Results 11 to 20 of about 26,472 (244)
Orphanet Journal of Rare Diseases, 2021 Background Hereditary angioedema associated to C1 inhibitor deficiency (C1-INH-HAE) is a pathological condition characterized by episodes of subcutaneous swelling and it is frequently associated with discomfort and social impairment of the patients, due ...
Livia Savarese +9 more
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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 +5 more
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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 +2 more
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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
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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
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Abstracts of 11th C1-inhibitor Deficiency & Angioedema Workshop [PDF]
Allergy, Asthma & Clinical Immunology, 2019 doaj +2 more sources
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 +4 more
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Allergy, Asthma & Clinical Immunology, 2020 In this letter to the editor, we present results of claims data analysis. This claims data analysis supports a hypothesis that in patients with hereditary angioedema due to C1-esterase inhibitor (C1-INH) deficiency, the occurrence and/or symptomatology ...
Henriette Farkas +5 more
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Pediatric hereditary angioedema: an update [version 1; referees: 2 approved]
F1000Research, 2017 Hereditary angioedema (HAE) with C1-inhibitor (C1-Inh) deficiency (C1-Inh-HAE) is a rare, life-threatening, and disabling genetic disorder characterized by self-limited tissue swelling caused by deficiency or dysfunction of C1-Inh. Our aim in this update
Geetika Sabharwal, Timothy Craig
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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 +6 more
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