C1 inhibitor (C1-INH)
Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate
... to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is a serious condition that may result in life-threatening asphyxiation due to laryngeal ...plasma-derived C1-INH ...
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Expression of the SERPING1 gene is not regulated by promoter hypermethylation in peripheral blood mononuclear cells from patients with hereditary angioedema due to C1-inhibitor deficiency
... to C1-Inhibitor (C1-INH) deficiency display a dominant-negative effect usually resulting in protein levels far below the expected ...on C1-INH ...with C1-INH mRNA ...
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Cinryze™ as the first approved C1 inhibitor in the USA for the treatment of hereditary angioedema: approval, efficacy and safety
... of C1 esterase inhibitor ...of C1-inhibitor manifested as subcutaneous or submucosal edema of the upper airway, face, extremities, or gastrointestinal ...of C1-INH for the ...
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Plasma levels of C1 inhibitor complexes and cleaved C1 inhibitor in patients with hereditary angioneurotic edema
... C1- inhibitor (C1(-)-Inh) catabolism in plasma of patients with hereditary angioneurotic edema (HANE) was assessed by measuring the complexes formed by C1(-)-Inh with its target ...
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Diagnosis and treatment of hereditary angioedema with normal C1 inhibitor
... of C1 esterase inhibitor (C1-INH) caused by mutations of the C1-INH ...normal C1-INH concentration and activity with respect to C1 esterase inhibition, ...
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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
... when C1 inhibitor esterase (C1-INH) deficiency was ...negative. C1-INH and complement fraction C4 plasma levels were significantly decreased at all measurements, but no ...
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Proteolytic inactivation of plasma C1 inhibitor in sepsis
... Because C1 inhibitor (C1-Inh) regulates the activation of both cascade systems, we studied the characteristics of plasma C1-Inh in 48 patients with severe sepsis on admission to ...
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Demonstration of modified inactive first component of complement (C1) inhibitor in the plasmas of C1 inhibitor deficient patients
... (C1) inhibitor plays a critical role in the regulation of the classical complement pathway and the contact system, and the deficiency of C1 inhibitor protein or function is associated with ...
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Platelet C1 inhibitor A secreted alpha granule protein
... of C1- inhibitor activity in 10(8) ...platelet C1- inhibitor antigen was immunochemically identical to plasma and purified C1- ...platelet C1- inhibitor antigen in ...
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Acquired C1 inhibitor (C1 INH) deficiency type II Replacement therapy with C1 INH and analysis of patients' C1 INH and anti C1 INH autoantibodies
... 96,000 C1-INH, it is concluded that this fragment is generated after cleavage of C1-INH in its active site by one of its target proteases without generating a covalent ...
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Autoantibody facilitated cleavage of C1 inhibitor in autoimmune angioedema
... normal C1-Inh, but show that this protein circulates in the patient's plasma in an inactive, structurally altered ...normal C1-Inh, by its target proteases, to the same species of ...
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Neutrophil activation during attacks in patients with hereditary angioedema due to C1-inhibitor deficiency
... compared with those from symptom-free periods. Re- markably, TNF-α levels were lower in the samples of symptom-free patients than in those from healthy con- trols. Although we confirmed this finding by extending the ...
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Abstracts of 11th C1-inhibitor Deficiency & Angioedema Workshop
... methods: C1-INH deficiency was diagnosed in 228 patients from 200 families in the Complement Laboratory, Hôpital Georges Pompidou, Paris, between 1995 and ...
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The International/Canadian Hereditary Angioedema Guideline
... deficit C1-inhibitor levels (HAE-1), HAE with dysfunctional C1-inhibitor (HAE-2), and HAE with normal C1-inhibitor function (HAE nC1-INH) previously referred to as type 3 ...
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The complement system in glioblastoma multiforme
... [43]. C1 inhibitor (C1 INH) inhibits the bradykinin-forming cascade at several levels: its local ab- sence at the glioma invasive edge may further initiate the classic complement cascade by ...
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C1 Inhibitor Gene Sequence Facilitates Frameshift Mutations
... Because this is such a rare event, as judged by the reversion rate, we were unable to identify successive repeats where the inverted repeat caused cycled tandem duplicati[r] ...
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Hereditary angioedema: New therapeutic options for a potentially deadly disorder
... and C1 inhibitor are the only medications approved in the United States for ...and C1 INH are used for ...increase C1 inhibitor via the liver and carry the risk of long-term side ...
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Allergic diseases in the elderly
... the C1 inhibitor (HAE-C1-INH) has its onset in the ...acquired C1 inhibitor deficiency (AAE-C1-INH) presents in the older age [59], and it is characterized by ...
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Mechanism of Action of Anti-C1-Inhibitor Autoantibodies: Prevention of the Formation of Stable C1s-C1-inh Complexes
... During a 6-hr incubation at 37°C, the presence of a 10-fold molar excess of autoantibody reactive sites over Cl-inh did not result in dissociation of the Cls-Cl-inh complex (data not sho[r] ...
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Frequency of the virilising effects of attenuated androgens reported by women with hereditary angioedema
... to C1 inhibitor deficiency (C1-INH-HAE) is an autosomal dominant disorder; a form of bradykinin-mediated ...with C1-INH-HAE are more likely to experience symptoms than are men ...
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