farkas, I Martinez‐Saguer, K Bork, T Bowen… - Allergy, 2017 - Wiley Online Library
Background The consensus documents published to date on hereditary angioedema with
C1 inhibitor deficiency (C1‐INH‐HAE) have focused on adult patients. Many of the previous …

Prophylactic use of an anti-activated factor XII monoclonal antibody, garadacimab, for patients with C1-esterase inhibitor-deficient hereditary angioedema: a …

T Craig, M Magerl, DS Levy, A Reshef, WR Lumry… - The Lancet, 2022 - thelancet.com
Background Hereditary angioedema is associated with dysregulation of the kallikrein–kinin
system. Factor XII (FXII) is a key initiator of the kallikrein–kinin system, which produces …

HAE pathophysiology and underlying mechanisms

BL Zuraw, SC Christiansen - Clinical reviews in allergy & immunology, 2016 - Springer
Remarkable progress in understanding the pathophysiology and underlying mechanisms of
hereditary angioedema has led to the development of effective treatment for this disorder …

[HTML][HTML] Hereditary angioedema: the plasma contact system out of control

S De Maat, ZLM Hofman, C Maas - Journal of Thrombosis and Haemostasis, 2018 - Elsevier
The plasma contact system contributes to thrombosis in experimental models. Even though
our standard blood coagulation tests are prolonged when plasma lacks contact factors, this …

Multiple roles of complement MASP-1 at the interface of innate immune response and coagulation

J Dobó, V Schroeder, L Jenny, L Cervenak… - Molecular …, 2014 - Elsevier
MASP-1 is a versatile serine protease that cleaves a number of substrates in human blood.
In recent years it became evident that besides playing a crucial role in complement …

Plasminflammation—an emerging pathway to bradykinin production

C Maas - Frontiers in Immunology, 2019 - frontiersin.org
Plasminogen activation is essential for fibrinolysis—the breakdown of fibrin polymers in
blood clots. Besides this important function, plasminogen activation participates in a wide …