Pediatric hereditary angioedema: an update
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چکیده
منابع مشابه
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...
متن کاملPediatric 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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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 (HAE) is a rare genetic condition that manifests as painful and potentially life-threatening episodic attacks of cutaneous and submucosal swelling. It results from functional deficiency of C1 esterase inhibitor (C1-INH), which is a regulator of the complement system, contact/kinin system and coagulation system. In HAE patients, the lowplasma concentration of functional C1-...
متن کاملHereditary 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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ژورنال
عنوان ژورنال: F1000Research
سال: 2017
ISSN: 2046-1402
DOI: 10.12688/f1000research.11320.1