Search results for "hereditary angioedema"
showing 10 items of 113 documents
Acquired and Hereditary Angioedema: Pathogenesis and Therapy
1988
There are two main pathogenetic ways by which angioedema can develop. These pathways are completely different and lead to completely different diseases, which may have angioedema of the skin in common. Most cases of cutaneous angioedema develop with involvement of the mast cell and its mediators; especially histamine is considered to play a major role. The exact pathogenesis, however, is not known. This type of angioedema is assumed to be related to urticaria for several reasons: 1 Often it appears alternately with urticaria 2 It responds to antihistaminic drugs 3 The same causes may provoke either urticaria or angioedema
Genotype‐phenotype correlations in Brazilian patients with hereditary angioedema due to C1 inhibitor deficiency. [Carta]
2019
Triggers of Exacerbation in Chronic Urticaria and Recurrent Angioedema—Prevalence and Relevance
2021
Patients with urticaria and angioedema often have triggers that cause an outbreak or a swelling episode or worsen their chronic condition. Exploring these factors with each patient may result in better understanding and control of their disease. Patients should be advised to avoid known triggers, if feasible, or prepare to prevent or control an exacerbation with appropriate pretreatment if avoidance is not possible. In this review, we describe and discuss a variety of factors for which there is evidence that they cause or exacerbate chronic spontaneous urticaria and angioedema. These potentially exacerbating factors include drugs, food additives, and naturally occurring pseudoallergens, men…
International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by …
2012
Background There are a limited number of publications on the management of gynecologic/obstetric events in female patients with hereditary angioedema caused by C1 inhibitor deficiency (HAE-C1-INH). Objective We sought to elaborate guidelines for optimizing the management of gynecologic/obstetric events in female patients with HAE-C1-INH. Methods A roundtable discussion took place at the 6th C1 Inhibitor Deficiency Workshop (May 2009, Budapest, Hungary). A review of related literature in English was performed. Results Contraception : Estrogens should be avoided. Barrier methods, intrauterine devices, and progestins can be used. Pregnancy : Attenuated androgens are contraindicated and should …
Side Effects Of Long-Term Treatment With Danazol And Stanozolol In Hereditary Angioedema
2007
Long-term efficacy of danazol treatment in hereditary angioedema
2010
Background No systematic study has been published yet on the long-term efficacy of attenuated androgens in hereditary angioedema (HAE). Our aim was to conduct a follow-up study in two (German and Hungarian) cohorts of HAE patients (45 and 39 patients, respectively) undergoing uninterrupted treatment for 6 years with similar (starting dose 128 ± 78 mg per day and 136 ± 70 mg per day, respectively) and constant doses of danazol. Design The frequencies of subcutaneous, abdominal and laryngeal attacks were recorded each year. Results The annual frequency of all the three types of attacks was significantly lower during the first year of danazol treatment, compared to the last year before baselin…
An evidence based therapeutic approach to hereditary and acquired angioedema
2014
Purpose of review Hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency (HAE-C1-INH), HAE with normal C1-INH, and acquired angioedema due to C1-INH deficiency are rare but important diseases that can be associated with significant morbidity and mortality. Research into the pathogenesis of angioedema has expanded greatly and has led to new clinical trials with novel therapeutic agents and strategies. Recent findings Strategies for managing HAE-C1-INH are aimed at treating acute attacks or preventing attacks through the use of prophylactic treatment. Agents available in Europe for treating acute attacks include plasma-derived C1-INH concentrates, a bradykinin B2 recepto…
A single nucleotide deletion at the C1 inhibitor gene as the cause of hereditary angioedema: insights from a Brazilian family
2011
To cite this article: Ferraro MF, Moreno AS, Castelli EC, Donadi EA, Palma MS, Arcuri HA, Lange AP, Bork K, Sarti W, Arruda LK. A single nucleotide deletion at the C1 inhibitor gene as the cause of hereditary angioedema: insights from a Brazilian family.Allergy 2011; 66: 1384–1390. Abstract Background: Hereditary angioedema is an autosomal dominant disease characterized by episodes of subcutaneous and submucosal edema. It is caused by deficiency of the C1 inhibitor protein, leading to elevated levels of bradykinin. More than 200 mutations in C1 inhibitor gene have been reported. The aim of this study was to analyze clinical features of a large family with an index case of hereditary angioe…