Coronary artery aneurysms in patients with hyper IgE recurrent infection syndrome

作者: Jennifer C. Ling , Alexandra F. Freeman , Ahmed M. Gharib , Andrew E. Arai , Robert J. Lederman

DOI: 10.1016/J.CLIM.2006.10.005

关键词:

摘要: Hyper IgE recurrent infection syndrome (HIES, or Job's syndrome) is a primary immunodeficiency characterized by skin and lung infections, eczema, elevated serum immunoglobulin E (IgE) levels, various connective tissue skeletal system abnormalities including characteristic facies, scoliosis, joint hyperextensibility, retained dentition, craniosynostosis, osteopenia, pathologic fractures. We have identified two patients with aneurysmal coronary artery disease. One was forty-three-year-old man HIES aneurysms ectasia on cardiac catheterization following myocardial infarction. The other 48-year-old ectasia-aneurysm after for evaluation of chest pain. Although are common in HIES, this the first report HIES. Further studies necessary to determine incidence, pathogenesis, optimal therapy these arterial

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