Biomarkers in hereditary angioedema

G Porebski, M Kwitniewski, A Reshef - Clinical Reviews in Allergy & …, 2021 - Springer
A biomarker is a defined characteristic measured as an indicator of normal, biologic,
pathogenic processes, or biological responses to an exposure or intervention. Diagnostic …

Prevention of hereditary angioedema attacks with a subcutaneous C1 inhibitor

H Longhurst, M Cicardi, T Craig, K Bork… - New England journal …, 2017 - Mass Medical Soc
Background Hereditary angioedema is a disabling, potentially fatal condition caused by
deficiency (type I) or dysfunction (type II) of the C1 inhibitor protein. In a phase 2 trial, the use …

“Nuts and bolts” of laboratory evaluation of angioedema

H Farkas, N Veszeli, E Kajdácsi, L Cervenak… - Clinical reviews in …, 2016 - Springer
Angioedema, as a distinct disease entity, often becomes a clinical challenge for physicians,
because it may cause a life-threatening condition, whereas prompt and accurate laboratory …

A focused parameter update: Hereditary angioedema, acquired C1 inhibitor deficiency, and angiotensin-converting enzyme inhibitor–associated angioedema

BL Zuraw, JA Bernstein, DM Lang, T Craig… - Journal of allergy and …, 2013 - Elsevier
These parameters were developed by the Joint Task Force on Practice Parameters (JTFPP),
representing the American Academy of Allergy, Asthma & Immunology (AAAAI); the …

Mutational spectrum and phenotypes in Danish families with hereditary angioedema because of C1 inhibitor deficiency

A Bygum, CR Fagerberg, D Ponard, N Monnier… - Allergy, 2011 - Wiley Online Library
To cite this article: Bygum A, Fagerberg CR, Ponard D, Monnier N, Lunardi J, Drouet C.
Mutational spectrum and phenotypes in Danish families with hereditary angioedema …

Cleavage of kininogen and subsequent bradykinin release by the complement component: mannose-binding lectin-associated serine protease (MASP)-1

J Dobó, B Major, KA Kékesi, I Szabó, M Megyeri… - PloS one, 2011 - journals.plos.org
Bradykinin (BK), generated from high-molecular-weight kininogen (HK) is the major
mediator of swelling attacks in hereditary angioedema (HAE), a disease associated with C1 …

Recombinant human C1 esterase inhibitor for prophylaxis of hereditary angio-oedema: a phase 2, multicentre, randomised, double-blind, placebo-controlled …

MA Riedl, V Grivcheva-Panovska, D Moldovan, J Baker… - The Lancet, 2017 - thelancet.com
Background Hereditary angio-oedema is a recurrent, oedematous disorder caused by
deficiency of functional C1 inhibitor. Infusions of plasma-derived C1 esterase inhibitor deter …

Phase II study results of a replacement therapy for hereditary angioedema with subcutaneous C1‐inhibitor concentrate

BL Zuraw, M Cicardi, HJ Longhurst, JA Bernstein… - Allergy, 2015 - Wiley Online Library
Background Hereditary angioedema (HAE) due to C1 inhibitor deficiency manifests as
recurrent swelling attacks that can be disabling and sometimes fatal. Long‐term prophylaxis …

Applying complement therapeutics to rare diseases

ES Reis, DC Mastellos, D Yancopoulou, AM Risitano… - Clinical …, 2015 - Elsevier
Around 350 million people worldwide suffer from rare diseases. These may have a genetic,
infectious, or autoimmune basis, and several include an inflammatory component …

Hereditary angioedema caused by C1-esterase inhibitor deficiency: a literature-based analysis and clinical commentary on prophylaxis treatment strategies

RG Gower, PJ Busse, E Aygören-Pürsün… - World Allergy …, 2011 - Springer
Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-
dominant disease resulting from a mutation in the C1-inhibitor gene. HAE is characterized …