Search results for " Angioedema"

Hereditary angioedema caused by missense mutations in the factor XII gene: clinical features, trigger factors, and therapy.

2009

Background Hereditary angioedema caused by mutations in the factor XII gene is a recently described disease entity that occurs mainly in women. It differs from hereditary angioedema caused by C1 inhibitor deficiency. Objective To assess the clinical symptoms, factors triggering acute attacks, and treatments of this disease. Methods Thirty-five female patients with hereditary angioedema and the factor XII mutations p.Thr309Lys and p.Thr309Arg who came from 13 unrelated families were studied. The observation period was 8.4 years on average (range, 2-26 years). Results Patients had on average 12.7 ± 7.9 angioedema attacks per year. Recurrent facial swellings occurred in all patients; skin swel…

Hereditary angioedema in children and adolescents - A consensus update on therapeutic strategies for German-speaking countries.

2020

Background/methods At a consensus meeting in August 2018, pediatricians and dermatologists from German-speaking countries discussed the therapeutic strategy for the treatment of pediatric patients with type I and II hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) for Germany, Austria, and Switzerland, taking into account the current marketing approval status. HAE-C1-INH is a rare disease that usually presents during childhood or adolescence with intermittent episodes of potentially life-threatening angioedema. Diagnosis as early as possible and an optimal management of the disease are important to avoid ineffective therapies and to properly treat swelling attacks. This art…

Uncommon Signs Associated With Hereditary Angioedema With Normal C1 Inhibitor.

2021

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…