Results 51 to 60 of about 98,834 (305)

Activation of the Coagulation Cascade in C1-Inhibitor Deficiencies [PDF]

Blood, 1997
AbstractActivation of the contact and complement systems in C1-inhibitor deficiencies is thought to contribute to the pathogenesis of angioedema attacks by releasing kinins. Trigger stimuli of attacks may also activate coagulation. This is particularly important because experimental data suggest that thrombin, the main enzyme of the coagulation cascade,
M. Cugno, M. Cicardi, BOTTASSO, BIANCAMARIA, R. Coppola, R. Paonessa, P. Mannucci, A. Agostoni   +6 more
openaire   +3 more sources

The central role of endothelium in hereditary angioedema due to C1 inhibitor deficiency.

International Immunopharmacology, 2020
An impairment of the endothelial barrier function underlies a wide spectrum of pathological conditions. Hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) can be considered the "pathophysiological and clinical paradigm" of Paroxysmal ...
M. Wu, M. Bova, S. Berra, R. Senter, D. Parolin, S. Caccia, M. Cicardi   +6 more
semanticscholar   +1 more source

Pediatric hereditary angioedema due to C1-inhibitor deficiency

Allergy, Asthma & Clinical Immunology, 2010
Hereditary angioedema (HAE) resulting from the deficiency of the C1 inhibitor (C1-INH) is a rare, life-threatening disorder. It is characterized by attacks of angioedema involving the skin and/or the mucosa of the upper airways, as well as the intestinal
Farkas Henriette
doaj   +1 more source

The deficiency of C1 inhibitor and its treatment

Immunobiology, 2007
In this article, we review the traditional therapies of hereditary angioedema (HAE) that have been used for several years. Some of these therapies were proposed before the definition of the underlying defect and the understanding of the pathogenesis of the disease. We also describe new compounds under investigation at present as potential therapies for
M. Cicardi, L.C. Zingale
openaire   +2 more sources

Recurrent Angioedema with Abdominal and Genital Involvement in Childhood: Hereditary Angioedema Type 2 Disease due to C1 Inhibitor Functional Deficiency

İstanbul Medical Journal, 2020
Hereditary angioedema is a rare disorder characterized by recurrent angioedema attacks due to C1 inhibitor antigen or functional deficiency. Here, two cases with recurrent swelling on extremities, genital organs and face that were later diagnosed with C1
Öner Özdemir, Halime Çiçek
doaj   +1 more source

Pediatric Emergency Department Management of C1 Inhibitor Deficiency.

, 2022
20.500.12530/87857C1 inhibitor deficiency is a rare, potentially life-threatening syndrome. Acute attacks of angioedema may occur at any time, so the emergency department (ED) constitutes an indispensable component of its care.
García Sánchez, Paula, Plata Gallardo, Marta, Caballero Molina, María Teresa, Pedrosa Delgado, María, de Ceano-Vivas la Calle, María   +4 more
core   +1 more source

Hereditary Angioedema due to C1-Inhibitor Deficiency in Pediatric Patients in Croatia – First National Study, Diagnostic and Prophylactic Challenges

Acta Clinica Croatica, 2019
Hereditary angioedema (HAE) is a rare autosomal dominant disease with deficiency (type I) or dysfunction (type II) of C1 inhibitor, caused by mutations in the C1-INH gene, characterized by recurrent submucosal or subcutaneous edemas including skin ...
Ljerka Karadža-Lapić, Marko Barešić, Renata Vrsalović, Irena Ivković-Jureković, Saša Sršen, Ingrid Prkačin, Matija Rijavec, Draško Cikojević   +7 more
doaj   +1 more source

Successful long-term prophylaxis of hereditary pregnancy-associated angioedema with plasma-derived C1-inhibitor concentrate: a case report

Медицинская иммунология, 2021
Hereditary angioedema (HAE) is a rare autosomal dominant disease caused by quantitative (type I) or functional (type II) deficiency in C1 esterase inhibitor (C1-INH). It may be caused by new mutations in up to 20% of patients.
D. V. Demina, A. O. Makeeva, L. M. Kudelya, E. V. Novikova, V. A. Kozlov   +4 more
doaj   +1 more source

Deucrictibant for angioedema due to acquired C1-inhibitor deficiency: a randomized-controlled trial.

Journal of Allergy and Clinical Immunology
BACKGROUND Angioedema due to acquired C1-inhibitor deficiency is a very rare but serious disease, with an estimated prevalence of 1 per 500,000 persons.
Remy S. Petersen, Lauré M. Fijen, Johannes Kelder, D. Cohn   +3 more
semanticscholar   +1 more source

Successful C1 inhibitor short-term prophylaxis during redo mitral valve replacement in a patient with hereditary angioedema

Journal of Cardiothoracic Surgery, 2010
Hereditary angioedema is characterized by sudden episodes of nonpitting edema that cause discomfort and pain. Typically the extremities, genitalia, trunk, gastrointestinal tract, face, and larynx are affected by attacks of swelling.
Coleman Suzanne, Bernstein Jonathan A, Bonnin Arturo J   +2 more
doaj   +1 more source