ArticleAtherosclerosis2017
Coronary atherosclerosis and dilation in hyper IgE syndrome patients: Depiction by magnetic resonance vessel wall imaging and pathological correlation.
Article in Atherosclerosis, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed.
- Impaired angiogenesis and extracellular matrix metabolism in autosomal-dominant hyper-IgE syndrome.The Journal of clinical investigation · 2020Trial
- Obesity and hepatic steatosis in STAT3 hyper-IgE syndrome.The journal of allergy and clinical immunology. In practice · 2026Article
- Case Report: Biliary hemorrhage by intrahepatic pseudoaneurysm and asymptomatic right coronary artery pseudoaneurysm in a patient with STAT3 hyper IgE syndrome.Frontiers in immunology · 2025Article
- Association of Coronary Wall Thickening and Diminished Diastolic Function in Asymptomatic, Low Cardiovascular Disease-Risk Persons Living with HIV.Radiology. Cardiothoracic imaging · 2024Observational
- STAT3 Hyper-IgE Syndrome-an Update and Unanswered Questions.Journal of clinical immunology · 2021Review
- The Child with Elevated IgE and Infection Susceptibility.Current allergy and asthma reports · 2020Review
- Hematopoietic Stem Cell Transplantation and Vasculopathy Associated With STAT3-Dominant-Negative Hyper-IgE Syndrome.Frontiers in pediatrics · 2020Article
- Sexual Dimorphism of Coronary Artery Disease in a Low- and Intermediate-Risk Asymptomatic Population: Association with Coronary Vessel Wall Thickness at MRI in Women.Radiology. Cardiothoracic imaging · 2019Article
- Article
- Comparison of two different measurement methods in evaluating basilar atherosclerotic plaque using high-resolution MRI at 3 tesla.BMC medical imaging · 2018Article
- Magnetic resonance imaging in heart failure, including coronary imaging: numbers, facts, and challenges.ESC heart failure · 2018Article
- Learning from Job: A Rare Genetic Disease and Lessons of Biblical Proportions.Rambam Maimonides medical journal · 2018Review
- Modulation of Immune-Inflammatory Responses in Abdominal Aortic Aneurysm: Emerging Molecular Targets.Journal of immunology research · 2018Review
- Autosomal Dominant Hyper-IgE Syndrome in the USIDNET Registry.The journal of allergy and clinical immunology. In practiceArticle
Corrections and comments
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Authors and funding
7 authors.
Funding
Abstract
BACKGROUND AND
aimsAutosomal dominant hyper-IgE (AD-HIES) is a primary immunodeficiency caused by mutations in STAT3. Elevated levels of IgE, an ineffective immune response, connective tissue abnormalities, and coronary arterial dilation and tortuosity characterize AD-HIES. To date, coronary artery evaluation in AD-HIES patients has been limited to lumenography measurements. Direct in vivo coronary vessel wall (VW) imaging may allow for better interrogation of coronary vessel abnormalities. The goal of this prospective study was to evaluate the coronary VW of AD-HIES patients using Magnetic Resonance Imaging (MRI) and histology. VW image findings were compared in healthy subjects and subjects with coronary atherosclerotic disease (CAD).
methodsA total of 28 subjects (10 with AD-HIES, 8 healthy, 10 with CAD) were studied by coronary VW MRI imaging. Additionally, a post-mortem coronary artery from one VW imaged AD-HIES patient was examined.
resultsCoronary VW in AD-HIES was thicker than in healthy controls but not significantly different from VW thickness in CAD subjects. AD-HIES coronaries showed increased VW area compared to healthy controls and CAD subjects. On histology, the AD-HIES coronary artery had findings consistent with atherosclerotic plaque, but had minimal luminal narrowing, deficient adventitia thickening and absence of both internal and external elastic laminae.
conclusionsThis is the first study to demonstrate subclinical coronary atherosclerosis in AD-HIES patients on VW imaging by MRI. Histologic evaluation confirmed the presence of atherosclerosis with lack of supportive adventitial thickening and elastic components. These findings suggest mechanisms for coronary dilation in AD-HIES and thereby help direct clinical management.
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