Assessment and management of disease burden and quality of life in patients with hereditary angioedema: a consensus report

K Bork, JT Anderson, T Caballero, T Craig… - Allergy, Asthma & …, 2021 - Springer
Background Hereditary angioedema (HAE) is a rare disease characterized by
unpredictable, potentially life-threatening attacks, resulting in significant physical and …

Defective glycosylation of coagulation factor XII underlies hereditary angioedema type III

J Björkqvist, S de Maat… - The Journal of …, 2015 - Am Soc Clin Investig
Hereditary angioedema type III (HAEIII) is a rare inherited swelling disorder that is
associated with point mutations in the gene encoding the plasma protease factor XII (FXII) …

Hereditary angioedema: an update on causes, manifestations and treatment

HJ Longhurst, K Bork - British Journal of Hospital Medicine, 2019 - magonlinelibrary.com
Hereditary angioedema is a rare genetic disorder caused by deficiency of C1 esterase
inhibitor (C1-INH) and characterized by recurrent episodes of severe swelling that affect the …

[HTML][HTML] Evaluation and management of angioedema in the emergency department

BJ Long, A Koyfman, M Gottlieb - Western Journal of Emergency …, 2019 - ncbi.nlm.nih.gov
Angioedema is defined by non-dependent, non-pitting edema that affects several different
sites and is potentially life-threatening due to laryngeal edema. This narrative review …

US Hereditary Angioedema Association Medical Advisory Board 2013 recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency

BL Zuraw, A Banerji, JA Bernstein, PJ Busse… - The Journal of Allergy …, 2013 - Elsevier
Background The treatment of hereditary angioedema (HAE) has undergone dramatic
changes as newer medicines have become available in recent years. Optimal care of these …

Pathogenesis of hereditary angioedema: the role of the bradykinin-forming cascade

AP Kaplan, K Joseph - Immunology and Allergy Clinics, 2017 - immunology.theclinics.com
Hereditary angioedema (HAE) represents a prototypic disorder in which episodic swelling,
that is, angioedema, is dependent on the generation of bradykinin. In contrast with the …

Plasma kallikrein: the bradykinin-producing enzyme

J Björkqvist, A Jämsä, T Renné - Thrombosis and haemostasis, 2013 - thieme-connect.com
Plasma prekallikrein is the liver-derived precursor of the trypsin-like serine protease plasma
kallikrein (PK) and circulates in plasma bound to high molecular weight kininogen. The …

A consensus parameter for the evaluation and management of angioedema in the emergency department

JJ Moellman, JA Bernstein, C Lindsell… - Academic …, 2014 - Wiley Online Library
Despite its relatively common occurrence and life‐threatening potential, the management of
angioedema in the emergency department (ED) is lacking in terms of a structured approach …

Risk of laryngeal edema and facial swellings after tooth extraction in patients with hereditary angioedema with and without prophylaxis with C1 inhibitor concentrate: a …

K Bork, J Hardt, P Staubach-Renz, G Witzke - Oral Surgery, Oral Medicine …, 2011 - Elsevier
Objective Tooth extractions may trigger clinical symptoms of hereditary angioedema due to
C1 inhibitor deficiency (HAE-C1-INH). The aim of this study was to determine how many …

Pathogenic mechanisms of bradykinin mediated diseases: dysregulation of an innate inflammatory pathway

AP Kaplan, K Joseph - Advances in immunology, 2014 - Elsevier
Binding of negatively charged macromolecules to factor XII induces a conformational
change such that it becomes a substrate for trace amounts of activated factor present in …