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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, David R Fulton
1Department of Cardiology at Children's Hospital, Boston, Massachusetts 02115, USA. jane.newburger@cardio.chboston.org
Insights
Kawasaki disease, a childhood vasculitis, can lead to heart issues. Intravenous immune globulin therapy is effective in preventing coronary aneurysms, though resistant cases require further research.
Area of Science:
- Pediatric rheumatology
- Cardiovascular pathology
- Immunology
Background:
- Kawasaki disease is a leading cause of acquired heart disease in children in the US.
- It presents as an acute vasculitis with characteristic clinical signs.
- Coronary artery aneurysms are a significant complication, potentially leading to severe cardiac events.
Purpose of the Study:
- To review key data on Kawasaki disease etiology, pathogenesis, and treatment.
- To highlight recent publications and advancements in the field.
- To discuss long-term outcomes and management strategies.
Main Methods:
- Comprehensive literature review of recent publications.
- Analysis of epidemiological data on acquired heart disease in children.
- Synthesis of current understanding of disease mechanisms and therapeutic responses.
Main Results:
- Intravenous immune globulin (IVIG) therapy is effective in reducing coronary aneurysms in acute Kawasaki disease.
- The cause of Kawasaki disease remains unknown.
- Treatment for IVIG-resistant cases is an area of ongoing research and clinical debate.
Conclusions:
- Kawasaki disease management and long-term outcomes are closely linked to coronary artery status.
- Continued research is vital for understanding and treating resistant cases.
- Effective acute-phase treatment significantly impacts pediatric cardiovascular health.
Purpose Of Review:
Kawasaki disease is an acute, self-limited vasculitis of childhood characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in approximately 15 to 25% of untreated children with the disease and may lead to myocardial infarction, sudden death, or ischemic heart disease.
Recent Findings:
In the United States, Kawasaki disease has now surpassed acute rheumatic fever as the leading cause of acquired heart disease in children. The cause of Kawasaki disease remains unknown, but fortunately intravenous immune globulin therapy has proved to be effective at reducing the prevalence of coronary aneurysms in most children treated in the acute phase. Therapy for Kawasaki disease resistant to intravenous immune globulin therapy is an area of research and controversy. The long-term treatment of children with Kawasaki disease is dependent on coronary artery status.
Summary:
This review covers key data on the etiology, pathogenesis, treatment, and long-term outcomes of Kawasaki disease, highlighting recent publications.
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