A transcriptomics study of hereditary angioedema attacks

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Hereditary angioedema: a family study.

Hereditary angioedema (HAE) is a rare, life-threatening, autosomal dominant disease characterized by recurrent episodes of angioedema, and caused by a deficiency of the plasma protein C1-esterase inhibitor (C1-INH). Clinical manifestation of HAE may first develop during childhood but typically presents around puberty with nonpruritic and non-pitting edema of the subcutaneous and mucosal tissues...

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Management of acute attacks of hereditary angioedema: role of ecallantide

Hereditary angioedema (HAE) is characterized as an episodic swelling disorder with autosomal dominant inheritance. Clinical features include nonpitting edema of external or mucosal body surfaces, and patients often present with swelling of the extremities, abdominal pain, and swelling of the mouth and throat, which can lead to asphyxiation. Patients with HAE classically have no associated urtic...

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Emergency Department Management of Hereditary Angioedema Attacks: Patient Perspectives.

BACKGROUND Emergency department (ED) management of hereditary angioedema (HAE) has been hindered by misdiagnosis and limited treatment options. Food and Drug Administration approval of 4 on-demand HAE therapies starting in 2009 and the publication of ED guidelines for angioedema management in 2014 should facilitate improvement of HAE management in the ED. OBJECTIVE The objective of this study...

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Dedicated call center (SOS-HAE) for hereditary angioedema attacks: study protocol for a randomised controlled trial

BACKGROUND Despite the availability of guidelines for the specific treatment of hereditary angioedema (HAE) attacks, HAE morbidity and mortality rates remain substantial. HAE attacks are a major medical issue requiring specific treatment as well as a considerable socio-economic burden. We report a protocol designed to test whether a dedicated call centre is more effective than usual practice in...

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Background The efficacy and safety of icatibant for the treatment of edematous hereditary angioedema (HAE) attacks was established in three Phase III trials, including the For Angioedema Subcutaneous Treatment-3 study (FAST-3; NCT00912093). Here, data from the double-blind, controlled phase and open-label extension (OLE) of FAST-3 were analyzed post-hoc to specifically evaluate icatibant for th...

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ژورنال

عنوان ژورنال: Journal of Allergy and Clinical Immunology

سال: 2018

ISSN: 0091-6749

DOI: 10.1016/j.jaci.2018.03.016