C1-Inh
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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Practicalities of a reduced volume formulation of a C1-INH concentrate for the treatment of hereditary angioedema: real-life experience
... with C1-INH (Berinert ® ) at the Royal London Hospital were selected to take part in this ...of C1-INH were considered for this study because of the benefits of a reduced volume in these ...
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C1-INH and the contact system in COVID-19-associated coagulopathy
... COVID-19, C1-INH could play a protective role. C1-INH replacement therapy has long been used in acute and prophylactic treatment of HAE 36 , and could be useful in the management of ...
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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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Diagnosis and treatment of hereditary angioedema with normal C1 inhibitor
... In 2001 the author of this article initiated a microsa- tellite scan of the total genom (performed by Dr. C. Hennies, Max-Delbrück Center, Berlin) in four HAE type III families which revealed major linkage signals for ...
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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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Bench to bedside review: The role of C1 esterase inhibitor in sepsis and other critical illnesses
... above, C1 INHs are currently licensed for routine prophylaxis and treatment of angioedema attacks in adolescents and ...adults. C1 INHs have also been used for the treatment of RAAS ...
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The effect of C1 esterase inhibitor on systemic inflammation in trauma patients with a femur fracture The CAESAR study: study protocol for a randomized controlled trial
... The primary outcome will be the change in serum IL-6 concentration before and after surgical repair of a femur fracture in trauma patients in the presence or absence of C1-INH. We have chosen IL-6 as our ...
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Effectiveness of icatibant for treatment of hereditary angioedema attacks is not affected by body weight: findings from the Icatibant Outcome Survey, a cohort observational study
... icatibant-treated C1-INH-HAE attacks and the use of any concomitant or rescue medications, including C1-INH, were collected via physician-completed electronic forms at routine visits ...
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Emerging concepts in the diagnosis and treatment of patients with undifferentiated angioedema
... emergency medical treatment has been IV fresh frozen plasma (FFP) and epsilon-aminocaproic acid [36]. Both anecdotal and published reports suggest that FFP replaces plasma C1-INH, thereby aborting an ...
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Diagnosis and screening of patients with hereditary angioedema in primary care
... Berinert [CSL Behring LLC, Kankakee, IL, USA] or Cinryze [ViroPharma Biologics, Inc., Exton, PA, USA]), ecallantide (trade name Kalbitor [Dyax Corporation, Burlington, MA, USA]), icatibant (trade name Firazyr [Shire ...
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Recent developments in the treatment of acute abdominal and facial attacks of hereditary angioedema: focus on human C1 esterase inhibitor
... nanofiltered C1-INH concentrate for the management of HAE are ...either C1-INH 1000 U intravenously (36 subjects) or placebo (35 ...the C1-INH group (median time 2 h) ...
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High attack frequency in patients with angioedema due to C1-inhibitor deficiency is a major determinant in switching to home therapy: a real-life observational study
... like C1-INH-HAE among the emer- gency department personnel is often insufficient resulting in the inadequate treatment of a swelling at- tack, or no treatment [4, ...home-based C1-INH treat- ...
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Molecular basis for the deficiency of complement 1 inhibitor in type I hereditary angioneurotic edema
... of C1 INH in patients' monocytes approached 50%. The study of C1 INH messenger RNA (mRNA) by Northern blot analysis indicated that in HANE patients' monocytes a message of normal size is ...
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Management of acute attacks of hereditary angioedema: role of ecallantide
... Ecallantide has been used in other forms of nonhistamin- ergic angioedema. Some forms of idiopathic angioedema are characterized by angioedema that is not responsive to antihistamines and corticosteroids, with normal ...
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Ontogeny of the Human Complement System: In Vitro Biosynthesis of Individual Complement Components by Fetal Tissues
... of C1 (C1 INH) as early as 11 wk after ...and C1 INH, that this production was temperature dependent and reversibly inhibited by well- known inhibitors of protein synthesis, and that 14 ...
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Hereditary Angioedema: Three Cases Report, Members of the Same Family
... excludes C1-INH, angioedema prevention measures include attenuated androgen administration (danazol-stanozolol), fresh frozen plasma (FFP), and antifibrogenolytic factors such as E-aminocaproic acid and ...
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Angioedema in the emergency department: a practical guide to differential diagnosis and management
... Review: The most common pathophysiology underlying angioedema is mediated by histamine; however, ED staff must be alert for the less common bradykinin-mediated forms of angioedema. Crucially, bradykinin-mediated ...
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The physician and hereditary angioedema friend or foe: 62-year diagnostic delay and iatrogenic procedures
... Angioedema of the serosa and walls of the intestines leads to thickening of the visceral walls, compression of the lumen, and clinical picture of temporary ileus lead- ing to emesis and diarrhea. Serosal extravasation ...
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Idiopathic non-histaminergic acquired angioedema: a case series and discussion of published clinical trials
... against InH AAE, the drug either interacts with IgE antibodies and influences the mast cells, as does omalizumab, or it interacts with the coagulation and contact activation pathway, as do tranexamic acid (TA), ...
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