Kawasaki disease (also known as lymph node syndrome, mucocutaneous node disease, infantile polyarteritis and Kawasaki syndrome) is an inflammation (vasculitis) of the middle-sized arteries. It affects many organs, including the skin, mucous membranes, lymph nodes, and blood vessel walls, but the most serious effect is on the heart where it can cause severe aneurysmal dilations. Without treatment, mortality may approach 1%, usually within 6 weeks of onset.
Signs and Symptoms of Kawasaki disease
The first phase, which can last for up to 2 weeks, usually involves a persistent fever higher than 104° Fahrenheit (39° Celsius) and lasts for at least 5 days.
Other symptoms that typically develop include:
* severe redness in the eyes
* a rash on the stomach, chest, and genitals
* red, dry, cracked lips
* swollen tongue with a white coating and big red bumps
* sore, irritated throat
* swollen palms of the hands and soles of the feet with a purple-red color
* swollen lymph nodes
During the second phase, which usually begins within 2 weeks of when the fever started, the skin on the hands and feet may begin to peel in large pieces. The child also may experience joint pain, diarrhea, vomiting, or abdominal pain. If your child shows any of these symptoms, call your doctor.
Complications of Kawasaki disease
The cardiac complications are the most important aspect of the disease. Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms. These aneurysms can lead to myocardial infarction (heart attack) even in young children.
Causes of Kawasaki disease
Like all autoimmune diseases, the cause of Kawasaki disease is presumably the interaction of genetic and environmental factors, possibly including an infection. The specific cause is unknown, but current theories center primarily on immunological causes for the disease. Evidence increasingly points to an infectious etiology, but debate continues on whether the cause is a conventional antigenic substance or a superantigen.
Diagnosis
Kawasaki disease can only be diagnosed clinically (ie. by medical signs and symptoms). There exists no specific laboratory test that for this condition. It is normally difficult to establish the diagnosis, especially early in the course of illness, and frequently children are not diagnosed until they have seen several health care providers, or visited a number of different health care providers. Many other serious illnesses can cause similar symptoms, and must be considered in the differential diagnosis, including scarlet fever, toxic shock syndrome, juvenile idiopathic arthritis, and childhood mercury poisoning (acrodynia).
Classically, five days of fever plus four of five diagnostic criteria must be met in order to establish the diagnosis. The criteria are: (1) erythema of the lips or oral cavity or cracking of the lips; (2) rash on the trunk; (3) swelling or erythema of the hands or feet; (4) red eyes (conjunctival injection) (5) swollen lymph node in the neck of at least 15 millimeters.
Many children, especially infants, eventually diagnosed with Kawasaki disease do not exhibit all of the above criteria. In fact, many experts now recommend treating for Kawasaki disease even if only three days of fever have passed and at least three diagnostic criteria are present, especially if other tests reveal abnormalities consistent with Kawasaki disease. In addition, the diagnosis can be made purely by the detection of coronary artery aneurysms in the proper clinical setting.
Treatment
Children with Kawasaki disease should be hospitalized and cared for by a physician who has experience with this disease. When in an academic medical center, care is often shared between pediatric cardiology and pediatric infectious disease specialists (although no specific infectious agent has been identified yet). It is imperative that treatment be started as soon as the diagnosis is made to prevent damage to the coronary arteries.
Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease and is administered in high doses with marked improvement usually noted within 24 hours. If the fever does not respond, an additional dose may have to be considered. IVIG by itself is most useful within the first 7 days of onset of fever, in terms of preventing coronary artery aneurysm.
Salicylate therapy, particularly aspirin, remains an important part of the treatment (though questioned by some) but salicylates alone are not as effective as Intravenous immunoglobulin. Aspirin therapy is started at high doses until the fever subsides, and then is continued at a low dose when the patient returns home, usually for 2 months to prevent blood clots from forming. Except for Kawasaki disease and a few other indications, aspirin is otherwise normally not recommended for children due to its association with Reye’s syndrome.
Corticosteroids have also been used, especially when other treatments fail or symptoms recur, but in a randomized controlled trial, the addition of corticosteroid to immune globulin and aspirin did not improve outcome.
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