Kawasaki disease

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at May 30, 2016
StartDiseasesKawasaki disease

Kawasaki disease is an uncommon autoimmune disorder in children. It attacks the mid-sized blood vessels and can be dangerous to the entire cardiovascular system. Symptoms of Kawasaki can last for many days and can be severe.

Contents

Definition & Facts 

Kawasaki disease was first discovered in Japan by Dr. Tomisaku Kawasaki in 1961. Researchers do not know how long the disease has existed, there was some research with a preserved heart from the 19th century that showed evidence of Kawasaki. It affects the heart, arterial walls, lymph nodes, and mucous membranes.

The disease is more prevalent in Asian countries such as Japan and Korea. In the United States, approximately 2,000 to 4,000 cases are diagnosed every year. Children under 5 years old are more vulnerable. This disease is a major cause of coronary artery disease in children. There have been cases where older children and even adults have suffered from this autoimmune disorder, but the cases are rare.

Since the whole cardiovascular system is involved in Kawasaki, it cannot be predicted. Doctors say that lingering problems after treatment usually disappear with time. Nevertheless, complications can include heart damage and arterial aneurysms. An aneurysm is a weak spot in the blood vessel that swells and can burst.

In these cases, surgical intervention is needed. The patient may have to have stents in their hearts to prevent blood clots. They may also require a coronary artery bypass graft to reroute the blood around a diseased blood vessel in the heart. On rare occasions, the Kawasaki patient may suffer from a fatal heart attack. Experts say that in these unusual circumstances, the victim may have had a weak heart or vascular system.

Symptoms & Complaints 

There are several symptoms of Kawasaki disease. Children with Kawasaki have a fever that usually lasts 5 days or more. The fever can get up to 104 degrees Fahrenheit or 40 degrees Celsius and will not respond to ibuprofen or acetaminophen. Parents may also notice that their child's eyes are red. This is because of the blood vessels being attacked.

The disease can cause a body rash without blisters around the middle of the body. Not only is a rash present, but the skin may crack and start to peel. It almost gives the child a flushed look. He or she may be too tired to play and will just lie in bed. The tongue and lips can be red, swollen, and cracked. Swelling, redness, and tenderness can also be observed in the hands and feet.

Some other symptoms include abdominal pain, diarrhea, and irritability. The child might also have a cough and runny nose. Because this is an autoimmune disease, the lymphatic system is affected. Children with Kawasaki often complain of swelling and pain in the neck’s lymph nodes. These symptoms may seem scary to parents. There are some children who do not get all the symptoms. 

Causes 

Medical researchers do not know what causes Kawasaki disease; however, they do not believe that it is contagious or hereditary. For some unknown reason, this disease affects more boys than it does girls. There are some theories that say that Kawasaki is due to bacteria or viruses.

There is a correlation between areas where El Nino winds cross and cases of Kawasaki occurring. This leads some researchers to believe that the disease may be environmental. Some genetic research has pointed out risk factors for Kawasaki, such as being male and Asian. Children of African and Caribbean descent are also more susceptible.

Diagnosis & Tests

Since there are no definitive tests for Kawasaki disease, it can be difficult to diagnose. If a child has a fever that will not go down, medical attention is needed. There are some things that parents can do in preparation for their doctor’s appointment. They should make a list of any symptoms and pain that the child is experiencing. The pediatrician will consider how many days the child has been feverish and if there are any of the other symptoms present.

Parents should also make a list of any kind of medication, herbal supplements, or vitamin supplements that their child takes on a regular basis. The doctor also needs to know what immunizations the child has taken and what childhood diseases he or she has already had--especially chickenpox.

Another helpful thing is for them to write down any questions they may have for the doctor. It can be easy to misdiagnose Kawasaki because some of its symptoms are present in influenza and other illnesses. The medical staff may also order regular lab tests such as blood tests and possibly an echocardiogram to examine the extent of damage to the cardiovascular system and to rule out any other type of disorder. 

Treatment & Therapy

When children have been diagnosed with Kawasaki disease, they must be admitted to the hospital. If treatment is not sought as soon as possible, damage to the heart and coronary arteries can happen. Treatment includes high doses of aspirin and IV therapies such as gamma globulin, which is an immune serum found in human plasma. The aspirin treats the fever, swelling, and other symptoms. Within a day, the fever is usually gone and the patient gets a lot better.

After treatment, the affected children can get some bed rest at home and are usually back to regular activities within a few days. Pediatricians and cardiologists recommend that any child who has recovered from Kawasaki disease should have follow-up appointments that include blood tests and an electrocardiogram (EKG).

Studies show that as many as 25% of children who get the standard treatment may still develop coronary artery problems. Some studies have shown positive results when corticosteroids are added to the treatment. There still has to be more testing with this added procedure before it is approved for use.

Prevention & Prophylaxis 

Because medical science still does not know the root causes of Kawasaki disease, no preventive measures are known. Though rates of recurrence are low, the possibility of recurrence still exists, and parents should remain vigilant about seeking checkups for their children if they have been previously diagnosed.