Close
Close

Kawasaki syndrome

Kawasaki disease, Lymph node syndrome, Mucocutaneous lymph node syndrome

Overview

Kawasaki syndrome is a rare childhood disease in which medium-sized blood vessels throughout the body become inflamed. It is largely seen in children under five years of age. It affects many organ systems, mainly those including the blood vessels, skin, mucous membranes, and lymph nodes. Its rarest but most serious effect is on the heart, where it can cause fatal coronary artery aneurysms in untreated children. Without treatment, mortality may approach 1%, usually within six weeks of onset. With treatment, the mortality rate is 0.17% in the U.S.

Symptoms - Kawasaki syndrome

Symptoms include:

  • High and persistent fever (lasting longer than 5 days)
  • Rash (nonspecific, characteristically located on the trunk but may spread to involve the face, extremities, and perineum). 
  • Swelling of the hands and feet
  • Irritation and redness of the whites of the eyes
  • Swollen lymph glands and neck
  • Irritation and inflammation of the mouth, lips and throat
  • Heart complications (observed in as many as one in four children)
  • Joint pain, diarrhoea and vomiting have also been reported

Causes - Kawasaki syndrome

The cause of Kawasaki disease is unknown. Because the illness frequently occurs in outbreaks within a population, an infectious agent (such as a virus) is the likely cause. Some researchers have suggested that a wind-borne pathogen could be responsible; winds blowing from central Asia have been found to correlate with Kawasaki disease cases in Japan, Hawaii, and San Diego.

A genetic predisposition for the syndrome is likely. Japanese children, for example, are more likely than other children to manifest the disease.  Kawasaki syndrome has also been known to affect more than one child in a family.

Prevention - Kawasaki syndrome

Cannot be  prevented.  

Diagnosis - Kawasaki syndrome

Kawasaki disease can look similar to other common childhood viral and bacterial illnesses. No single test can detect Kawasaki disease, so doctors usually diagnose it by checking the symptoms and ruling out other conditions. If Kawasaki disease is suspected, the doctor may order tests to monitor heart function (such as an echocardiogram) and might take blood and urine (pee) samples to rule out other conditions, such as scarlet fever or measles. 

Prognosis - Kawasaki syndrome

With early treatment, rapid recovery from the acute symptoms can be expected, and the risk of coronary artery aneurysms is greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited (i.e. the patient will recover eventually), but the risk of coronary artery involvement is much greater. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every one or two years to screen for progression of cardiac involvement.

A relapse of symptoms may occur soon after initial treatment with IVIG. This usually requires rehospitalization and retreatment. Treatment with IVIG can cause allergic and nonallergic acute reactions, aseptic meningitis, fluid overload and, rarely, other serious reactions.

Treatment - Kawasaki syndrome

Treatment should be started as soon as the diagnosis is made to prevent damage to the coronary arteries. Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease and is administered in high doses with marked improvement usually noted within 24 hours. IVIG by itself is most useful within the first seven days of onset of fever, in terms of preventing coronary artery aneurysm.

A child may also be given a high dose of aspirin, to lower the risk of heart problems. Once the fever subsides, aspirin may be administered at a low dose, usually for two months to prevent blood clots from forming. Because of the risk of Reye syndrome, vaccinations against varicella and influenza are required.

 

Resources - Kawasaki syndrome

https://www.heart.org/en/health-topics/kawasaki-disease

https://kidshealth.org/en/parents/kawasaki.html

 

Videos
by Abidemi Uruejoma
457 views
Research Publications