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Undiagnosed Kawasaki Disease

What is Kawasaki Disease? 

Kawasaki Disease, also called Kawasaki Syndrome or mucocutaneous lymph node syndrome is an illness mainly affecting children under five. It is characterized by inflammation of the blood vessels (‘vasculitis’) and in particular damages the coronary arteries that supply blood to the heart.

 

About 1/3 of untreated children with KD suffer heart damage, making it the most common cause of heart disease acquired in childhood in many countries, including the UK. 

 

Following from its first diagnosis in 1967 in Japan, KD is being diagnosed with increasing frequency in children in Asia, the USA, and Western Europe, and systemic models suggest that by 2030 one in every 1,600 adults in the USA will have suffered from KD.

The cause

The cause of KD remains unknown, but is believed to be due to an infection that all children get in early childhood and to which a minority of children react abnormally. There is good evidence to suggest that these children have an inherited predisposition to KD (and also to developing heart damage once they have KD). Projects therefore investigate the genes controlling the immune system that may be important in determining susceptibility and heart damage. This will tell us not only about the cause of KD, but may also help understand the basis of ischaemic heart disease in adults (heart attacks, angina etc).

The symptoms

Patients usually begin with a high fever lasting at least five days. Symptoms may include red eyes, lips, and mouth; rash; swollen and red hands and feet; and swollen lymph nodes. The disorder affects the mucus membranes, lymph nodes, walls of the blood vessels, and the heart. The most important aspect of the disease is how it effects the heart. The disease can cause inflammation of blood vessels in the coronary arteries, which can lead to aneurysms.

 

If your child has the following symptoms for more than 4 days ask the doctor for a heart scan by a Pediatrician.

  • High fever

  • Red, bloodshot eyes

  • Angry rash

  • Red, cracked lips and strawberry tongue

  • Swollen lymph nodes in the neck

  • Peeling feet and hands

Due to the poor knowledge about KD, many cases are missed during childhood, which can lead to severe cardiovascular complications later in life, including myocardial infarction, congestive heart failure, and sudden death during adulthood.

Difference between “Classic KD” and “Incomplete KD”

KD occurs in two forms: classic and incomplete (“atypical”) cases. Children with classic cases have prolonged fever and at least four of the six clinical findings. Patients with incomplete cases have prolonged fever with fewer than four of the five known clinical findings. If a child has Complete has all the 6 symptoms within 10 days of illness it’s called Complete KD where if 2 or 3 symptoms are seen in the same time period it’s recognized as Incomplete KD.

Classic and incomplete cases share the same laboratory profile and pathologic features. At present, the development of coronary artery abnormalities is the only means by which a patient with an incomplete case can be confirmed to have had KD. However, because early treatment can reduce the prevalence of coronary artery abnormalities, 2 timely diagnosis of either a classic or an incomplete case of KD is essential. The same treatment applies to both types of KD.

Treatment

Immediate treatment is critical to avoid permanent damage to the coronary arteries and heart. Standard treatment includes high doses of Intravenous gamma globulin. The patient’s condition usually greatly improves within 24 hours of treatment.
Radiography (x-ray) of the chest may be used to look for changes in the aorta. Angiography or arteriography is used to help determine the location and appearance of vessels affected by the disease.

Kawasaki disease UK research

Leaflet

We have designed a leaflet with the aim of spreading awareness of Kawasaki Disease. We are hoping to have this leaflet distributed to as many GPs, hospitals and paediatric centres as possible, so that people become familiar with the warning signs of the disease.

If you would like to print a copy and share, please use the link below to access a PDF version.

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