Coronary Artery Abnormalities in Hyper-IgE Syndrome
| Autor: | Pamela Welch, Elizabeth Mannino Avila, Colleen Hadigan, Alexandra F. Freeman, Pamela A. Shaw, Joie Davis, Jatin R. Matta, Amy P. Hsu, Steven M. Holland, Ahmed M. Gharib, Roderic I. Pettigrew |
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| Rok vydání: | 2011 |
| Předmět: |
Adult
Male STAT3 Transcription Factor medicine.medical_specialty Adolescent Immunology Population Coronary Artery Disease Coronary Angiography Article Magnetic resonance angiography Cohort Studies Coronary artery disease Aneurysm Internal medicine medicine Humans Immunology and Allergy Child education Genes Dominant Coronary artery aneurysm education.field_of_study medicine.diagnostic_test business.industry Coronary Aneurysm Magnetic resonance imaging Middle Aged medicine.disease Coronary Vessels medicine.anatomical_structure Job Syndrome Case-Control Studies Hypertension Mutation Cardiology Female Radiology Tomography X-Ray Computed business Magnetic Resonance Angiography Artery |
| Zdroj: | Journal of Clinical Immunology. 31:338-345 |
| ISSN: | 1573-2592 0271-9142 |
| DOI: | 10.1007/s10875-011-9515-9 |
| Popis: | Hyper-IgE syndrome (HIES) is a rare primary immunodeficiency caused by autosomal dominant STAT3 mutations resulting in recurrent infections and connective tissue abnormalities. Coronary artery abnormalities have been reported infrequently. We aimed to determine the frequency and characteristics of coronary artery abnormalities. STAT3-mutated HIES patients (n = 38), ranging in age from 8 to 57 years, underwent coronary artery imaging by computed tomography or magnetic resonance imaging. Images were evaluated for tortuosity, dilation, and aneurysm. Charts were reviewed for cardiac risk factors. To allow blinded image interpretation, an age- and gender-matched non-HIES group was also evaluated (n = 33). Coronary artery tortuosity or dilation occurred in 70% of HIES patients, with aneurysms present in 37%, incidences much higher than in the literature and in our non-HIES group, in which 21% had tortuosity or dilation and 3% had aneurysms. Hypertension was more common in the HIES group than in the general population and was associated with vessel abnormalities. Atherosclerosis was uncommon and mild. Coronary artery aneurysms and tortuosity are common in HIES, despite a paucity of atherosclerosis, suggesting that STAT3 plays an integral role in human vascular remodeling and atherosclerosis. |
| Databáze: | OpenAIRE |
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