Results 11 to 20 of about 98,834 (305)

Daratumumab-based treatment of monoclonal gammopathy–associated angioedema due to acquired C1-inhibitor deficiency [PDF]

Journal of Allergy and Clinical Immunology: Global
Daratumumab-based treatment could control severe, treatment-refractory, life-threatening angioedema due to acquired C1-inhibitor deficiency associated with monoclonal gammopathy.
Remy S. Petersen, MD, Lauré M. Fijen, MD, PhD, Laurens E. Franssen, MD, PhD, Josephine M.I. Vos, MD, PhD, Danny M. Cohn, MD, PhD   +4 more
doaj   +3 more sources

Lymphoproliferative disease and acquired C1 inhibitor deficiency

Haematologica, 2007
Angioedema due to acquired deficiency of the C1-inhibitor is a bridging condition between autoimmunity and lymphoproliferation. We report 32 patients with acquired C1 inhibitor deficiency: 23 have anti C1-inhibitor autoantibodies; 13 have monoclonal ...
Roberto Castelli, Daniela Lambertenghi Deliliers, Lorenza C. Zingale, Enrico Maria Pogliani, Marco Cicardi   +4 more
doaj   +4 more sources

How Angioedema Quality of Life Questionnaire Can Help Physicians in Treating C1-Inhibitor Deficiency Patients? [PDF]

Clin Rev Allergy Immunol, 2021
The Angioedema Quality of Life Questionnaire (AE-QoL) is an angioedema (AE)-specific validated questionnaire, which surveys the quality of life of diagnosed patients. The questionnaire has been used in multiple clinical trials. Our aim was to investigate
Balla Z, Ignácz B, Varga L, Kőhalmi KV, Farkas H.   +4 more
europepmc   +2 more sources

Berotralstat in hereditary angioedema due to C1 inhibitor deficiency: first real-world evidence from a Canadian center. [PDF]

Front Immunol
Background Hereditary angioedema due to C1 inhibitor deficiency is a rare genetic condition that causes recurrent swelling with consequent functional impairment and decreased quality of life.
Srinivasan C, Ritchie B, Adatia A.
europepmc   +2 more sources

The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor Deficiency. [PDF]

Int J Mol Sci, 2022
Background: Hereditary angioedema (HAE) is a rare, genetic disease caused by the decreased level or function of the C1 inhibitor. The primary mediator of symptoms in HAE is bradykinin acting through its two receptors, namely receptors 1 (BR1) and 2 (BR2).
Dyga W, Obtulowicz A, Mikolajczyk T, Bogdali A, Dubiela P, Obtulowicz K.   +5 more
europepmc   +2 more sources

The characteristics of upper airway edema in hereditary and acquired angioedema with C1-inhibitor deficiency. [PDF]

Clin Transl Allergy, 2021
Background Angioedemas localized in the upper airway are potentially life threatening, and without proper treatment, they may lead to death by suffocation. Upper airway edemas (UAE) in bradykinin‐mediated angioedemas can even be the first symptoms of the
Balla Z, Andrási N, Pólai Z, Visy B, Czaller I, Temesszentandrási G, Csuka D, Varga L, Farkas H.   +8 more
europepmc   +2 more sources

The Global Registry for Hereditary Angioedema due to C1-Inhibitor Deficiency. [PDF]

Clin Rev Allergy Immunol, 2021
Hereditary angioedema (HAE) is a rare condition, mostly due to genetic deficiency of complement C1 inhibitor (C1-INH). The rarity of HAE impedes extensive data collection and assessment of the impact of certain factors known to affect the course of this ...
Zanichelli A, Farkas H, Bouillet L, Bara N, Germenis AE, Psarros F, Varga L, Andrási N, Boccon-Gibod I, Castiglioni Roffia M, Rutkowski M, Cancian M.   +11 more
europepmc   +2 more sources

Hereditary angioedema due to C1 inhibitor deficiency in Belarus: epidemiology, access to diagnosis and seven novel mutations in SERPING1 gene. [PDF]

Clin Mol Allergy, 2021
Background Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare disease. Few states in developing countries have an adequate management of HAE, but none of them belongs to the former USSR area.
Guryanova I, Suffritti C, Parolin D, Zanichelli A, Ishchanka N, Polyakova E, Belevtsev M, Perego F, Cicardi M, Zharankova Y, Konoplya N, Caccia S, Gidaro A.   +12 more
europepmc   +2 more sources

Mechanisms of C1-Inhibitor Deficiency

Immunobiology, 2002
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, L.C. Zingale, TERLIZZI, MARIA ADELAIDE, A. Zanichelli, A. Folcioni, M. Cicardi   +5 more
openaire   +4 more sources

Psychometric study of the SF-36v2 in hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE). [PDF]

Orphanet J Rare Dis, 2022
Background The generic 36-item Short-Form Health Survey (SF-36v2) has been used to assess health related quality of life in adult patients with hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) even though it has not yet been validated ...
Palao-Ocharan P, Prior N, Pérez-Fernández E, Caminoa M, DV-HAE-QoL Study Group, Caballero T.   +5 more
europepmc   +2 more sources