C1-INH-HAE
Comprehensive study into the activation of the plasma enzyme systems during attacks of hereditary angioedema due to C1-inhibitor deficiency
... the C1-inhibitor – the key regulator in C1- INH-HAE – correlated with aPTT, the activity of factor XI, and with fibrinogen ...Interestingly, C1- INH activity showed no ...
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Glucocorticoid receptor gene polymorphisms in hereditary angioedema with C1-inhibitor deficiency
... by C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant ...disorder. C1-INH-HAE is characterized by edema – formation, which may occur in response to ...of ...
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Frequency of the virilising effects of attenuated androgens reported by women with hereditary angioedema
... plasma-derived C1-INH), the use of attenuated androgens in women with HAE raises a number of concerns ...increase C1-INH level by stimulating hep- atic synthesis, as well as by ...
5
The physician and hereditary angioedema friend or foe: 62-year diagnostic delay and iatrogenic procedures
... in C1-INH-HAE ...and C1-INH concentrate. C1-INH (plasma-derived and recombinant forms) and bradykinin antagonists are now the standard of care for acute attacks of ...
6
Serum fetuin-A, tumor necrosis factor alpha and C-reactive protein concentrations in patients with hereditary angioedema with C1-inhibitor deficiency
... Peripheral blood samples were obtained from patients with C1-INH-HAE both during symptom-free periods and during attacks (before acute treatment). “Symptom-- free” samples were obtained during the ...
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Emotional processes and stress in children affected by hereditary angioedema with C1-inhibitor deficiency: a multicenter, prospective study
... with C1-inhibitor deficiency (C1-INH-HAE) is characterized by recurrent edema of unpredictable frequency and ...with C1-INH-HAE, to determine whether they are also present ...
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A nationwide survey of hereditary angioedema due to C1 inhibitor deficiency in Italy
... Comparing C1-INH-HAE subjects and general Italian population for distribution in different decades of life, patients are less represented in the early and late de- ...in ...
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Elderly versus younger patients with hereditary angioedema type I/II: patient characteristics and safety analysis from the Icatibant Outcome Survey
... However, C1-INH-HAE attacks may occur at any age and recur throughout a patient’s lifetime [7, ...managing C1-INH-HAE attacks in elderly patients are ...plasma-derived ...
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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 (C1-INH). HAE has traditionally been divided into two ...system, HAE Types I and II are inherited as an autosomal dominant ...an HAE attack is caused by ...
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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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Neutrophil activation during attacks in patients with hereditary angioedema due to C1-inhibitor deficiency
... of C1-INH-HAE WBC count, NGC, Red blood cell (RBC) count and hematocrit were determined in the samples using Advia 120 Hematology System automate (Siemens, Erlangen, ...
8
The effect of long-term danazol treatment on haematological parameters in hereditary angioedema
... plasma-derived C1-INH concentrate, and progestins are available for prophylaxis ...In C1-INH-HAE, the earliest and the most extensive experience is available with attenuated androgens ...
7
C1-INH and the contact system in COVID-19-associated coagulopathy
... with C1-INH suggests that this essential regulator of the contact system is inhibited during viral infection, leading to a propensity to activate the complement cascade, the bradykinin pathway and the ...
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Emerging concepts in the diagnosis and treatment of patients with undifferentiated angioedema
... ACEI-induced angioedema is associated with the re- duction in bradykinin degradation that is caused by ACEIs. As in HAE, increased levels of bradykinin lead to the symptoms of swelling, pain, and inflammation that ...
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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 diagnostic ...
5
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 massive ...
10
Practicalities of a reduced volume formulation of a C1-INH concentrate for the treatment of hereditary angioedema: real-life experience
... sought. Efforts to improve patients’ quality of life have included self-administered C1-INH. Implementation and acceptance of self-administration has been widespread amongst physicians, nurses and patients ...
7
Management of upper airway edema caused by hereditary angioedema
... minimal side effects.. The viral safety of preparations manufactured using innovative pasteurization and nano- filtration technologies is excellent [30]. Treatment with pdC1-INH concentrate does not lead to the ...
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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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Management of Hereditary Angioedema in Pediatric Patients
... of HAE. Once diagnosed, HAE is associated with further difficulties during its ...care. C1-inhibitor concentrate is not available everywhere for the treatment of acute, life-threatening ...of ...
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