Infectious Diseases A to Z – Kawasaki Disease

With this new column, Elite Healthcare will compile an index of various infectious diseases, with occasional highlights of emerging conditions.

Kawasaki Disease

General definition and information:

A rare disease that develops during childhood (typically before the age of 5) Kawasaki disease causes the walls of the blood vessels throughout the body to become inflamed. A form of vasculitis (inflammation of the blood vessels), the disease can affect any type of blood vessel in the body, including arteries, veins, and capillaries. Although rarely considered to be serious, Kawasaki disease can lead to long-term heart complications when coronary arteries are impacted. The heart muscle, lining, valves, and the outer membrane around the heart can also become inflamed and arrhythmias or abnormal functioning of heart valves also can occur. Early detection and treatment are important to prevent further complications. Named after Dr. Tomisaku Kawasaki, a Japanese pediatrician, the condition was first recognized as a separate syndrome until 1967.1 The disease produces definitive symptoms over two stages. The first phase is marked by a fever that lasts for at least five days and can linger for up to two weeks.2 Other symptoms include severe redness in the eyes; rash on the stomach, chest, and genitals; red, dry, cracked lips; swollen tongue with a white coating and big red bumps (“strawberry tongue”); sore, irritated throat; swollen palms of the hands and soles of the feet with a purple-red color; and swollen lymph nodes.

Phase two typically begins within two weeks of fever onset, and the skin of the hands and feet may begin to peel in large pieces.2 Children may also experience joint pain, diarrheavomiting, and/or abdominal pain. Because the disease can produce symptoms similar to other common childhood viral and bacterial illnesses, diagnosis typically occurs by checking the symptoms and ruling out other conditions (no single test can detect the condition).2 Kawasaki disease affects children of all races and ages, and both genders, but has been found to occur most often in boys and in children of Asian and Pacific Island descent.3

Causes & Modes of Transmission:

The cause of the disease is unknown, however, there is a belief that the body’s response to a virus or infection combined with genetic factors may be culprits.3 No specific virus or infection has been found, and the role of genetics is not known, however, because the illness frequently occurs in outbreaks within a population (most often during winter or early spring months), an infectious agent (such as a virus) is the likely cause, according to the American Heart Association (AHA). More than one child in a family can develop Kawasaki disease, but the condition is not contagious. Incidence is highest in Japan, and in the United States is the most common cause of acquired heart disease among children. Infants less than 1-year-old are usually the most seriously ill and are at greatest risk for heart complications.1

Treatment Strategies:

Ideally, treatment begins within 10 days of fever onset.2 Typical treatment includes IV doses of gamma globulin and children may also be prescribed a high dose of aspirin to lower the risk of heart complications (or a low dose for a long period of time). It’s considered very important for these children to get their annual flu shot to help prevent viral illnesses while they are taking aspirin because of the risk of Reye syndrome, a rare but serious illness in children who take aspirin during a viral illness.2

Most children see marked improvement after a single treatment with gamma globulin.2 Most kids recover completely overall, but some (especially those who develop heart problems after Kawasaki disease) may need further testing and treatments with a cardiologist.2 Patients should also follow a healthy diet and adopt healthy lifestyle habits to help lower the risk of future heart disease, and children who have had immune globulin should wait 11 months before having the measles and chicken pox vaccines.3

When Kawasaki disease affects the coronary arteries, blood-thinning medicines (eg, warfarin) may be prescribed to help prevent blood clots from forming in the affected arteries. According to the National Heart, Lung, and Blood Institute, a small number of children will not be able to heal their coronary arteries and will likely require routine tests, such as echocardiography, electrocardiogram, or stress test. In rare cases, cardiac catheterization will be required. Even rarer, a child may need to have other procedures or surgery if inflammation narrows his or her coronary arteries and blocks blood flow to the heart.3 Percutaneous coronary intervention, stent placement, or coronary artery bypass grafting may be used. Joining a support group may help parents adjust to caring for a child living with Kawasaki disease.3

Prevention Parameters:

There is no known prevention for Kawasaki disease, according to the AHA, which also suggests that healthcare providers reinforce with parents of children who are living with the condition that nothing could have been done to prevent the disease. Approximately one child in 100 may develop the disease a second time, according to the AHA.

References

  1. Kawasaki disease: complications, treatment, and prevention. AHA. 2017. Accessed online www.heart.org/en/health-topics/kawasaki-disease
  2. Clute J. Kawasaki Disease. KidsHealth. 2014. Accessed online: kidshealth.org/en/parents/kawasaki.html
  3. Kawasaki disease. NIH. No date. Accessed online: www.nhlbi.nih.gov/health-topics/kawasaki-disease

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