Results 21 to 30 of about 1,197,187 (269)
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
doaj +1 more source
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
doaj +1 more source
Abstracts of 11th C1-inhibitor Deficiency & Angioedema Workshop [PDF]
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Pediatric hereditary angioedema: an update [version 1; referees: 2 approved]
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
doaj +1 more source
Pediatric hereditary angioedema due to C1-inhibitor deficiency
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
Risk of angioedema following invasive or surgical procedures in HAE type I and II : the natural history [PDF]
Background: Hereditary angioedema (HAE), caused by deficiency in C1-inhibitor (C1-INH), leads to unpredictable edema of subcutaneous tissues with potentially fatal complications.
Aygören-Pürsün, Emel +4 more
core +1 more source
C1 inhibitor deficiency: consensus document
We present a consensus document on the diagnosis and management of C1 inhibitor deficiency, a syndrome characterized clinically by recurrent episodes of angio-oedema.
Morrison L +11 more
core +5 more sources
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
The deficiency of C1 inhibitor and its treatment
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
Effect of mutations within the coding region of C1-INH gene on protein function in families with HAE
C1 esterase inhibitor (C1-INH) is a serine protease inhibitor (serpin). Serpins are a superfamily of proteins that fold into a conserved structure and utilize a unique suicide substrate-like inhibitory mechanism.
M. Cicardi +3 more
core +2 more sources

