Allergy and asthma proceedings :
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Allergy Asthma Proc · Sep 2011
Case ReportsHereditary angioedema: a case of near fatal laryngeal swelling in a 41-year-old woman.
Hereditary angioedema (HAE) is characterized by nonpitting, nonpruritic subcutaneous, or submucosal edema that may be accompanied by a nonpruritic serpentine erythematous rash. The swelling in this autosomal dominantly inherited disorder is mediated by uncontrolled localized bradykinin production. The extremities, face, gastrointestinal tract, genitalia, larynx, and trunk are commonly involved. ⋯ Recently approved treatments for HAE swelling attacks include C1 esterase inhibitor concentrate and ecallantide. This case describes a 41-year-old woman who was treated ineffectively for more than 20 years, was switched to an effective therapy, but suffered near fatal laryngeal edema due to a missed dose of the effective therapy. The case underscores the need for increased awareness of HAE among clinicians and the importance of good compliance to therapy among patients with HAE.
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Allergy Asthma Proc · Sep 2011
Descriptive epidemiology of hereditary angioedema emergency department visits in the United States, 2006-2007.
Hereditary angioedema (HAE) is a rare inherited disorder of complement factor C1 inhibitor. In 2007 there were over 2000 HAE-related emergency department (ED) visits, nearly one-half of which culminated in a hospitalization. This study examines epidemiology and outcomes of hospital ED visits among HAE patients. ⋯ Mean HAE ED visits costs were $1479 (95% confidence interval, $1028-1929). HAE ED visit volume is substantial. Although likely representing a fraction of the entire HAE population, prevention and acute treatment strategies aimed at those at risk for frequent exacerbations and disproportionate resource use need to be examined.