Kawasaki disease — most common in children under 5
KAWASAKI disease, also known as Kawasaki syndrome or mucocutaneous lymph node syndrome, is a rare heart condition which affects several thousand children across the world annually. The condition, according to paediatrician Dr Anona Griffith, is most common in children below five years and is the commonest cause of acquired heart disease in children in developed countries.
“Kawasaki disease is named after Japanese paediatrician Dr Tomisaku Kawasaki, who first documented the features that have become the criteria for diagnosis in 1967. It is characterised by inflammation of blood vessels, mainly medium sized, which means it can affect many organs and manifest itself in many ways,” Dr Griffith said.
Some of the most common areas they affect include the coronary arteries, which supply blood to the heart muscle as well as lymph nodes, skin, and the mucous membranes inside the mouth, nose and throat.
She pointed out that while the cause of Kawasaki disease is unknown, at least one thing is clear — while children of all ethnic backgrounds can be affected by the illness, one group, in particular, those of Asian descent, seems to be more susceptible.
Manifestations of the non-contagious condition may involve any organ system, but the following are not only very common, they form the basis of the criteria for diagnosis and generally appear in two phases.
Phase one
Fever, which is usually high, runs longer than five days and does not respond readily to anti-fever medications. It may be accompanied by other symptoms such as:
•Red eyes without a discharge.
•Dry, red lips, which may be cracked.
•Swelling and redness of the tongue often described as a strawberry tongue.
•Swollen lymph nodes or lumps in the neck area usually more on one side.
•Swelling of the hands and feet, including palms and soles.
• Rash — this may be variable in appearance, like a fine red rash, and usually starts around the genitals, spreading to the rest of the body. The rash in the genital area usually begins to strip after a few days.
Phase two
This stage of the condition, which usually begins within two weeks of the onset of the fever, is generally characterised by the stripping or peeling of the skin around the nails, hands and feet. Other symptoms that may manifest in this phase include:
• Joint pain
• Diarrhoea
• Vomiting
• Abdominal pain
Other non-specific symptoms that may be present include symptoms of a common cold or stomach upset.
“The diagnosis is made based on a combination of information given in a history, as well as blood and urine tests. Imaging studies such as an echocardiogram or ultrasound of the heart are essential in confirming the effect of the disease in the blood vessels of the heart,” Dr Griffith said.
She said that once a diagnosis is made, treatment can begin. One method of treatment is the intravenous immunoglobulins (IVIG).
“IVIG is the recommended treatment for those patients who have met the diagnostic criteria of Kawasaki disease. It is thought to act as an anti-inflammatory agent which acts quickly once administered, being effective in reducing complications involving the heart, especially when used in the first seven to 10 days of illness,” Dr Griffith advised.
Unfortunately, some patients may not respond well to the use of immunoglobulin and are therefore classified as immunoglobulin-resistant. They may benefit from alternative medications, including steroids.
Another treatment method is the use of Aspirin. This, Dr Griffith explains, is another anti-inflammatory agent used in the treatment of Kawasaki disease. It, however, has been shown to be far less effective than IVIG.
Importantly, Dr Griffith advised that while the acute illness is self-limiting, long-term effects include an increased risk for ischaemic heart disease in adulthood. It is therefore recommended that once your child was infected with Kawasaki disease, that routine exams are completed because of the possibility of a long-term heart condition.
