Kawasaki disease (KD) is an acute, self-limiting, systemic vasculitis of unknown aetiology, which most commonly occurs in children aged 6 mo to 5 y, with a peak incidence at 9-11 mo. The inflammatory process preferentially involves the coronary arteries, potentially resulting in coronary arteritis, aneurysmal lesions, arterial thrombotic occlusion and sudden death. Kawasaki disease is the most common cause of acquired coronary vessel abnormalities in children. The cause of KD is not known, but evidence is presented for an inflammatory response and a genetic predisposition. The diagnostic tests are not yet defined, but treatment with immunoglobulin and aspirin is effective at reducing the risk of cardiac complications from 25% to 4.7% in the UK. Sequelae may occur, either acutely with myocardial, endocardial or pericardial inflammation, or many years after the original illness. There may be abnormalities of myocardial blood flow as assessed by MRI, radionucleide studies or echo Doppler. Such abnormalities of coronary arteries may require ongoing medication, interventional catheterization or even cardiac surgery. Conclusion: In the future, we hope to have more accurate diagnostic tests or prophylaxis against the disease, in addition to improved means of determining the susceptibility to or presence of long-term complications.
|Number of pages||5|
|Journal||Acta Paediatrica, International Journal of Paediatrics, Supplement|
|Publication status||Published - 1 Dec 2004|
- Coronary aneurysms
- Endothelial dysfunction