Results 1 to 10 of about 3,089,224 (331)

C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency [PDF]

Orphanet Journal of Rare Diseases, 2023
Background Autoantibodies against C1-inhibitor (C1-INH-Ab) have a diagnostic value in acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE), even though antibodies can circulate in complexes, which can be undetectable by proven methods. Our aim
Zsofia Polai, Erika Kajdacsi, Laszlo Cervenak, Zsuzsanna Balla, Szabolcs Benedek, Lilian Varga, Henriette Farkas   +6 more
doaj   +3 more sources

Secreted Phospholipases A2 in Hereditary Angioedema With C1-Inhibitor Deficiency [PDF]

Frontiers in Immunology, 2018
Background Hereditary angioedema (HAE) caused by deficiency (type I) or dysfunction (type II) of the C1 inhibitor protein (C1-INH-HAE) is a disabling, potentially fatal condition characterized by recurrent episodes of swelling.
Stefania Loffredo, Anne Lise Ferrara, Maria Bova, Francesco Borriello, Chiara Suffritti, Nóra Veszeli, Angelica Petraroli, Maria Rosaria Galdiero, Gilda Varricchi, Francescopaolo Granata, Andrea Zanichelli, Henriette Farkas, Marco Cicardi, Gérard Lambeau, Gianni Marone   +14 more
semanticscholar   +6 more sources

Mechanisms involved in hereditary angioedema with normal C1-inhibitor activity. [PDF]

Front Physiol, 2023
Patients with the inherited disorder hereditary angioedema (HAE) suffer from episodes of soft tissue swelling due to excessive bradykinin production.
Shamanaev A, Dickeson SK, Ivanov I, Litvak M, Sun MF, Kumar S, Cheng Q, Srivastava P, He TZ, Gailani D.   +9 more
europepmc   +2 more sources

Clinical features of genetically characterized types of hereditary angioedema with normal C1 inhibitor: a systematic review of qualitative evidence [PDF]

Orphanet Journal of Rare Diseases, 2020
Background Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH) (HAEnCI) is associated with skin swellings, abdominal attacks, and the risk of asphyxia due to upper airway obstruction. Several different gene mutations linked to the HAE phenotype
Konrad Bork, Thomas Machnig, K. Wulff, Guenther Witzke, Subhransu Prusty, Jochen Hardt   +5 more
openalex   +2 more sources

Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate

Orphanet Journal of Rare Diseases, 2019
Background Acquired angioedema due to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is a serious condition that may result in life-threatening asphyxiation due to laryngeal edema. It is associated with malignant B-cell lymphoma and other disorders.
Konrad Bork, Petra Staubach-Renz, Jochen Hardt   +2 more
doaj   +2 more sources

Edema of the Scrotum and Penile Shaft: An Uncommon Initial Presentation of Acquired Angioedema With Low C1-Inhibitor [PDF]

Case Reports in Urology
Acquired angioedema with low C1-inhibitor (AAE-C1-INH) is a rare disorder characterized by an acquired deficiency in the C1 esterase inhibitor (C1-INH). This case report describes a 79-year-old patient presenting to the emergency department for painless ...
Meghan V. Matheny, Timothy Craig, Joseph Y. Clark   +2 more
doaj   +2 more sources

C1-inhibitor to prevent intracerebral hemorrhage-related secondary brain injury [PDF]

Fluids and Barriers of the CNS
Background Preclinical studies indicate that the systemic application of C1-inhibitor, clinically used to treat hereditary angioedema, reduces secondary brain injury after ischemic stroke. This study assessed the effect of C1-inhibitor on secondary brain
Kevin Akeret, Bart R. Thomson, Subhajit Ghosh, Marc Nolte, Urs Fischer, Rok Humar, Luca Regli, Dominik J. Schaer, Michael Hugelshofer, Raphael M. Buzzi   +9 more
doaj   +2 more sources

Editorial: C1 inhibitor deficiency and angioedema [PDF]

Frontiers in Allergy, 2022
Henriette Farkas, Anastasios E. Germenis, Hilary Longhurst   +2 more
doaj   +2 more sources

A nationwide study of acquired C1-inhibitor deficiency in France

Medicine, 2016
Acquired angioedema (AAE) due to C1-inhibitor (C1INH) deficiency is rare. Treatment options for acute attacks are variable and used off-label. Successful treatment of the associated lymphoma with rituximab seems to prevent acute attacks in subjects with ...
Delphine Gobert, Romain Paule, D. Ponard, Pierre Lévy, Véronique Frémeaux‐Bacchi, Laurence Bouillet, Isabelle Boccon‐Gibod, Christian Drouet, Stéphane Gayet, David Launay, Ludovic Martin, A. Mékinian, Véronique Leblond, O. Fain   +13 more
openalex   +2 more sources

C1 inhibitor deficiency enhances contact pathway-mediated activation of coagulation and venous thrombosis. [PDF]

Blood, 2023
Grover SP, Kawano T, Wan J, Tanratana P, Polai Z, Shim YJ, Snir O, Brækkan S, Dhrolia S, Kasthuri RR, Bendapudi PK, Bendapudi PK, McCrae KR, Wolberg AS, Hansen JB, Farkas H, Mackman N.   +16 more
europepmc   +2 more sources