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Free Content Hereditary angioedema: Epidemiology and burden of disease

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<br></br>Hereditary angioedema (HAE) is a rare autosomal dominant genetic disorder characterized by swelling of subcutaneous, mucosal, and submucosal tissue without associated pruritus or wheals caused by a temporary localized increase in vascular permeability. Swelling attacks primarily affect the cutaneous tissue, abdominal viscera, genitals, or airways. <br></br><title/> <br></br>Reports of the prevalence of HAE C1 inhibitor (C1-INH) deficiency varies widely, from 1:50,000 to 1:100,000. The prevalence of HAE normal C1-INH is unknown but is likely much lower than HAE C1-INH. Approximately one-third of patients with recurrent angioedema without wheals have HAE. <br></br><title/> <br></br>The burden of disease for patients with HAE is substantial. Attacks are unpredictable with respect to frequency, severity, and the site that swells. Laryngeal attacks can be fatal if not treated promptly and appropriately. Feelings of stress, anxiety, and depression can trigger attacks, and begin a cycle of attacks that cause anxiety that, in turn, triggers further attacks. Despite full physical recovery between attacks, patients often experience continual emotional impairment and reduced quality of life (QoL). Absenteeism from work and presenteeism at work or educational activities for patients and caregivers increase stress and reduce productivity during and between attacks. Missed opportunities for career development are common. <br></br><title/> <br></br>Significant advances have been made in the past decade to expand both acute and prophylactic treatment options for patients with HAE, lowering both the disease and treatment burden, and improving the QoL of patients with HAE. </div> <div id="Refs" class="tab-pane" role="tabpanel" aria-hidden="true" aria-labelledby="tab-refs"> <p><strong>22 References</strong>.</p> <div id="ref-hook" class="fa fa-spinner fa-spin" title="Please wait, loading reference information..."></div> <div id="ref-hook-content" class="hidden"></div> </div> <div id="Cits" class="tab-pane" role="tabpanel" aria-hidden="true" aria-labelledby="tab-cits"> <!-- No Citations for this article ?? --> <div id="cit-hook" class="fa fa-spinner fa-spin" title="Please wait, loading citation information..."></div> <div id="cit-hook-content" class="hidden"></div> </div> <div id="Supp" class="tab-pane" role="tabpanel" aria-hidden="true" aria-labelledby="tab-supp"> No Supplementary Data. </div> <div id="Data" class="tab-pane rs_skip" aria-hidden="true" aria-labelledby="tab-data">No Article Media</div> <div id="Metr" class="tab-pane rs_skip" aria-hidden="true" aria-labelledby="tab-metr">No Metrics</div> </div> <div id="Info"> <p><strong>Keywords:</strong> <a href="/search;jsessionid=2p575bklce0jr.x-ic-live-03?option1=keywords&value1='Burden+of+disease'">Burden of disease</a>; <a href="/search;jsessionid=2p575bklce0jr.x-ic-live-03?option1=keywords&value1='Cost'">Cost</a>; <a href="/search;jsessionid=2p575bklce0jr.x-ic-live-03?option1=keywords&value1='Epidemiology'">Epidemiology</a>; <a href="/search;jsessionid=2p575bklce0jr.x-ic-live-03?option1=keywords&value1='Hereditary+angioedema'">Hereditary angioedema</a>; <a href="/search;jsessionid=2p575bklce0jr.x-ic-live-03?option1=keywords&value1='Quality+of+life'">Quality of life</a> </p> <p><strong>Document Type:</strong> Research Article</p> <p><strong>Affiliations:</strong> <strong id="aff_1">1: </strong> <span id="aff_ From the University of Texas Southwestern Medical School, Dallas, Texas; _contents"> From the University of Texas Southwestern Medical School, Dallas, Texas; </span> <strong id="aff_2">2: </strong> <span id="aff_ Warren Alpert Medical School, Brown University, Providence, RI_contents"> Warren Alpert Medical School, Brown University, Providence, RI</span> </p> <p>Publication date: November 1, 2020 </p> <div id="moredetails"> <span class="expand">More about this publication?</span> <ul class="collapse normal"> <li class="descriptionitem"><div class="originaldescription"><i>Allergy and Asthma Proceedings</i> 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. <br><br>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. <a href="http://www.allergyandasthmaproceedings.com">AAP </a> welcomes the submission of original works including peer-reviewed original research and clinical trial results. Additionally, as the official journal of the <a href="https://easternallergyconference.org"> Eastern Allergy Conference (EAC)</a>, AAP will publish content from EAC poster sessions as well as review articles derived from EAC lectures. <br><br>Featured topics include asthma, rhinitis, sinusitis, food allergies, allergic skin diseases, diagnostic techniques, allergens, and treatment modalities. 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