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Identifying Dysregulated Genes Induced by Kaposi's Sarcoma-associated Herpesvirus KSHV
Published on: September 14, 2010
Kawasaki Disease
Jane W Newburger1, Masato Takahashi2, Jane C Burns3
1Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
Insights
Early treatment of Kawasaki disease with intravenous immunoglobulin significantly reduces coronary artery aneurysm risk. Untreated, this childhood vasculitis can lead to long-term heart complications, including myocardial infarction in young adults.
Area of Science:
- Pediatrics
- Cardiology
- Rheumatology
Background:
- Kawasaki disease is a critical pediatric vasculitis impacting coronary arteries.
- Delayed treatment elevates the risk of coronary artery aneurysms, a major cause of cardiac morbidity.
- Aneurysms can progress, leading to stenosis and myocardial infarction, even in adulthood.
Purpose of the Study:
- To highlight the critical importance of early intravenous immunoglobulin treatment for Kawasaki disease.
- To underscore the long-term cardiovascular sequelae of untreated or delayed-treated Kawasaki disease.
- To inform about the evolving nature of coronary artery aneurysms and their management.
Main Methods:
- Review of clinical data and outcomes in Kawasaki disease patients.
- Analysis of the impact of treatment timing on coronary artery aneurysm formation.
- Longitudinal observation of aneurysm evolution and associated cardiovascular events.
Main Results:
- Intravenous immunoglobulin within 10 days of fever onset reduces aneurysm risk fivefold.
- Coronary artery aneurysms peak in size by 6 weeks but can progress over years.
- Kawasaki disease is an emerging cause of acute coronary syndrome in young adults.
Conclusions:
- Prompt diagnosis and treatment of Kawasaki disease are vital to prevent severe coronary artery damage.
- Long-term cardiovascular surveillance is necessary for patients with Kawasaki disease, especially those with aneurysms.
- Management strategies for Kawasaki disease-induced coronary artery disease include surgical and interventional approaches.
Abstract:
Kawasaki disease is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and children. If not treated early with high-dose intravenous immunoglobulin, 1 in 5 children develop coronary artery aneurysms; this risk is reduced 5-fold if intravenous immunoglobulin is administered within 10 days of fever onset. Coronary artery aneurysms evolve dynamically over time, usually reaching a peak dimension by 6 weeks after illness onset. Almost all the morbidity and mortality occur in patients with giant aneurysms. Risk of myocardial infarction from coronary artery thrombosis is greatest in the first 2 years after illness onset. However, stenosis and occlusion progress over years. Indeed, Kawasaki disease is no longer a rare cause of acute coronary syndrome presenting in young adults. Both coronary artery bypass surgery and percutaneous intervention have been used to treat Kawasaki disease patients who develop myocardial ischemia as a consequence of coronary artery aneurysms and stenosis.
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