Kawasaki disease

Kawasaki disease: Description:Kawasaki diseaseA systemic vasculitis of unknown origin that occurs primarily in children under 8 years of age. Symptoms include a fever lasting more than 5 days, polymorphic rash, erythematous, dry, cracking lips; conjunctival injection, swelling of the hands and feet, irritability, adenopathy, and a perineal desquamative rash. Approximately 20% of untreated patients may develop coronary artery aneurysms. As the child recovers from the illness, thrombocytosis and peeling of the fingertips occurs.Alternative Names: Kawasaki syndrome, mucocutaneous lymph node syndrome.ICD-9-CM: 446.1.Kawasaki disease is a rare childhood disease. It's a form of a disease called vasculitis.In Kawasaki disease, the walls of the blood vessels throughout the body become inflamed. The disease can affect any type of blood vessel in the body, including the arteries, veins, and capillaries.In some cases, Kawasaki disease affects the coronary arteries, which carry oxygen-rich blood to the heart. As a result, a small number of children who have Kawasaki disease may develop serious heart problems.Most children with Kawasaki disease get better and have no long-term effects, even if they do not receive treatment. However, treatment shortens the illness and greatly reduces the chances of having problems from Kawasaki disease. About 20% of children who are not treated will have problems in the arteries that supply blood to the heart (coronary artery disease). Weakened coronary arteries may get enlarged or can narrow or develop blood clots. In very rare cases, this can lead to a heart attack.The risk of heart attack is greatest within 1 year after the illness. However, studies in Japan indicate that children who develop coronary artery damage are more prone to having a heart attack as young adults.If your child has symptoms of Kawasaki disease, seek medical attention immediately. Early diagnosis and treatment often prevent life-threatening problems. If your child has been diagnosed with Kawasaki disease, it is important to follow up with your doctor. An echocardiogram may be done at 2 to 3 weeks and 6 to 8 weeks after the illness to check for aneurysms.Seek emergency care if your child has been diagnosed with heart problems (coronary artery problems) and has chest pain, is short of breath, faints, or has a lack of energy.Kawasaki disease often begins with a high and persistent fever greater than 102 °F, often as high as 104°F. A persistent fever lasting at least 5 days is considered a classic sign. The fever may last for up to 2 weeks and does not usually go away with normal doses of acetaminophen (Tylenol) or ibuprofen.Symptoms:Symptoms often include:Swollen hands and feet.
  • Skin rashes on the middle of the body, NOT blister-like.
  • Peeling skin in the genital area, hands, and feet (especially around the nails, palms, and soles).
  • Swollen lymph nodes (frequently only one lymph node is swollen), particularly in the neck area.
  • Joint pain and swelling, frequently one both sides of the body.
  • Extremely bloodshot or red eyes (without pus or drainage).
  • Bright red, chapped, or cracked lips.
  • Red mucous membranes in the mouth.
  • Strawberry tongue, white coating on the tongue, or prominent red bumps on the back of the tongue.
  • Red palms of the hands and the soles of the feet.
  • Irritability.
  • Diarrhea, vomiting, and abdominal pain.
  • Cough and runny nose.
Causes and Risk Factors:The cause of Kawasaki disease isn't known. The body's response to a virus or infection combined with genetic factors may cause the disease. However, no specific virus or infection has been found, and the role of genetics isn't known.Kawasaki disease can't be passed from one child to another. Your child won't get it from close contact with a child who has the disease. Also, if your child has the disease, he or she can't pass it on to another child.Kawasaki disease, which afflicts children, may be caused by a common bacterium and may be spread by the house-dust mite, a tiny creature less than a millimeter long, Japanese researchers have reported.Kawasaki disease generally affects children under the age of 5. Though most recover completely, the disease can sometimes damage the heart, liver and brain, or cause sudden death from a burst artery.The suspect bacterium is called Propionibacterium acnes. It is anaerobic, growing only in the absence of oxygen. The bacterium is commonly found on the skin of adults and older children, but much less frequently on the skin of young children.An American expert said, in calling the report ''fascinating,'' that he would never have guessed that P. acnes might cause the disease. The expert said it was unclear why the disease, if it is caused by the bacterium, does not seem to respond to penicillin or other antibiotics.If further studies confirm the bacterium as the cause of the disease, doctors presumably could develop a diagnostic test.Although the bacterium is a common one, the researchers reported that it behaved uncommonly in the laboratory. They said that it took from three to four weeks, compared to the usual period of several days, to culture the organism by standard microbiological techniques.In Japan, where Kawasaki disease is most prevalent, house-dust mites have been increasing with changes in housing styles. The fact that the strains of P. acnes isolated from the lymph node, patients' blood and mites were identical was considered strong evidence suggesting the role of P. acnes in causing Kawasaki disease.Further, when the researchers injected the organism into guinea pigs and rabbits, they found that it produced damage similar to that seen in humans with Kawasaki disease.The researchers discounted the possibility that the bacterium was a contaminant because of the way they collected their samples and obtained the scientific evidence.Risk Factors:Three things are known to increase your child's risk of developing Kawasaki disease, including:Age. Children between the ages of 2 and 5 years old are most at risk of Kawasaki disease.
