Results 11 to 20 of about 1,197,187 (269)

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
core   +6 more sources

A CASE OF ANGIOEDEMA: C1 INHIBITOR DEFICIENCY

National Journal of Medical Research, 2015
Angioedema is rapid swelling (oedema) of subcutaneous tissue involving dermis, mucosa and sub mucosal tissues. It may be IgE dependant, bradykinin mediated, complement mediated, non immunologic or idiopathic. It may be heriditory or acquired.
Arijit Sinha, Suvrendu Sankar Kar, Subhrojyoti Mitra, Ujjal Bandopadhyayay Bandopadhyay, Bikas Chandra Seth, Md Hamid Ali   +5 more
doaj   +1 more source

Monocyte C1-Inhibitor Synthesis [PDF]

, 1987
C1-inhibitor is the major control protein of the classical pathway of complement. In man, its hereditary deficiency results in the clinical condition of hereditary angio-oedema (HAE).
Jones, Linda Margaret
core   +6 more sources

Hereditary angioedema (HAE) in children and adolescents : a consensus on therapeutic strategies [PDF]

, 2012
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 ...
Martinez-Saguer, I, Wahn, V, V. Wahn, I. Martinez-Saguer, Magerl, Markus, Eberl, W, Meyer-Olson, D, Kurnik, K, P. Staubach-Renz, Staubach-Renz, Petra, Eberl, Wolfgang, Kreuz, Wolfhart, Faßhauer, M, K. Kurnik, Magerl, M, D. Meyer-Olson, T. Kühne, W. Aberer, Staubach-Renz, P, Späth, Peter J., Aberer, W, Späth, P, Kreuz, W, Kurnik, Karin, W. Eberl, Wahn, Volker, P. Späth, Aberer, Werner, Meyer-Olson, Dirk, Martinez Saguer, Inmaculada, M. Faßhauer, W. Kreuz, Faßhauer, Maria, M. Magerl, Kühne, T, Kühne, Thomas   +35 more
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HAE international home therapy consensus document [PDF]

, 2010
Hereditary angioedema (C1 inhibitor deficiency, HAE) is associated with intermittent swellings which are disabling and may be fatal. Effective treatments are available and these are most useful when given early in the course of the swelling.
Bowen, Tom, Craig, Timothy, Boysen, Henrik, E. Aygören Pürsün, Janet Long, Zuraw Bruce, Avner Reshef, Longhurst, Hilary J., Cicardi Marco, Bygum, Anette, Hilary J Longhurst, Bruce Zuraw, Anette Bygum, Martinez-Saguer, Inmaculada, Bork Konrad, Inmaculada Martinez-Saguer, Sofia Grigoriadou, Caballero, Teresa, Claire Bethune, I. Martinez Saguer, Reshef Avner, Martinez Saguer, Inmaculada, M. Raguet, H. J. Longhurst, J. Bjorkander, Laurence Bouillet, Levi Marcel M, Raguet Michel, Timothy Craig, Bethune Claire, T. Bowen, Michel Raguet, L. Bouillet, Marco Cicardi, Gompels Mark, Bork, Konrad, C. Bethune, Dempster, John, Huffer Ursula, Marcel M Levi, Longhurst, Hilary, Aygören-Pürsün Emel, M. Cicardi, Farkas Henriette, Henriette Farkas, Raguet, Michel, Bygum Anette, Bjorkander Janne, Levi, Marcel M, Longhurst Hilary J, Martinez-Saguer Inmaculada, Teresa Caballero, Ursula Huffer, Bouillet, Laurence, H. Farkas, Boysen Henrik, Huffer, Ursula, M. M. Levi, M. Gompels, Gompels, Mark, S. Grigoriadou, Kreuz, Wolfhart, Janne Bjorkander, A. Bygum, Craig Timothy, Longhurst, Hilary J, Farkas, Henriette, Bygum, Anette; id_orcid, Bowen Tom, Gooi Jimmy, Bouillet Laurence, Boysen, Henrik B., A. Reshef, J. Gooi, J. Dempster, Konrad Bork, Henrik Boysen, Gooi, Jimmy, Jimmy Gooi, Cicardi, Marco, T. Craig, B. Zuraw, Caballero Teresa, Bethune, Claire, U. Huffer, Levi, Marcel M., Dempster John, John Dempster, Grigoriadou Sofia, Reshef, Avner, Long Janet, H. Boysen, Grigoriadou, Sofia, Bjorkander, Janne, T. Caballero, Tom Bowen, Zuraw, Bruce, J. Long, W. Kreuz, Mark Gompels, Emel Aygören-Pürsün, Aygören-Pürsün, Emel, K. Bork, Kreuz Wolfhart, Long, Janet, Wolfhart Kreuz   +105 more
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2010 international consensus algorithm for the diagnosis, therapy and management of hereditary angioedema [PDF]

