Kawasaki Syndrome – A Brief History
In 1961 a Japanese pediatrician named Tomisaku Kawasaki saw his first patient with symptoms of a medical condition for which he could find no previous literature for. He scoured the medical journals and reference books to try to diagnose this 4-year-old Japanese boy – but to no avail. Almost a year later, another young Japanese boy presented with similar symptoms as the 4-year-old boy he had seen a year before. At this time, Dr. Kawasaki began to realize that he was witnessing something new – perhaps never before seen or documented. Over the next 6 years he saw 50 more children who presented with the same strange series of symptoms. During this period of time he began to document all of the clinical manifestations he was seeing. After a period of time he gathered his information and wrote a report for a clinical conference he was attending at the hospital. The report was entitled “Acute Febrile Mucocutaneous Lymph Node Syndrome.”
Following the presentation of his report Dr. Kawasaki began to hear from a number of other physicians regarding their similar experience with the same sequence of symptoms in children. By 1970, it was recognized by the Japanese Committee of Health and Welfare that a new disease involving all of the major arteries of the body was developing.
Approximately 10 years later a certain pharmaceutical intervention was commonly being used to treat children with Acute Febrile Mucocutaneous Lymph Node Syndrome – later to become known as Kawasaki Syndrome or Kawasaki’s Disease. (The Japan Times Online)
Signs and Symptoms of Kawasaki’s Disease
(Wikipedia & American Heart Association & Kids Health.org )
Fever – The earliest stage of Kawasaki Syndrome is the onset of fever. What is different about this fever is that it lasts longer than 5 days and can persist as long as 25 days. The fever can be very high and is also unresponsive to usual fever treatments (such as Children’s Tylenol).
Rash: A rash usually appears on the mid-section of the body but can also spread down the arms and legs.
Swollen Neck Lymph Nodes: Lymph nodes in the neck become swollen. The lymphadenopathy is usually unilateral.
Red Mucous Membranes: Mouth, lips, tongue, nipples, genitals may all appear strawberry red.
Swollen & Red Hands and Feet: The palms of the hands and bottoms of the feet usually become very red and can be hot to touch. The top of the hands and feet may also be involved. The skin of the hands and feet may begin to peel.
Red Eyes: The whites of the eyes can become very red. The cheeks may also be involved.
Additional symptoms may include:
- Irritability
- Diarrhea, vomiting, and abdominal pain
- Cough and runny nose
- Joint pain and swelling, frequently on both sides of the body
Kawasaki’s disease usually affects children under 5 years of age.
Etiology
While it is not entirely known what causes Kawasaki Syndrome, current research and theory suggests that it is an autoimmune disease. There is also a possibility that the disease originates from environmental causes (such as viral or bacteriologic infections) or immunologic and/or genetic factors. It is suggested that certain antigens could cause the body to have an immunologic response creating a reaction in the body that evokes inflammation of the arteries and surrounding tissues. There appears to be a genetic link as well. Although the disease has been recorded all around the world, young Asian boys have the highest incidence of the disease. There is also a familial link. It has often been seen that children of the same family can suffer from the illness.
Complications of Kawasaki’s Disease
(Wikipedia & Mayo Clinic)
Cardiac complications are the most severe side effect of Kawasaki Syndrome. Typically the acute inflammation seen in this disease affects the body at the vascular level. Almost all of the blood vessels (including the major vessels of the heart) become inflamed and can lead to the formation of vascular aneurysms – especially in the coronary arteries. It is vital that the child be treated as soon as possible after diagnosis because delay in pharmaceutical intervention may lead to the onset of cardiac and vascular problems in the future. Kawasaki Syndrome can lead to sudden death from myocardial infarction if left untreated through the duration of the illness. The earlier the treatment – the less likely the child will develop any form of heart disease in the future. Kawasaki’s disease is one of the major causes of infant and childhood heart disease.
Heart complications include:
- Inflammation of the heart muscle (myocarditis)
- Heart valve problems (mitral regurgitation)
- Abnormal heart rhythm (arrhythmia)
- Inflammation of blood vessels (vasculitis), usually the coronary arteries, which supply blood to the heart
Any of the above complications can cause the child’s heart to malfunction. The inflammation of the coronary arteries can lead to weakening and bulging of the artery wall (aneurysm). Aneurysms increase the risk of blood clots forming and blocking the artery, which could lead to a heart attack or cause life-threatening internal bleeding.
For a small percentage of children who develop coronary artery problems, Kawasaki disease is fatal, even with treatment. See Reference List (6).
Diagnosis
(Wikipedia & American Heart Association)
Kawasaki syndrome is diagnosed solely by the clinical signs and symptoms. That is to say, there is currently no laboratory test that is available to confidently confirm that the patient has acquired Kawasaki’s disease. It is extremely difficult to make a definitive diagnosis because of the lack of awareness by the health care providers about the classic symptoms of the disease. In most cases, the children may have to make frequent visits to several health care providers before Kawasaki’s disease is suggested as a possible cause for the presenting symptoms. There are many other illnesses that cause a list of similar symptoms, therefore differential diagnosis is often used as a clinical tool for diagnosis. By ruling out several other illness – the health care team gets closer and closer to establishing the final diagnosis of Kawasaki’s disease. Some of the childhood diseases that exhibit a similar list of symptoms are Stevens-Johnson Syndrome, measles, Rocky Mountain spotted fever, mercury poisoning, scarlet fever, toxic shock syndrome and juvenile arthritis. See Reference List (5).
Historically, physicians have made a definitive diagnosis of Kawasaki Syndrome if the child has had a fever (unresponsive to antipyretics) for at least five days. This fever must be accompanied by the presence of at least four of five other signs and symptoms also seen with Kawasaki’s disease. The other accompanying criteria are:
- Body rash
- Strawberry tongue, red lips, mouth and mucous membranes
- Lymphadenopathy (usually unilateral and most often seen in the neck)
- Swollen/red hands and feet
- Red eyes (conjunctivitis)
Other tests available to help diagnose Kawasaki Syndrome are:
Echocardiography: This test uses sound waves to help create images of the heart. From these acquired images physicians are able to visualize the arteries and determine the presence of coronary aneurysms.
Blood Tests: Certain blood tests can determine the presence of inflammation in the blood vessels (ie: C-Reactive Protein).
Chest X-Ray: This test can help to determine if the heart has been affected.
EKG: By testing the electrical activity of the heart – doctors can see if the heart has been affected by the inflammation.
Prognosis
Treating Kawasaki Syndrome within 10 days may greatly reduce the chances of lasting damage. Most children who develop Kawasaki Syndrome fully recover within weeks of their initial symptoms. Early diagnosis and treatment, however, are vital to reduce the risk of long term damage to the heart a blood vessels. If the disease has caused permanent damage to the child’s heart on-going cardiac monitoring and care may be necessary throughout childhood and into his or her adult life. 1% of all children diagnosed with Kawasaki Syndrome will die of complications associated with the disease. Approximately 30% develop cardiac disease, 20% develop coronary artery abnormalities and 30% develop inflammatory arthritis. See reference list (4).
Pathophysiology of Kawasaki Syndrome
The pathophysiology of the disease is currently under investigation and is still unknown. See reference list (8).
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