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Glucocorticoid receptor gene polymorphisms in hereditary angioedema with C1-inhibitor deficiency
... 36 C1-INH-HAE ...Nucleotide Polymorphisms) were assessed using ANOVA. C1-INH-HAE patients have decreased coping capabilities compared to healthy ...the C1-INH-HAE patients, the carriers of the ... See full document
436
Emotional processes and stress in children affected by hereditary angioedema with C1-inhibitor deficiency: a multicenter, prospective study
... Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is a rare autosomal dominant disorder with a reported prevalence of approximately 1 case per 50,000 persons [1, ... See full document
8
A nationwide survey of hereditary angioedema due to C1 inhibitor deficiency in Italy
... Hereditary angioedema (HAE) due to C1 Inhibitor (C1-INH) deficiency (C1-INH-HAE) is a rare auto- somal dominant disease due to reduced C1-INH plasma levels ... See full document
7
The diagnosis of hereditary angioedema with C1 inhibitor deficiency: a survey of Canadian physicians and laboratories
... of C1-INH function using contact-phase proteases as target, assessment of kinin formation during attacks through spontaneous kininogenase (amidase) activity, activation of kinin-forming zymogens, and HMWK plasma ... See full document
50
Management of Children With Hereditary Angioedema Due to C1 Inhibitor Deficiency
... 2 receptor antagonist currently approved for treatment of HAE attacks in patients >18 years of ...of angioedema, patients with HAE may need short-term prophylactic treatment to reduce the likelihood of ... See full document
5
The influence of trigger factors on hereditary angioedema due to C1-inhibitor deficiency
... Hereditary angioedema (HAE) due to C1-INH deficiency (HAE-C1-INH) is a rare autosomal dominant ...The deficiency of C1-INH may cause activation of four closely ... See full document
94
Comprehensive study into the activation of the plasma enzyme systems during attacks of hereditary angioedema due to C1-inhibitor deficiency
... Thirty-nine C1-INH-HAE patients (12 men and 27 women, median age: 35 years, 25 to 75 th percentiles: 22– 50 years), 33 with type I, and 6 with type II of C1-INH- HAE, were enrolled into our ...of ... See full document
9
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
... The approach employed in this paper for the analysis of SERPING1 epigenetics is evidently hindered by the impos- sibility of obtaining hepatocyte samples from patients and controls in which to perform re-expression ... See full document
7
Abdominal attacks and treatment in hereditary angioedema with C1-inhibitor deficiency
... a deficiency in C1- inhibitor (HAE-C1INH), although HAE with normal C1-INH (HAEnC1) has been described ...[6]. C1-INH is a serine-protease inhibitor that regulates the activation ... See full document
12
Hereditary angioedema due to C1 - inhibitor deficiency in Switzerland: clinical characteristics and therapeutic modalities within a cohort study
... trauma and physical exertions are the most relevant trig- ger factors for both genders, followed by food and infec- tions. Foodstuff as a trigger factor is also described by Zotter et al. If it is intolerance or allergy ... See full document
51
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 ...of angioedema involving the skin and/or the mucosa of ... See full document
88
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, ... See full document
71
Ecallantide is a novel treatment for attacks of hereditary angioedema due to C1 inhibitor deficiency
... Angioedema attacks caused by C1-INH deficiency are medi- ated by the excessive release of vasoactive bradykinin. This release is initiated by the uncontrolled activation of factor XII, a protein that ... See full document
18
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
... of angioedema without urticaria, four types of acquired (AAE) and three types of hereditary angioedema (HAE) were identified as separate forms ...acquired angioedema without wheals may be: ... See full document
116
Frequency of the virilising effects of attenuated androgens reported by women with hereditary angioedema
... Hereditary angioedema due 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 ... See full document
35
Abstracts of 11th C1-inhibitor Deficiency & Angioedema Workshop
... for hereditary angioedema (HAE), asking for oral treatment to replace the burden of current ...B2 receptor, for oral on-demand treatment of acute HAE attacks and for prophylactic preven- tion of ... See full document
12
Abstracts from the 10th C1-inhibitor deficiency workshop
... acquired angioedema (AAE) or late-onset hereditary angioedema (HAE) with spontaneous ...(DPP-4) inhibitor in some oral antidiabetics is excluded, a blood test for C4, C1-esterase ... See full document
12
Management of hereditary angioedema in pregnant women: a review
... An in-depth search of the available English language literature was performed on PubMed and EMBASE using the following key words: hereditary angioedema, HAE, type III HAE, C1-inhibitor, and ... See full document
7
Diagnosis and treatment of hereditary angioedema with normal C1 inhibitor
... and hereditary angioedema (HAE) share this clinical ...II) deficiency of C1 esterase inhibitor (C1-INH) caused by mutations of the C1-INH ...genetic deficiency of ... See full document
100
Management of upper airway edema caused by hereditary angioedema
... Hereditary angioedema (HAE) is a rare disorder of auto- somal dominant ...the gene encoding the C1-inhibitor (C1-INH) or the factor XII protein (FXII) ...the C1-INH ... See full document
5
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