, 2010
Background We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) and updated this as Hereditary angioedema: a current state-of-the-art ...
S. Waserman, Harmat, George, E. Leith, Bernstein Jonathan, Bowen, Tom, Leith, Eric, Doris Neurath, Craig, Timothy, Paul K Keith, Eric Leith, A. Hamed, D. Moldovan, Zuraw Bruce, Betschel Stephen, Avner Reshef, Dean, John, Cicardi Marco, Moldovan, Dumitru, Lilian Varga, Hilary J Longhurst, Rowe Anne, Nordenfelt Patrik, Barbara Mako, Harmat George, B. Ritchie, Martinez-Saguer, Inmaculada, Fust George, Della Cogar, Inmaculada Martinez-Saguer, George Fust, Devaraj, Ramachand, Mike Frank, Richard Warrington, J. Bernstein, Rowan, Dereth, Nielsen Erik W, Li, Henry, Tsai Ellie, D. Cogar, Timothy Craig, Zhi Xiang, Poon Man-Chiu, J. Brosz, E. Tsai, Marco Cicardi, R. Schellenberg, R. Warrington, Sur, Smeeksha, B. Mako, I. Nagy, Bork, Konrad, Frank Mike, George Harmat, Frank, Mike, Karen Binkley, H. Lyons, Schellenberg, Robert, Longhurst, Hilary, Erik W Nielsen, L. Varga, Farkas Henriette, Henriette Farkas, Mako, Barbara, Devaraj Ramachand, Longhurst Hilary J, G. E. Rivard, Rusicke, Eva, Hamed, Azza, Kreuz, Wolfhart, Eva Rusicke, Poon, Man-Chiu, Späth, Peter, Binkley Karen, Anne Rowe, Craig Timothy, Istvan Nagy, Neurath, Doris, Xiang, Zhi Yu, Bowen Tom, E. W. Nielsen, Lacuesta, Gina, Sean Mace, Bouillet Laurence, Nagy Istvan, Lacuesta Gina, Brosz Kristylea, Nielsen, Erik W, John Brosz, D. Rowan, D. Neurath, Peter Späth, A. Kanani, Nagy, Istvan, Ameratunga Rohan, Bruce Ritchie, Aygoeren-Pürsün Emel, Li Henry, A. Rowe, G. Lacuesta, Keith Paul K, B. Zuraw, T. Craig, Ritchie, Bruce, Caballero Teresa, Cogar Della, S. Mace, M. Frank, Dumitru Moldovan, S. Betsche, Mace Sean, M. Poon, Zhi Yu Xiang, Lyons, Harriet, Waserman Susan, J. Dean, T. Caballero, Xiang Zhi, Tom Bowen, Rohan Ameratunga, Gina Lacuesta, M. Levi, Smith-Foltz Sarah, S. Sur, Brosz, John, Björkander, Janne, Aygören-Pürsün, Emel, Warrington Richard, Aygoeren-Pürsün, Emel, Susan Waserman, Binkley, Karen, Waserman, Susan, Ritchie Bruce, Donald Stark, Keith, Paul K, Xiang, Zhi Y, Bygum, Anette, Bruce Zuraw, Anette Bygum, H. Li, R. Ameratunga, Bork Konrad, Robert Schellenberg, Rivard Georges-Etienne, Sarah Smith-Foltz, Caballero, Teresa, I. Martinez Saguer, Reshef Avner, Martinez Saguer, Inmaculada, H. J. Longhurst, Varga Lilian, J. Björkander, Laurence Bouillet, Palinder Kamra, Leith Eric, Betschel, Stephen, Bernstein, Jonathan, Ellie Tsai, T. Bowen, K. Brosz, Ramachand Devaraj, J. Hebert, Rowe, Anne, L. Bouillet, Kristylea Brosz, Dereth Rowan, Marcel Levi, Neurath Doris, P. Späth, Kanani, Amin, G. Harmat, M. Cicardi, Kanani Amin, Bygum Anette, Stephen Betschel, Sur Smeeksha, Martinez-Saguer Inmaculada, G. Fust, Nordenfelt, Patrik, Teresa Caballero, Bouillet, Laurence, Fust, George, H. Farkas, Jonathan Bernstein, Levi, Marcel, Jacques Hebert, Keith, Paul K., Stark, Donald, P. Nordenfelt, Hebert, Jacques, Xiang, Zhi, Warrington, Richard, D. Stark, Harriet Lyons, A. Bygum, Longhurst, Hilary J, Stark Donald, Henry Li, Rowan Dereth, Farkas, Henriette, Lyons Harriet, Bygum, Anette; id_orcid, Levi Marcel, Kamra, Palinder, Dean John, Ameratunga, Rohan, E. Aygoeren Pürsün, A. Reshef, Brosz, Kristylea, Janne Björkander, R. Devaraj, Konrad Bork, P. K. Keith, Björkander Janne, Kamra Palinder, Bernstein, Jonathan A., Späth Peter, Azza Hamed, Schellenberg Robert, P. Kamra, Cicardi, Marco, Amin Kanani, Mace, Sean, Smeeksha Sur, Levi, Marcel M., Y. X. Zhi, Varga, Lilian, Reshef, Avner, S. Smith Foltz, Georges-Etienne Rivard, Rusicke Eva, Rivard, Georges-Etienne, Smith-Foltz, Sarah, K. Binkley, Yu Xiang, Zhi, Emel Aygoeren-Pürsün, Cogar, Della, Hebert Jacques, Patrik Nordenfelt, E. Rusicke, Brosz John, John Dean, Mako Barbara, Zuraw, Bruce, Moldovan Dumitru, W. Kreuz, K. Bork, Tsai, Ellie, Kreuz Wolfhart, Man-Chiu Poon, Wolfhart Kreuz, Nielsen, Erik W., Hamed Azza   +251 more
core   +1 more source

