Assessment of coronary wall thickening in autosomal dominant hyper- immunoglobin E syndrome (AD-HIES) using TRAPD-MRI
نویسندگان
چکیده
منابع مشابه
Assessment of coronary wall thickening in autosomal dominant hyper- immunoglobin E syndrome (AD-HIES) using TRAPD-MRI
Background Autosomal dominant hyper-IgE (AD-HIES), also called Job’s syndrome, is a rare primary immunodeficiency caused by dominant mutations in STAT3. AD-HIES is characterized by elevated levels of IgE, an ineffective immune response to several infectious agents, and connective tissue and arterial abnormalities. To date, coronary artery evaluation in AD-HIES patients has been limited to lumen...
متن کاملAutosomal dominant hyper-IgE syndrome
Th e hyper-IgE recurrent infection syndromes (HIES) comprise a group of primary immunodefi ciency dis orders that exhibit markedly elevated IgE levels, recur rent staphylococcal skin abscesses, eczema and pulmonary infections. Both autosomal dominant and autosomal recessive forms of the disorder have been described. Most autosomal dominant HIES (AD-HIES) have been found to be due to mutations i...
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چکیده ندارد.
Update on Hyper IgE syndrome (HIES)
Article type: Review Article Hyper IgE Syndrome (HIES) is a rare primary immunodeficiency disease. Most of HIES cases are sporadic. Autosomal dominant HIES is caused by mutation in signal transducer and activator of transcription-3 (STAT-3). A number of mosaicism HIES has been reported that is associated with intermediate phenotype. Autosomal recessive HIES is due to mutation in Dock-8 or cytok...
متن کاملupdate on hyper ige syndrome (hies)
hyper ige syndrome (hies) is a rare primary immunodeficiency disease. most of hies cases are sporadic. hies type ad is caused by mutation in signal transducer and activator of transcription-3 (stat-3). a number of mosaicism hies has been reported that is associated with intermediate phenotype. autosomal recessive hies (ar-hies) is due to mutation in dock-8 or cytokine sis 8 and tyk2 or tyrosine...
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ژورنال
عنوان ژورنال: Journal of Cardiovascular Magnetic Resonance
سال: 2016
ISSN: 1532-429X
DOI: 10.1186/1532-429x-18-s1-o5