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HOW IS KAWASAKI DISEASE DIAGNOSED?
What does Kawasaki Disease look like and how it is diagnosed?
There is no laboratory test that can diagnose Kawasaki disease with 100% surety. Instead, we use a collection of criteria developed by Dr. Kawasaki and described by the Centres for Disease Control (Table 1). A child must have fever for more than 5 days plus four of five findings from the criteria on physical exam, without evidence of another disease. Lips and mouth are often bright red, as are both eyes, usually without fluid or crusting. The top layer of the tongue peels off, creating the appearance of "strawberry tongue," which is red and glossy. Hands may be swollen and red. Kawasaki disease typically produces a rash as well, which is often worse in the groin area. There can be swollen glands (lymphadenopathy), usually one gland in the neck measuring at least 1.5 cm (normally glands are less than 0.5 cm).
There are other clinical and laboratory findings that can support the diagnosis (Table 2). Children are often extremely irritable over the course of the entire illness. Occasionally the diagnosis of Kawasaki disease is unclear and a spinal tap is done to evaluate for causes of fever; this can show mild inflammation of the spinal fluid (aseptic meningitis). One-third of patients have a temporary joint pain (arthritis), usually in the small joints initially (fingers and toes), with progression to the large weight-bearing joints (knees, wrists, elbows, hips). Many children have diarrhea, nausea, and vomiting. The gall bladder may be large and children may have abdominal pain. The diagnostic criteria in Table 1 should be viewed only as a guideline, since some children develop coronary artery changes without meeting diagnostic criteria, referred to as "atypical Kawasaki disease". A typical disease is more common in infants, who often present with subtle or incomplete findings.
Summary: Symptoms of Kawasaki disease include:
¨ a fever that lasts at least 5 days
¨ red eyes
¨ swollen red lips and tongue
¨ a body rash
¨ swollen feet and hands
¨ swollen lymph nodes
How Is Kawasaki Disease Treated?
Gamma Globulin: The use of intravenous immunoglobulin (IVIG) shortens the acute phase of Kawasaki disease somewhat and prevents much of the coronary damage. This treatment is currently the standard of care in Kawasaki disease, in a dose of 2 g/kg through the vein over 8 to 12 hours. In an NIH study, gamma globulin decreased the number of aneurysms by 3-5 times, when given in the first ten days of illness. For children who are diagnosed after the 10th day and continue to have fever, IVIG still may be helpful. Children who still have fever two days after IVIG may benefit from further treatments with IVIG. Careful monitoring is necessary during the administration of gamma globulin because it rarely can cause an allergic-like reaction.
Aspirin: In the past, aspirin was used for Kawasaki disease because it decreases inflammation and lowers fever, as well as prevents blood clots. Aspirin has never been shown in a study to decrease the risk of aneurysms. High doses of aspirin (20-25 mg/kg/dose every 6 hours) are used in the first few days to control fever. After a child has no fever for 2 or 3 days, the dose is decreased to one that makes the blood "slippery" and less likely to clot, an "antiplatelet" dose (3-5 mg/kg/day). Children continue to take low-dose aspirin through the convalescent phase and then stop unless they have coronary damage.
Treating Children with Aneurysms
Children with coronary aneurysms require long-term therapy to decrease the chance of clotting in the aneurysm. The chance of having a clot in the coronary arteries is greatest after the acute phase, when the platelet count is high and there continues to be inflammation of the blood vessels. Aspirin therapy is the most common treatment for children with aneurysms. If a patient has "giant aneurysms", other medicines are used to thin the blood. Heparin or warfarin (Coumadin) effect proteins in the blood that promote clotting. Treatment with Coumadin requires monitoring with blood tests every month. Many children with giant aneurysms are treated with both Coumadin and aspirin. Low molecular weight heparin is occasionally used instead of Coumadin; this treatment requires injections twice a day under the skin but does not require frequent blood testing. Newer drugs that affect the platelets, such as clopidogrel or Plavix, can also be used together with aspirin, though experience in children is still limited. The medicines used to prevent clotting will depend on the cardiologist's opinions about the risks and benefits of different treatment regimens for the individual child.
Because aspirin is associated with Reye's syndrome, children who take daily aspirin should have yearly influenza vaccines. If the child develops influenza or chickenpox, two viral illnesses especially associated with Reye's syndrome and aspirin, aspirin must be stopped temporarily. Aspirin therapy also should not be given during the six weeks after a chicken pox vaccine. For children with especially large aneurysms for whom aspirin therapy must be interrupted, we can use other anti-platelet agents in the short-term. When there is a blood clot in a coronary aneurysm, either as detected by echocardiography or by symptoms of a heart attack, special treatments are used to prevent further clotting, to dissolve the clot, and to protect the heart muscle. These treatments are identical to those used in adults who have heart attacks.
More about Reye's Syndrome
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