Kawasaki Syndrome (Disease)
Kawasaki Syndrome, which mostly occurs in children below 5 years of age, results in vasculitis, or inflammation of the walls of the blood vessels, throughout the body.
- The common symptoms include fever, a bright red coloration of the tongue, usually called strawberry tongue, swollen and cracked lips, and rashes in different parts of the body.
- In some cases, the disease causes serious heart complications which can become fatal, if not treated.
- Kawasaki Syndrome is thought to be associated with some infection, but the exact cause is not known.
- Kawasaki Syndrome is diagnosed based entirely on symptoms, as no specific diagnostic test is available.
- Almost all the children who are affected by Kawasaki Syndrome recover completely with timely medical intervention.
- Treatment involves administration of aspirin and immunoglobulin in high doses.
Kawasaki Syndrome, which is also known as Kawasaki disease, causes the walls of the blood vessels to become inflamed. The inflammation is usually wide spread, affecting almost all the blood vessels in the body. Most of the serious consequences of the disease result from the inflammation of the blood vessels of the heart. Kidneys and pancreas also can be affected. The exact cause of Kawasaki Syndrome is not yet known, but it is thought to be an auto immune disease triggered by a virus or some other organism or an abnormal response of the immune system to certain infections. A genetic predisposition to the disease is observed in children of Japanese descent.
Children less than 5 years of age are the most affected, while most cases of Kawasaki Syndrome occur in children between 1 to 8 years of age. However, younger infants as well as adolescents are also affected. The incidence in boys is double that of girls. Though the disease is more prevalent in children of Japanese origin, thousands of cases are reported every year in the United States alone.
Symptoms
High fever marks the beginning of the disease. High temperature, often above 102° F or 38.9° C, may prevail for about 1 week to 3 weeks. Conjunctivitis or redness of eyes may appear in a day or two, but no discharge from the eye is seen. Rashes in different parts of the body may appear in about 5 days. The trunk, diaper area, and the mucous membranes are the most affected. The lining of the mouth, and vagina in girls, become red in color; the throat, tongue and the lips also become bright red. The swollen, red lips become dry and develop vertical cracks. The palm of the hand and the soles of the feet appear purplish red. The hands and feet may become swollen and even painful. Peeling of skin from the fingers and toes may start about ten days after the onset of the disease. Swollen lymph nodes found in the neck area is a typical symptom, and they may be tender to touch. The disease may persist from a few weeks to a few months.
In about half the cases, the disease may affect the heart and cause irregular heart rhythms and rapid heartbeats. These complications usually start within the first one to four weeks, and affect one out of every five children with the disease. If it is not treated immediately, half of these affected children may develop a severe heart problem known as coronary artery aneurysm. It is bulge in the arterial wall of the coronary artery, which can promote blood clotting that may result in heart attacks. Those who have aneurisms are at greater risk of sudden death due to a blockage of the coronary artery, or rupture of the aneurism. Meningitis, or the inflammation of the membranes lining the brain, and that of tissues lining the eyes, ears, urethra, and joints, may result in painful conditions. Liver and gallbladder also may be affected. However, these symptoms do not cause any permanent damage, and get resolved eventually.
Diagnosis
Diagnosis of Kawasaki Syndrome is based on certain predefined symptoms. If a child has four out of these five bench mark symptoms, Kawasaki Syndrome is diagnosed. Other diseases such as juvenile idiopathic arthritis, scarlet fever, and measles, which have similar symptoms, are ruled out by blood tests and blood culture. Throat swabs are also taken for cultural study. Pediatric cardiologists experienced in treating children with heart disorders are consulted, to deal with the heart complications which may develop due to Kawasaki Syndrome.
On diagnosing Kawasaki Syndrome, further investigations are initiated to detect possible heart complications such as leaking heart valves and coronary artery aneurysms. Pericarditis, or inflammation of the pericardium enveloping the heart, and heart muscle inflammation, known as myocarditis, is also investigated. Electrocardiography as well as echocardiography is done to determine the type and extent of the damage. Heart complications may not always develop at the beginning of the disease, but they can appear at any time during the following months, even up to a year from the onset of the disease. Hence, the ECG and echocardiography are repeated at specific intervals, first at two to three weeks, then again at six to eight weeks, and may be after six months to one year.
If the results of Electrocardiography tests or echocardiography are found to be abnormal, a stress test may be conducted. Heart catheterization may be required, if aneurisms are detected.
Prognosis
Complete recovery is possible in most cases of Kawasaki Syndrome, especially if the coronary arteries of the children are not affected by the disease in the first two months. If coronary artery is affected, the prognosis usually depends on the extent of damage caused; but with timely treatment, fatality due to Kawasaki Syndrome is almost nil in the United States. If the condition is not treated, about 1% of affected children may die of the disease or its complications, mostly within 6 months from the time of appearance of the symptoms. But in some cases, death may occur even 10 years after having the disease. Aneurysms may get resolved within 1 year in most of the cases. If aneurisms are large, the chances of them resolving are less. Even if the aneurysms eventually resolve, children who have had them are at a higher risk of developing heart problems later in life.
Treatment
Treatment initiated within 10 days of the onset of fever can considerably reduce the damage caused to the coronary artery. Other distressing symptoms such as rashes, high temperature, and general discomfort, are also reduced within days of starting drug therapy. Immunoglobulin is administered intravenously in high doses for one to four days, while aspirin in high doses is given orally. The dosage of aspirin is reduced when the fever subsides, usually within four or five days. However, lower doses of aspirin should be continued for at least two months from the first appearance of symptoms. It is discontinued only if all symptoms of inflammation have disappeared, and if coronary artery aneurysms are absent. To minimize the risk of blood clotting, aspirin has to be used log-term, if coronary artery aneurism is present.
The use of aspirin in children who have a viral infection such as chicken pox or influenza considerably increases the risk of a potentially fatal condition called Reye syndrome. Children who require long-term use of aspirin, such as those who have developed Kawasaki Syndrome, are vaccinated against these infectious viral diseases as a precaution. Influenza vaccine needs to be repeated every year; and it is given as an injection, instead of as a nasal spray. If a child on aspirin therapy contracts chickenpox or influenza, aspirin is temporarily discontinued, and dipyridamole is given instead, to minimize the risk of developing Reye syndrome. If a child has a large coronary aneurysm, anticoagulants such as dipyridamole or warfarin may be used to prevent blood clots from forming.
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Yasser Elnahas
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