  • Sex. Boys are more likely than girls are to develop Kawasaki disease.
  • Ethnicity. People living in Asia or of Asian descent, such as Japanese or Chinese, have higher rates of Kawasaki disease
Diagnosis:There is no specific test for Kawasaki disease, so diagnosing the disease can be difficult. Doctors use specific criteria developed by the American Heart Association (AHA) to diagnose Kawasaki disease.Routine lab tests may be done to rule out Kawasaki disease. These tests may reveal another condition, usually a bacterial or viral infection. The most common tests are:A complete blood count: a combination of the following determinations: red blood cell count, white blood cell count, erythrocyte indices, hematocrit, differential blood count, and sometimes platelet count.
  • Urinalysis: This test can help determine whether illness is present.
  • Sedimentation rate: This blood test can help determine whether inflammation is present.
Treatment:If your doctor suspects Kawasaki disease, he or she may order a C-reactive protein blood test to look for inflammation and an echocardiogram to check for heart problems.Children who have had Kawasaki disease need to have a follow-up exam at 2 to 3 weeks and at 2 to 3 months after the initial disease. Tests done during the exam will include:Blood tests to monitor how well the medications are working.
  • An echocardiogram to check heart functioning and to detect aneurysms of the coronary arteries.On rare occasions, other tests are used to see whether there is damage to the heart.
  • An electrocardiogram (ECG or EKG) may be used to identify problems with the heart's electrical function.
  • A chest X-ray helps check heart size and the chance of heart failure.
  • A coronary angiogram may be done if the echocardiogram reveals problems with the coronary arteries.
  • A stress test may be done to see how the heart responds to exercise.
Kawasaki disease is treated with medications. Long-term care may be needed and might include continued medication, limited physical activity, and repeated testing. Early treatment of Kawasaki disease prevents most blood vessel and heart damage. If children with Kawasaki disease are hospitalized and treated within 10 days after the first signs of illness, the risk of heart disease and aneurysms greatly decreases.Kawasaki disease is best treated by a pediatrician or cardiologist.Medications: Medications used to treat Kawasaki disease include intravenous immunoglobulin (IVIG), aspirin, and possibly anticoagulants.Intravenous immunoglobulin (IVIG).Intravenous immunoglobulin (IVIG) is used to reduce inflammation of blood vessels. IVIG usually is given in 1 dose through a vein, over 8 to 12 hours. The treatment usually requires that the child stay in the hospital for at least 24 hours. After IVIG treatment, the illness usually improves very quickly. IVIG works best if it is given before the tenth day of fever. Occasionally, a second dose of IVIG is needed because the first dose did not reduce the fever and inflammation.Aspirin: At first, high-dose aspirin is used to relieve inflammation and fever. If there are no complications, smaller daily doses are then given for 2 to 3 months to lower the risk of dangerous blood clots. Because of the risk of Reye's syndrome, aspirin should be given only under the guidance of a doctor. If the child is exposed to or develops chickenpox or flu (influenza) while taking aspirin, talk with a doctor right away. For more information, see the topic Reye's Syndrome.Anticoagulants: Anticoagulants may be used if the child has a large aneurysm. Anticoagulants help prevent new blood clots.After initial treatment, the child's fever and inflammation should improve, but the child may still be irritable, not have an appetite, and not be very active. The child may also develop deep lines across his or her fingernails (Beau's lines). It may be several weeks before the child feels completely well.The medical management of Kawasaki disease primarily involves the use of gamma globulin. Although some have suggested that aspirin is no longer needed, most use high-dose aspirin for a variable period of time, followed by lower-dose aspirin for its antiplatelet effects.Immune globulin, intravenous (Carimune, Gammagard S/D, Gammar-P. Gamunex. Polygam S/D).
  • Aspirin (Anacin, Ascriptin, Bayer Aspirin, Bayer Buffered Aspirin).
Note: The following drugs and medications are in some way related to, or used in the treatment. This service should be used as a supplement to, and NOT a substitute for, the expertise, skill, knowledge and judgment of healthcare practitioners.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care. 

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