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Discrepancies between guidelines and international practice in treatment of hereditary angioedema

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Hereditary angioedema (HAE) is an autosomal dominant disorder characterized by decreased expression or loss of function of C1 esterase inhibitor (C1-INH). In 2010, international guidelines were published regarding the management of both acute HAE attacks and prophylactic treatment. Additionally, several clinical trials for HAE therapies were published in 2010. The purpose of this study was to assess the adherence of internationally based physicians to the current evidence-based studies and the 2010 International Consensus Algorithm. Internationally based physician members of the World Allergy Organization were surveyed regarding their diagnosis and management of patients with HAE. Only physicians who treat HAE patients were included in the analyses. Of the 201 responding physicians, the most highly used therapies for acute HAE attacks were C1-INH (59%), fresh frozen plasma (FFP; 42%), and icatibant (32%). For their preferred long-term prophylactic therapy, 74% used attenuated androgens and 18% used antifibrinolytics. Physicians in Latin and South America, in particular, were less likely than their international counterparts to prescribe C1-INH and more likely to prescribe attenuated androgens and FFP, and European physicians were the most likely to prescribe icatibant. Over one-third of physicians described themselves as “unfamiliar” with emerging HAE therapies. Many international physicians neither follow current evidence-based studies nor adhere to the 2010 International Consensus Algorithm for treating HAE. Further education of physicians, government authorities, and professional medical groups is necessary to enhance availability to therapies and appropriate use of them.
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Keywords: C1 esterase inhibitor; HAE; ecallantide; guidelines; hereditary angioedema; icatibant; prophylaxis; treatment

Document Type: Research Article

Publication date: May 1, 2012

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  • Allergy and Asthma Proceedings is a peer reviewed publication dedicated to distributing timely scientific research regarding advancements in the knowledge and practice of allergy, asthma and immunology. Its primary readership consists of allergists and pulmonologists.

    The goal of the Proceedings is to publish articles with a predominantly clinical focus which directly impact quality of care for patients with allergic disease and asthma and by having the potential to directly impact the quality of patient care. AAP welcomes the submission of original works including peer-reviewed original research and clinical trial results. Additionally, as the official journal of the Eastern Allergy Conference (EAC), AAP will publish content from EAC poster sessions as well as review articles derived from EAC lectures.

    Featured topics include asthma, rhinitis, sinusitis, food allergies, allergic skin diseases, diagnostic techniques, allergens, and treatment modalities. Published material includes peer-reviewed original research, clinical trials and review articles.

    Articles marked "F" offer free full text for personal noncommercial use only.

    The journal is indexed in Thomson Reuters Web of Science and Science Citation Index Expanded, plus the National Library of Medicine's PubMed service.
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