How physicians approach hereditary angioedema: a single center study.

Background: Hereditary angioedema (HAE) is a rare autosomal dominant disorder caused by C1-inhibitor deficiency. It is characterized by recurrent attacks of cutaneous and upper respiratory tract swelling, and abdominal pain due to mucosal edema. Early detection and treatment prevent unnecessary interventions, improves quality of life, and prevents potentially fatal attacks. Objective: The present study aims to investigate physicians level of knowledge and awareness regarding HAE. Methods: A questionnaire about HAE was applied to 393 physicians from a university hospital. Participants were requested to choose one or several answers to multiple-choice questions. Results: Seven and three tenths percent of study participants stated to have never heard of HAE. Twenty-seven physicians (7.4%) chose the exact correct answers regarding diagnostic tests, and 2 (0.8%) chose the exact correct answers regarding emergency management. A composite of internists, pediatrists and emergency medicine specialists had a significantly higher mean score than other physicians (p = 0.047). Physicians from internal medical sciences scored significantly higher than physicians from surgical medical sciences (p = 0.022). Conclusion: The present study reveals that physician awareness about HAE is low, and physicians misdiagnose HAE attacks as histaminergic angioedema attacks, and therefore provide ineffective treatment. Although HAE is a rare disease, physician awareness must be increased, because early diagnosis and effective treatment are vital for the patients.

Tolerability to paracetamol and preferential COX-2 inhibitors in patients with cross-reactive nonsteroidal anti-inflammatory drugs hypersensitivity.

BACKGROUND: Acetylsalicylic acid/aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs) are widely used drugs that may cause hypersensitivity reactions in a substantial proportion of patients. Physicians ought to be aware of these situations. OBJECTIVE: We aimed to present the clinical characteristics and rates of tolerability to cyclooxygenase (COX)-2 inhibitor analgesics in patients who had admitted due to multiple cross-reactive type of NSAID hypersensitivity. METHODS: The files of the patients who had admitted with multiple NSAIDs-induced symptoms were investigated retrospectively. Age, sex, underlying diseases, clinical manifestation, skin test results, and drug provocation test results were analyzed. RESULTS: In 105 patients with multiple cross-reactive type of NSAID hypersensitivity, we found the rate of cross-reactivity to any of the relatively safe alternatives including paracetamol, meloxicam, and nimesulide to be 16.1%. The rate of cross-reactivity to these relatively safe drugs was significantly higher in patients with a history of anaphylaxis induced by NSAID intake (p = 0.006). CONCLUSION: The diagnosis of COX-1-mediated multiple NSAID hypersensitivity can be often established with a detailed history. Although rare, severe hypersensitivity reactions may be observed in these patients. Undesired situations for both patients and physicians may be avoided by testing relatively safe paracetamol and COX-2 inhibitors in experienced centers.