Kawasaki Disease

(Also known as mucocutaneous lymph node syndrome)

Kawasaki disease is a rare disease that causes inflammation or swelling of the blood vessels throughout the body. It is also known as mucocutaneous lymph node syndrome and the cause is not fully understood.

The condition mainly affects children under five-years-old. 

The symptoms (such as high fever and peeling of the skin) are like those of an infection, so a bacterium or a virus may be responsible.

The complications of Kawasaki disease are mainly related to the heart. These generally relate to either focal enlargement of portions of the arteries (aneurysms) or to narrowing of the blood vessels between aneurysms (stenoses). When these complications occur, they need to be closely followed as they can lead to serious adverse consequences in adult life if not managed properly.

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Diagnosis

A cardiologist will carry out a full medical examination, arrange for an electrocardiogram (ECG), a chest X-ray, an echocardiogram, and possibly blood tests. Other tests, including magnetic resonance imaging (MRI), cardiopulmonary treadmill testing (metabolic cart), transesophageal echocardiogram, and heart catheterization may be performed.

Treatments

Regular follow up and testing will assist in detecting early changes in cardiac function and allow for the best possible treatment outcomes for patients with cardiac complications because of Kawasaki disease. Several patients are followed at regular intervals and require no intervention. Others may require more extensive cardiac treatments, including anticoagulation to prevent clot (thrombus) formation within aneurysms. Occasionally, cardiac surgery or angioplasty is necessary to deal with the effect of blood flow restriction through narrowed (stenotic) arteries.