Pediatric hereditary angioedema

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Pediatric hereditary angioedema

Hereditary angioedema (HAE) is a lifelong illness characterized by recurrent swelling of the skin, intestinal tract, and, ominously, the upper airway. It is caused by inadequate activity of the protein C1-inhibitor, with dysfunction in the kallikrein/bradykinin pathway underlying the clinical symptoms. In addition to the physical symptoms, patients experience significant decrements in vocationa...

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Pediatric hereditary angioedema: an update

Hereditary angioedema (HAE) with C1-inhibitor (C1-Inh) deficiency (C1-Inh-HAE) is a rare, life-threatening, and disabling genetic disorder characterized by self-limited tissue swelling caused by deficiency or dysfunction of C1-Inh. Our aim in this update is to discuss new advances in HAE therapy, focusing mainly on the various treatment options that have become available recently and also drugs...

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Future Therapy for Pediatric Hereditary Angioedema

Hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency is characterized by recurrent attacks of swelling with symptoms typically beginning in childhood. The introduction of targeted therapies for prevention and treatment of attacks has been of significant benefit to patients, including children, with HAE. However, the ideal of a safe, convenient therapy that prevents all attacks ha...

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Hereditary Angioedema

Copyright © 2008 Massachusetts Medical Society. A 19-year-old woman presents to the emergency department with light-headedness, severe abdominal pain, and intractable nausea and vomiting that began 12 hours earlier. The patient reports previous episodes of abdominal pain and swelling of her hands and feet that have been attributed possibly to food allergies, which have recently become more freq...

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Pediatric hereditary angioedema due to C1-inhibitor deficiency

Hereditary angioedema (HAE) resulting from the deficiency of the C1 inhibitor (C1-INH) is a rare, life-threatening disorder. It is characterized by attacks of angioedema involving the skin and/or the mucosa of the upper airways, as well as the intestinal mucosa. In approximately 50 per cent of cases, clinical manifestations may appear during childhood. The complex management of HAE in pediatric...

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ژورنال

عنوان ژورنال: Pediatric Allergy and Immunology

سال: 2013

ISSN: 0905-6157,1399-3038

DOI: 10.1111/pai.12168