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• W2083996924 abstract "Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH), also known as HAE type III, is a familial condition only clinically recognized within the past three decades. Similar to HAE from C1-INH deficiency (HAE types I and II), affected individuals experience unpredictable angioedema episodes of the skin, gastrointestinal tract, and airway. Unique clinical features of HAE with normal C1-INH include the predominance of affected women, frequent exacerbation by estrogen, and a prominence of angioedema that involves the face and oropharynx. The underlying pathophysiology of HAE with normal C1-INH is poorly understood, but indirect evidence points to contact pathway dysregulation with bradykinin-mediated angioedema. Currently, evaluation is complicated by a lack of confirmatory laboratory testing such that clinical criteria must often be used to make the diagnosis of HAE with normal C1-INH. Factor XII mutations have been identified in only a minority of persons affected by HAE with normal C1-INH, limiting the utility of such analysis. To date, no controlled clinical studies have examined the efficacy of therapeutic agents for HAE with normal C1-INH, although published evidence supports frequent clinical benefit with medications shown effective in HAE due to C1-INH deficiency. Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH), also known as HAE type III, is a familial condition only clinically recognized within the past three decades. Similar to HAE from C1-INH deficiency (HAE types I and II), affected individuals experience unpredictable angioedema episodes of the skin, gastrointestinal tract, and airway. Unique clinical features of HAE with normal C1-INH include the predominance of affected women, frequent exacerbation by estrogen, and a prominence of angioedema that involves the face and oropharynx. The underlying pathophysiology of HAE with normal C1-INH is poorly understood, but indirect evidence points to contact pathway dysregulation with bradykinin-mediated angioedema. Currently, evaluation is complicated by a lack of confirmatory laboratory testing such that clinical criteria must often be used to make the diagnosis of HAE with normal C1-INH. Factor XII mutations have been identified in only a minority of persons affected by HAE with normal C1-INH, limiting the utility of such analysis. To date, no controlled clinical studies have examined the efficacy of therapeutic agents for HAE with normal C1-INH, although published evidence supports frequent clinical benefit with medications shown effective in HAE due to C1-INH deficiency. RE: Successful management of hereditary angioedema with normal C1-INH (type III HAE) when using on-demand ecallantideThe Journal of Allergy and Clinical Immunology: In PracticeVol. 2Issue 2PreviewRiedl1 recently reviewed clinical characteristics of hereditary angioedema with normal C1 inhibitor (HAE-nmlC1-INH), also known as type III hereditary angioedema. In his review, the potential role of bradykinin in the pathogenesis of HAE-nmlC1-INH is discussed in light of several case series that demonstrated successful on-demand treatment of acute attacks with the bradykinin B2 receptor antagonist icatibant and one case report that demonstrated successful on-demand treatment of an acute attack of HAE-nmlC1-INH in an adolescent boy with ecallantide. Full-Text PDF" @default.
• W2083996924 created "2016-06-24" @default.
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• W2083996924 date "2013-09-01" @default.
• W2083996924 modified "2023-09-27" @default.
• W2083996924 title "Hereditary Angioedema with Normal C1-INH (HAE Type III)" @default.
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• W2083996924 doi "https://doi.org/10.1016/j.jaip.2013.06.004" @default.
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