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Kawasaki disease in children
1Bristol Royal Hospital for Children, Bristol, UK.
Insights
Kawasaki disease (KD) is a childhood vasculitis affecting coronary arteries. Prompt treatment with immunoglobulin and aspirin significantly lowers severe cardiac complications in affected children.
Area of Science:
- Pediatric Cardiology
- Immunology
- Rheumatology
Background:
- Kawasaki disease (KD) is the leading cause of acquired heart disease in children.
- It involves acute, self-limiting systemic vasculitis with unknown origins.
- Coronary artery inflammation can lead to aneurysms, thrombosis, or sudden death.
Purpose of the Study:
- To provide a comprehensive review of Kawasaki disease.
- To discuss its history, diagnosis, epidemiology, and etiology.
- To explore immunopathogenesis, treatment strategies, and long-term cardiovascular outcomes.
Main Methods:
- Literature review of Kawasaki disease.
- Analysis of diagnostic challenges and epidemiological data.
- Synthesis of information on pathology, immunopathogenesis, and treatment.
Main Results:
- No specific diagnostic test exists for Kawasaki disease.
- Intravenous immunoglobulin and aspirin reduce cardiac complications from 25% to 4.7%.
- Myocardial and endothelial dysfunction can necessitate ongoing medical or surgical intervention.
Conclusions:
- Kawasaki disease requires prompt diagnosis and management to prevent severe cardiac sequelae.
- Understanding genetic influences and long-term cardiovascular effects is crucial.
- Emerging pharmacological treatments offer potential for improved management in children and adolescents.
Abstract:
Kawasaki disease (KD) is an acute self-limiting systemic vasculitis of unknown aetiology. It is the most common cause of acquired heart disease in young children. The intense inflammatory process has a predilection for the coronary arteries, resulting in the development of aneurysmal lesions, arterial thrombotic occlusion or, potentially, sudden death. There is no specific diagnostic test; however, treatment with immunoglobulin and aspirin effectively reduces cardiac complications from 25% to 4.7% in the UK. Inflammation of the myocardium, endocardium or pericardium can occur early in the disease and endothelial dysfunction along with abnormalities of myocardial blood flow may require continuing medication, interventional catheterisation or even cardiac surgery. Several new pharmacological treatments may have important roles to play in managing KD in children and adolescents. This review discusses the history of the disease, the diagnostic challenges, epidemiology, aetiology, pathology, immunopathogenesis, treatment, genetic influences and the long-term cardiovascular sequelae.
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