Results 31 to 40 of about 214,320 (304)

Successful treatment of post-pericardiotomy syndrome via C1 inhibitor replacement therapy in a hereditary angioedema patient with Marfan syndrome

The Turkish Journal of Pediatrics, 2023
Background. Hereditary angioedema with C1 inhibitor deficiency (HAE-C1INH) is caused by dysfunctional C1-INH protein due to mutations in the SERPING1 gene encoding C1-INH.
Ezgi Topyıldız, Handan Duman Şenol, Figen Gülen, Esen Demir, Nihal Mete Gökmen   +4 more
doaj   +1 more source

Na⁺ entry through heteromeric TRPC4/C1 channels mediates (-)Englerin A-induced cytotoxicity in synovial sarcoma cells [PDF]

, 2017
The sesquiterpene (-)Englerin A (EA) is an organic compound from the plant Phyllanthus engleri which acts via heteromeric TRPC4/C1 channels to cause cytotoxicity in some types of cancer cell but not normal cells. Here we identified selective cytotoxicity
A Batova, A Beck, A Greka, A Riccio, AL Jackson, BC Fuchs, C Carson, C Sourbier, C Sourbier, CE Herzog, EL Spurrell, FJ Sulzmaier, H Al-Hussaini, H Suzuki, HN Rubaiy, J Myeong, J Myeong, JE Camacho Londono, JM David, JM Richter, KN Westlund, M Miller, MJ Ludlow, N Utsunomiya-Tate, R Ratnayake, RT Williams, T Rodrigues, U Storch, V Caropreso, VV Tsvilovskyy, Y Akbulut, Y Itoh, Z Wu   +32 more
core   +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   +1 more source

The hereditary angioedema burden of illness study in Europe (HAE-BOIS-Europe) : background and methodology [PDF]

, 2012
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, Beusterien, Kathleen, Boysen, Henrik B., Bygum, Anette, Caballero, Teresa, Gholizadeh, Shadi, Musingarimi, Patience, Wait, Suzanne   +7 more
core   +2 more sources

WAO guideline for the management of hereditary angioedema [PDF]

, 2012
Hereditary Angioedema (HAE) is a rare disease and for this reason proper diagnosis and appropriate therapy are often unknown or not available for physicians and other health care providers.
Aygören-Pürsün, Emel, Bork, Konrad, Bowen, Tom, Boysen, Henrik B., Craig, Timothy, Farkas, Henriette, Grumach, Anete, Katelaris, Constance H., Lockey, Richard, Longhurst, Hilary, Lumry, William, Magerl, Markus, Martinez-Saguer, Immaculada, Maurer, Marcus, Nast, Alexander, Pawankar, Ruby, Ritchie, Bruce, Zuraw, Bruce   +17 more
core   +1 more source

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1-Inhibitor deficiency [PDF]

, 2016
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, Agostoni, Agostoni, Altorjai, Andrew, Assadi, Aygoren-Pursun, Aygoren-Pursun, Bautista-Llacer, Bennett, Berkun, Betschel, Bork, Bork, Bork, Bork, Bork, Bouillet, Bouillet, Bouillet, Bowen, Bowen, Bowen, Bygum, Caballero, Caballero, Caballero, Caballero, Castelli, Chagas, Church, Cicardi, Cicardi, Cicardi, Cicardi, Cicardi, Craig, Craig, Craig, Craig, Craig, Csuka, Cugno, Czaller, Dinkel, Doherty, Ebo, El-Hachem, Fabiani, Farkas, Farkas, Farkas, Farkas, Farkas, Farkas, Farkas, Farkas, Farkas, Foix-L'Helias, Fouche, Gomez-Traseira, Gompels, Grumach, Grumach, Hogasen, Hubiche, Janardhanan, Kalmar, Kaplan, Kelemen, Kim, Kreuz, Kruger, Levy, Li, Lindecken, Longhurst, Lumry, MacGinnitie, Magerl, Martinez-Saguer, Martinez-Saguer, Martinez-Saguer, Martinez-Saguer, McCollough, Nanda, Nielsen, Nurnberger, O'Bier, Ohsawa, Pappalardo, Pappalardo, Pedrosa, Petraroli, Prior, Pritzker, Quintana, Rasmussen, Ren, Riedl, Riedl, Roach, Roche, Sanchez, Sanhueza, Shah, Speletas, Strawczynski, Tuong, Veszeli, Visy, Wahn, Wang, Wasserman, Wilson, Zanichelli, Zanichelli, Zilberberg, Zotter, Zotter, Zuraw, Zuraw, Zuraw   +122 more
core   +3 more sources

Formation of C1s-C1-inhibitor, kallikrein-C1-inhibitor, and plasmin- alpha 2-plasmin-inhibitor complexes during cardiopulmonary bypass [PDF]

Blood, 1989
Abstract Stimulation of platelets and neutrophils occurs during clinical cardiopulmonary bypass. We investigated whether the classical complement, contact, or fibrinolytic pathways are activated as potential sources of neutrophil agonists.
Y T, Wachtfogel, P C, Harpel, L H, Edmunds, R W, Colman   +3 more
openaire   +3 more sources

Recombinant and Plasma-Purified Human C1 Inhibitor for the Treatment of Hereditary Angioedema

World Allergy Organization Journal, 2010
Background Agents for prophylaxis of hereditary angioedema (HAE) have been available in the United States for several decades, but their usefulness is limited by side effects and they cannot be used at all in some patients.
Michael M Frank, MD
doaj   +1 more source

Acquired Form of Angioedema of the Head and Neck Related to a Deficiency in C1-Inhibitor: A Case Report with a Review of the Literature

Case Reports in Otolaryngology, 2012
Angioedema related to a deficiency in the C1-inhibitor protein is characterized by its lack of response to therapies including antihistamine, steroids, and epinephrine. In the case of laryngeal edema, mortality rate is approximately 30 percent. The first
Bassel Hallak, Propser Konu, Florian Lang, Christian Simon, Philippe Monnier   +4 more
doaj   +1 more source

Long-term Prophylaxis with Androgens in the management of Hereditary Angioedema (HAE) in emerging countries

Orphanet Journal of Rare Diseases, 2022
Hereditary angioedema (HAE) is a rare autosomal dominant genetic disease characterized by repetitive subcutaneous or submucosal angioedema, activation of the kinin system, and increased vascular permeability.
Yinshi Guo, Huanping Zhang, He Lai, Huiying Wang, Herberto J. Chong-Neto, Solange O. R. Valle, Rongfei Zhu   +6 more
doaj   +1 more source