What is Kawasaki Disease?

Introduction of Kawasaki Disease:

Kawasaki disease, also known as mucocutaneous lymph node syndrome, is a rare but serious illness that primarily affects children under the age of five. The exact cause of the disease is unknown, but it is believed to be related to an abnormal immune response to an infection or environmental trigger. Kawasaki disease can cause inflammation in blood vessels throughout the body, leading to a range of symptoms, including high fever, rash, redness and swelling of the hands and feet, swollen lymph nodes, and irritability. If left untreated, Kawasaki disease can lead to serious complications, including damage to the coronary arteries that supply blood to the heart. Prompt diagnosis and treatment with intravenous immunoglobulin and aspirin are essential to reduce the risk of long-term complications and improve outcomes for affected children.

What is Kawasaki Disease?

OVERVIEW

What is Kawasaki disease? Where did it originate from?

Kawasaki disease, also known as mucocutaneous lymph node syndrome, is a rare childhood illness that primarily affects children under the age of 5. It is characterized by a prolonged fever lasting for more than five days, along with other symptoms such as rash, red eyes, red lips and tongue, swollen hands and feet, and swollen lymph nodes.

The exact cause of Kawasaki disease is unknown, but it is thought to be triggered by an infection or an abnormal immune response. The disease was first described by a Japanese pediatrician named Tomisaku Kawasaki in 1967 after he observed a series of cases of children with similar symptoms.

Kawasaki disease was initially thought to be a regional illness that primarily affected children in Japan, but it has since been reported in many other countries around the world. It is now recognized as the most common cause of acquired heart disease in children in developed countries. While the exact origin of Kawasaki disease is unknown, it is thought to have a genetic component, as it is more common in people of Asian and Pacific Islander descent.

CAUSES AND RISK FACTORS

The exact cause of Kawasaki disease is unknown, but it is believed to be related to an abnormal immune response to an infection or environmental trigger. Some possible risk factors that have been associated with the development of Kawasaki disease include:

• Age: Kawasaki disease primarily affects children under the age of five, with the highest incidence occurring in children between the ages of one and two.
• Sex: Boys are slightly more likely to develop Kawasaki disease than girls.
• Ethnicity: Kawasaki disease is more common in people of Asian descent, particularly Japanese.
• Season: Cases of Kawasaki disease tend to be more common in the winter and early spring.
• Genetics: There may be a genetic predisposition to Kawasaki disease, as it tends to run in families.
• Environmental factors: Some studies have suggested that exposure to certain environmental triggers, such as toxins or chemicals, may increase the risk of developing Kawasaki disease.

It is important to note that while these factors may increase the risk of developing Kawasaki disease, most children who develop the disease have no identifiable risk factors.

SIGNS AND SYMPTOMS OF KAWASAKI DISEASE

Kawasaki disease is characterized by a range of signs and symptoms, which can appear in stages over a period of several days. Some of the most common signs and symptoms of Kawasaki disease include:

• High fever that lasts for five or more days
• Red, bloodshot eyes (conjunctivitis)
• Bright red, swollen, and cracked lips
• Strawberry tongue (a swollen, bumpy, and bright red tongue)
• Swollen and irritated throat
• Rash on the trunk and extremities, which may be raised or flat, and may be patchy or splotchy
• Swollen, red, and peeling skin on the hands and feet
• Swollen lymph nodes in the neck and elsewhere in the body
• Irritability and fussiness in young children

If left untreated, Kawasaki disease can lead to serious complications, including damage to the coronary arteries that supply blood to the heart. It is important to seek prompt medical attention if your child is experiencing any of these symptoms, especially if they have a fever that lasts for several days.

HOW TO DIAGNOSE WITH LAB TESTS? 

The diagnosis of Kawasaki disease is based on a combination of clinical findings and laboratory tests. There is no single diagnostic test for Kawasaki disease, so doctors will use a combination of tests to help make a diagnosis.

Some of the laboratory tests commonly used in the diagnosis of Kawasaki disease include:

• Blood tests: These may include tests to check for markers of inflammation, such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), as well as tests to check for abnormalities in the blood cell counts.
• Urine tests: These may be used to check for signs of kidney inflammation or damage.
• Echocardiogram: This is a non-invasive imaging test that uses ultrasound waves to create images of the heart. It may be used to check for abnormalities in the coronary arteries, which can occur in some cases of Kawasaki disease.
• Electrocardiogram (ECG): This is a test that measures the electrical activity of the heart. It may be used to check for abnormalities in heart rhythm or function.

