Kawasaki disease (川崎症), also known as mucocutaneous lymph node syndrome, is an acute systemic vasculitis (inflammation of mostly medium-sized blood vessels) common in children aged 2 to 5. It is the most common cause of acquired heart disease in children in developed countries. The cause is still unknown but a yet unknown inciting agent is theorized. There are about 100 cases in Hong Kong every year. Kawasaki disease can cause inflammation of blood vessels throughout the body, with a predilection to affect the coronary (heart) arteries. In severe cases, coronary artery aneurysm may occur, leading to blood vessel rupture and death. Children aged 6 months to 5 years old have a higher risk for Kawasaki disease. Early recognition and initiation of treatment are the mainstay of management to prevent morbidity and mortality.
The cause of Kawasaki disease is still unknown. The medical community generally believes it is related to exposure to certain substances. If a parent or sibling has been infected, the incidence rate will increase exponentially, but it is not contagious. It is known to increase the risk of kawasaki disease in children, including:
Kawasaki disease has a rapid onset and symptoms appear in three stages, namely: acute, subacute and convalescent.
The Acute Stage lasts from 1-2 weeks (up to 4 weeks if left untreated) and has the following symptoms:
The Subacute Stage starts when the fever dissipates to about three weeks thereafter, it is characterised by:
The Convalescent stage typically starts on subsidence/resolution of clinical signs, usually within three months of the onset of acute illness. “Beau’s lines”, characteristic transverse groove on the fingernails, can be noticed in patients on this stage.
The doctor will conduct a physical examination and ask about the child's symptoms, asking if there is abrupt, high-grade fever of 5 days or more and at least 4 of the following symptoms:
Other tests that may be needed include:
Initiation of immune globulin therapy within 7-10 days of the onset of illness is the cornerstone of treatment and decreases the incidence of developing coronary artery aneurysm from 25% to 3-5%.
Over 50% of patients will have complete resolution of coronary artery lesions one to two years after recovery. For children with residual cardiac findings, regular monitoring of cardiac status (e.g. ECG, echocardiography) and long-term medication is recommended.
There are currently no preventive measures against Kawasaki disease, but a high index of suspicion, early diagnosis and treatment can lead to more favorable outcomes and avoidance of long-term cardiac complications.
Kawasaki disease is an acute systemic vasculitis with an unknown cause. It is common in children aged 2 to 5 years old. It causes inflammation of blood vessels in the body with a predilection to affect the coronary arteries. Weakening of the walls of the coronaries can lead to a condition known as an aneurysm (ballooning) which can then rupture and cause death. The peak incidence of Kawasaki disease is in children aged 6 months to 5 years old. Parents should pay attention to the symptoms to facilitate early check-up, diagnosis and treatment. If you want to know more, you can make an appointment with a doctor for consultation.
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