A UK national audit of hereditary and acquired angioedema.

Hereditary angioedema (HAE) and acquired angioedema (AAE) are rare life-threatening conditions caused by deficiency of C1 inhibitor (C1INH). Both are characterized by recurrent unpredictable episodes of mucosal swelling involving three main areas: the skin, gastrointestinal tract and larynx. Swelling in the gastrointestinal tract results in abdominal pain and vomiting, while swelling in the larynx may be fatal. There are limited UK data on these patients to help improve practice and understand more clearly the burden of disease. An audit tool was designed, informed by the published UK consensus document and clinical practice, and sent to clinicians involved in the care of HAE patients through a number of national organizations. Data sets on 376 patients were received from 14 centres in England, Scotland and Wales. There were 55 deaths from HAE in 33 families, emphasizing the potentially lethal nature of this disease. These data also show that there is a significant diagnostic delay of on average 10 years for type I HAE, 18 years for type II HAE and 5 years for AAE. For HAE the average annual frequency of swellings per patient affecting the periphery was eight, abdomen 5 and airway 0·5, with wide individual variation. The impact on quality of life was rated as moderate or severe by 37% of adult patients. The audit has helped to define the burden of disease in the UK and has aided planning new treatments for UK patients.

The use of adrenaline autoinjectors by children and teenagers.

BACKGROUND: Although adrenaline is recommended as first line treatment for anaphylaxis, it is often not utilized. There has been a debate about when adrenaline autoinjectors should be prescribed and how many should be dispensed. OBJECTIVES: To see how many adrenaline autoinjectors were used during anaphylactic reactions and to determine why they were not used in situations where they were clinically indicated. METHODS: Patients were recruited prospectively at 14 paediatric allergy clinics throughout UK. Participants completed a questionnaire covering demographic data, atopic status and details of allergic reactions in the previous year and reasons for using more than one device. RESULTS: A total of 969 patients were recruited of whom 466 (48.1%, 95% CI: 37.9-58.2) had had at least one reaction in the previous year; 245 (25.3%, 95% CI: 16.2-34.4) of these reactions were anaphylaxis. An adrenaline autoinjector was used by 41 (16.7%, 95% CI: 11.7-21.3) participants experiencing anaphylaxis. Thirteen participants received more than one dose of adrenaline, for nine of these a health professional gave at least one. The commonest reasons for using more than one were severe breathing difficulties (40%), lack of improvement with first dose (20%) and miss-firing (13.3%). The commonest reasons for not using adrenaline in anaphylaxis were 'thought adrenaline unnecessary' (54.4%) and 'unsure adrenaline necessary' (19.1%). Many with wheeze did not use their autoinjector. CONCLUSIONS AND CLINICAL RELEVANCE: Adrenaline is used by only a minority of patients experiencing anaphylaxis in the community. Thirteen of the 41 patients with anaphylaxis who used their autoinjector needed another dose of adrenaline. Further research is needed to consider how to best encourage the usage of adrenaline when clinically indicated in anaphylaxis.

Staphylococci and staphylococcal superantigens in asthma and rhinitis: a systematic review and meta-analysis.

BACKGROUND: There is a need for new treatment options of allergic respiratory diseases based on a better knowledge of their pathogenesis. An association between bacterial products and allergic airway diseases has been suggested by the results of human and animal studies that describe a link between Staphylococcus aureus enterotoxins and atopic diseases. The aim of the systematic review is to assess the evidence for a role of Staphylococcus aureus enterotoxins, as an environmental risk factor, for the development and/or the severity of asthma and allergic rhinitis. METHODS: We performed a systematic review of controlled clinical studies in adults and/or children affected by asthma/early wheeze and/or allergic rhinitis. To be eligible, studies had to use reproducible methods to provide evidence of exposure to S. aureus, clinical outcome and disease severity. RESULTS: Ten studies, published between 2000 and 2007, fulfilled all eligibility criteria. Patients with asthma or allergic rhinitis showed an increased prevalence of positivity for measures of exposure to S. aureus in nine studies: differences were statistically significant (P < 0.05) in seven studies. In a meta-analysis of study results, patients with asthma were more likely than controls to have serum-specific IgE to Staphylococcus aureus enterotoxins (OR = 3.3, 95% CI: 1.6-7.1, P = 0.002); similarly, patients with allergic rhinitis were more likely than controls to test positive for local or systemic exposure to Staphylococcus aureus and/or or its enterotoxins (OR = 2.4, 95% CI: 1.3-4.7, P = 0.008). CONCLUSIONS: A potential role of S. aureus superantigens in allergic respiratory diseases is supported by results of this meta-analysis of clinical studies.