Kawasaki disease
Approximately 50 percent of children with Kawasaki disease may develop inflammation of the heart muscle and potentially heart failure, in severe cases.

Kawasaki disease (KD) is an acute, self-limiting, multisystem, inflammatory disease of unknown etiology. It primarily affects infants and young children, usually aged younger than five years old.

Approximately, 50 percent of children may develop inflammation of heart muscle, which is often associated with an abnormally increased heart rate, decreased lower heart chamber functioning, and, in severe cases, the impaired ability of the heart to effectively pump blood to the lungs and the rest of the body (heart failure).

Kawasaki disease is also called:

Why is it called Kawasaki disease?

Kawasaki disease (KD) is named after the Japanese pediatrician Tomisaku Kawasaki, who in 1967 described the first case of the disease.

Originally thought to be a rare disease, KD is the primary cause of acquired heart disease in children in developed countries.

What causes Kawasaki disease?

The exact cause is unknown, but evidence suggests infection or inappropriate immune response to an infection could be the cause of Kawasaki disease.

Another speculation suggests that the disease may be caused by certain toxic substances called bacterial “superantigens,” produced by bacteria, such as streptococci or staphylococci.

They may trigger an exaggerated response of the immune system, resulting in infiltration of blood vessel walls with certain white blood cells, associated blood vessel inflammation (vasculitis), and cardiovascular damage.

Who is at risk of Kawasaki disease?

Risk factors of Kawasaki disease include:

  • Age: 80 to 90 percent of the cases occur in children younger than five years.
  • Gender: Boys are 1.5 times more likely to be at risk than girls.
  • Ethnicity: Children of Asian descent; Japanese and Korean children are at the highest risk.
  • Seasonal: Kids are more likely to get infected in the winter and spring seasons.

What are the signs and symptoms of Kawasaki disease?

Kawasaki disease does not spread from person to person.

The most common signs and symptoms include:

  • A high fever temperature of more than 102.2°F that lasts for five or more days
  • Inflammation of the mucus membranes of the mouth and throat, resulting in dry, red, cracked, swollen lips, and a strawberry-red tongue
  • Red skin rash between the chest and legs and in the genital or groin area
  • Lymphadenopathy (swelling of lymph nodes), especially of the neck
  • Redness and swelling of the hands and feet
  • Redness of the eyes
  • Irritability
  • Diarrhea
  • Vomiting

Is Kawasaki disease life-threatening?

Kawasaki disease can affect the heart within 10 days to 2 weeks after symptom onset.

In a few cases, affected children may develop additional symptoms and complications, such as:

  • Coronary arteritis: Inflammation of arteries transporting blood to the heart.
  • Vasculitis: Inflammation of blood vessels.
  • Aneurysms: Widening or bulging of the coronary artery.
  • Myocarditis: Inflammation of the heart muscles.
  • Pericarditis: Inflammation of the membranous sac surrounding the heart.
  • Aortic or mitral valve insufficiency: Leakage of heart valves.
  • Bilateral conjunctivitis: Inflammation of the white area of the eyes.
  • Desquamation: Peeling of skin on fingers and toes.
  • Arthritis: Inflammation of the joints.
  • Hepatosplenomegaly: Enlargement of the liver and spleen.
  • Aseptic meningitis: Inflammation of the membranes covering the brain.
  • Otitis media: Inflammation of the middle ear.
  • Dysrhythmia: Abnormal heart rhythm.
  • Rupture of an aneurysm.
  • Myocardial infarction (heart attack).

QUESTION

In the U.S., 1 in every 4 deaths is caused by heart disease. See Answer

How is Kawasaki disease diagnosed?

There is no specific, single test to diagnose Kawasaki disease. Diagnosis can be made based on the physical signs and symptoms, and the patient should present with a high fever for more than five days.

Can Kawasaki disease be cured?

Most children recover without serious problems if they receive treatment within 10 days of symptom onset.

Prompt treatment is critical to prevent significant heart problems, which may include:

  • Intravenous immunoglobulin: Antibodies help fight infection and decrease the risk of damage to the coronary arteries.
  • Aspirin: To prevent blood clots.
  • Surgery: In rare cases, coronary artery bypass surgery or heart transplantation is recommended for severe heart involvement.

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Medically Reviewed on 12/1/2021
References
Image Source: iStock Images

Brennan D. Kawasaki disease. WebMD. https://www.webmd.com/children/what-is-kawasaki-disease

American Academy of Pediatrics. Kawasaki disease in infants and young children. https://www.healthychildren.org/English/health-issues/conditions/heart/Pages/Kawasaki-Disease.aspx

National Organization for Rare Disorders. Kawasaki disease. https://rarediseases.org/rare-diseases/kawasaki-disease/

Gordon JB, Kahn AM, Burns JC. When children with Kawasaki disease grow up: Myocardial and vascular complications in adulthood. J Am Coll Cardiol. 2009;54(21):1911-1920. doi:10.1016/j.jacc.2009.04.102