Abstracts of the 14th C1-inhibitor Deficiency and Angioedema Workshop [PDF]
doaj +2 more sources
Background Hereditary angioedema associated to C1 inhibitor deficiency (C1-INH-HAE) is a pathological condition characterized by episodes of subcutaneous swelling and it is frequently associated with discomfort and social impairment of the patients, due ...
Livia Savarese +9 more
doaj +1 more source
C1 inhibitor deficiency: 2014 United Kingdom consensus document [PDF]
C1 inhibitor deficiency is a rare disorder manifesting with recurrent attacks of disabling and potentially life-threatening angioedema. Here we present an updated 2014 United Kingdom consensus document for the management of C1 inhibitor-deficient ...
Ashworth, F +11 more
core +3 more sources
Hereditary angioedema (HAE) in children and adolescents : a consensus on therapeutic strategies [PDF]
Hereditary angioedema due to C1 inhibitor (C1 esterase inhibitor) deficiency (types I and II HAE-C1-INH) is a rare disease that usually presents during childhood or adolescence with intermittent episodes of potentially life-threatening angioedema ...
Aberer, Werner +11 more
core +1 more source
International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1-Inhibitor deficiency [PDF]
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 recommendations have not been adapted to pediatric patients.
Agostoni +122 more
core +3 more sources
The hereditary angioedema burden of illness study in Europe (HAE-BOIS-Europe) : background and methodology [PDF]
Background: Hereditary angioedema (HAE) is a rare but serious disease marked by swelling attacks in the extremities, face, trunk, airway, or abdominal areas that can be spontaneous or the result of trauma and other triggers.
Aygören-Pürsün, Emel +7 more
core +2 more sources
Mechanisms of C1-Inhibitor Deficiency
C1 inhibitor (C1-Inh) is a protease inhibitor of the serpin family. It interacts and forms complexes with several serine proteases although not all these interactions were proved to be relevant in vivo. Based on studies in deficient patients, C1-Inh appears pivotal in regulating the activation of complement classical pathway and of contact system.
E. Pappalardo +5 more
openaire +3 more sources
Risk of angioedema following invasive or surgical procedures in HAE type I and II : the natural history [PDF]
Background: Hereditary angioedema (HAE), caused by deficiency in C1-inhibitor (C1-INH), leads to unpredictable edema of subcutaneous tissues with potentially fatal complications.
Aygören-Pürsün, Emel +4 more
core +1 more source
Abstracts of 11th C1-inhibitor Deficiency & Angioedema Workshop [PDF]
doaj +2 more sources
Paths reunited: initiation of the classical and lectin pathways of complement activation [PDF]
Understanding the structural organisation and mode of action of the initiating complex of the classical pathway of complement activation (C1) has been a central goal in complement biology since its isolation almost 50 years ago.
Keeble, Anthony H. +4 more
core +1 more source