Nine year follow-up of a rare case of angioedema due to acquired C1-inhibitor deficiency with late onset and good response to attenuated androgen

Allergy, Asthma & Clinical Immunology, 2018
Background Angioedema due to acquired deficiency of C1-inhibitor (C1-INH-AAE) is a rare disease sharing some clinical and laboratory similarities with hereditary angioedema, but with late onset and no positive family history. The underlining cause may be
Polliana Mihaela Leru, Vlad Florin Anton, Horia Bumbea   +2 more
doaj   +1 more source

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

C1 Inhibitor Deficiency and Angioedema of the Small Intestine Masquerading as Crohn’s Disease

Canadian Journal of Gastroenterology, 2000
A case of C1 inhibitor deficiency presenting as localized edema of the small intestine is described. A 16-year-old, previously healthy woman presented with recurrent attacks of abdominal pain and vomiting following minor abdominal trauma.
Kelly W Burak, Gary R May
doaj   +1 more source

Clinical profile of patients with C1-inhibitor deficiency from Eastern India

Indian Journal of Allergy Asthma and Immunology, 2016
C1-inhibtor deficiency or hereditary angioedema is a rare, autosomal dominant disorder that is characterized by severe episodic attacks of angioedema that can affect any part of the body.
Sujoy Khan
doaj   +1 more source