In addition to these tests, doctors will also take a careful medical history and perform a physical exam to look for the characteristic signs and symptoms of Kawasaki disease. If Kawasaki disease is suspected, treatment may be started before all test results are available, in order to reduce the risk of serious complications.

MANAGEMENT AND TREATMENT OF KAWASAKI DISEASE

The treatment for Kawasaki disease involves reducing inflammation in the blood vessels and preventing complications. The following are some of the most common treatments used for Kawasaki disease:

• Intravenous immunoglobulin (IVIG): This is a medication that is given through an IV to help reduce inflammation and boost the immune system. IVIG is the most effective treatment for Kawasaki disease and is usually given as a single dose.
• Aspirin: Low-dose aspirin is often given to help reduce fever and inflammation, and to prevent blood clots from forming in the coronary arteries.
• Corticosteroids: In some cases, corticosteroids may be given to reduce inflammation and prevent complications. However, they are generally reserved for cases where IVIG is not effective or if there is a risk of severe complications.
• Close monitoring: Children with Kawasaki disease are closely monitored for signs of complications, including heart problems. Echocardiograms and other tests may be done to check for abnormalities in the heart or blood vessels.
• Supportive care: Children with Kawasaki disease may need supportive care to manage their symptoms, such as fluids and electrolytes to prevent dehydration, and pain relievers to help manage fever and discomfort.

It is important to begin treatment for Kawasaki disease as soon as possible, ideally within 10 days of the onset of symptoms, in order to reduce the risk of serious complications. With prompt treatment, most children with Kawasaki disease recover fully without any long-term problems. However, in some cases, complications can occur, including damage to the coronary arteries, which can lead to heart disease later in life.

COMPLICATIONS OF KAWASAKI DISEASE

Kawasaki disease can lead to several complications, especially if it is not promptly diagnosed and treated. Some of the most serious complications of Kawasaki disease include:

• Coronary artery aneurysms: These are bulges or weak spots in the walls of the coronary arteries that can occur when inflammation damages the blood vessels. In severe cases, aneurysms can lead to heart attacks or sudden death.
• Coronary artery thrombosis: This occurs when blood clots form in the coronary arteries, which can lead to heart attacks or other serious heart problems.
• Heart valve problems: In some cases, Kawasaki disease can cause inflammation of the heart valves, which can lead to problems with blood flow in the heart.
• Myocarditis: This is inflammation of the heart muscle, which can cause the heart to become weak and less able to pump blood effectively.
• Pericarditis: This is inflammation of the lining around the heart, which can cause chest pain, fever, and other symptoms.
• Arthritis: Some children with Kawasaki disease may develop joint pain, swelling, and stiffness, which can be a sign of arthritis.
• Skin problems: Kawasaki disease can cause skin rashes, peeling, and other skin problems, which can be uncomfortable and unsightly.

It is important to seek prompt medical attention if your child is experiencing symptoms of Kawasaki disease, in order to reduce the risk of complications. With prompt diagnosis and treatment, most children with Kawasaki disease recover fully without any long-term problems. However, some children may require ongoing monitoring and treatment to manage complications.

PREVENTION AND PROGNOSIS OF KAWASAKI DISEASE

There is no known way to prevent Kawasaki disease, as the exact cause of the condition is still not fully understood. However, early diagnosis and prompt treatment can help reduce the risk of complications and improve outcomes.

With appropriate treatment, the prognosis for children with Kawasaki disease is generally good. Most children recover fully without any long-term problems. However, the prognosis may be less favorable for children who experience complications, such as coronary artery damage or heart valve problems.

Regular follow-up care is important for children who have had Kawasaki disease, in order to monitor for potential long-term complications and provide appropriate treatment if necessary. Children who have had Kawasaki disease may also be advised to avoid certain activities, such as contact sports, that could increase the risk of injury to the heart or blood vessels.

It is important to seek medical attention promptly if your child develops symptoms of Kawasaki disease, in order to reduce the risk of complications and improve outcomes.