Kawasaki Disease

Symptoms Of Kawasaki Disease

Kawasaki disease (KD) generally affects children between the ages of 0-5 years. The signs and symptoms of Kawasaki disease usually occur in three phases. Initial symptoms of KD include:

Phase one (acute)

• High fever (102.2 F or 39 C)

• Conjunctivitis (bloodshot eyes) without a heavy discharge

• A rash on the trunk of the body and in the genital area

• Red, dry, and cracked, lips

• Strawberry tongue (tongue appears red, swollen, and covered with a bumpy texture)

• Red, swollen skin on the palms of the hands and soles of the feet

• Cervical lymphadenopathy (neck lymph nodes that are swollen)

Phase two (subacute)

• Peeling of the skin on the hands and feet

• Joint pain or discomfort

• Diarrhea

• Vomiting

• Stomachache

Phase three (convalescent)

• Symptoms usually fade in this phase, unless complications arise.

• However, fatigue, irritability, and low energy can persist for up to eight weeks.

Causes Of Kawasaki Disease

• Kawasaki disease involves a likely infection that enters through breathing. 

• This triggers a response causing inflammation, involving immune cells and chemicals.

• This can harm the heart and blood vessels, forming weak spots that might become aneurysms (bulges in blood vessel walls).

• While the exact cause of Kawasaki disease remains unknown, certain risk factors have been associated with an increased likelihood of developing the condition which are discussed below.

Risk Factors of Kawasaki Disease

The factors that can lead to Kawasaki disease include:

1. Age

• Children under the age of 5 are most vulnerable to Kawasaki disease.
  
  

2. Gender

• Boys are more commonly affected by Kawasaki disease than girls. 
  
  

3. Ethnicity

• Kawasaki disease is more commonly reported in individuals of Asian descent, particularly Japanese and Korean ethnicity. 

4. Family history

• Children whose parents have had Kawasaki disease are two times more likely to develop the disorder than those without. 
  
  

5. Fever

• A fever lasting more than 8 days is a key risk factor for Kawasaki disease, as it may trigger an overactive immune response causing inflammation.
  
  

6. Seasonal variation

• Kawasaki disease cases often exhibit seasonal patterns, with a higher incidence reported in late winter and early spring. 

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Diagnosis Of Kawasaki Disease

Kawasaki disease is diagnosed when a patient presents with a persistent fever lasting more than 5 days, along with at least four of the following five clinical signs:

Conjunctival Involvement

• Redness affecting both eyes (bilateral)

• No associated pain (painless)

• No discharge (nonexudative)

Lymph Node Enlargement

• Swollen cervical lymph nodes, typically larger than 1.5 cm

• Usually affects only one side (unilateral)

Skin Rash

• Frequently appears as a red, raised rash (maculopapular)

Changes in Extremities

• Acute Phase: Redness and swelling of the hands and/or feet

• Subacute Phase: Peeling of the skin around the nails (periungual desquamation)

Mucosal Alterations

• Red, dry, and cracked lips

• "Strawberry tongue," is characterized by a red tongue with enlarged taste buds

• Widespread redness in the mouth or throat (diffuse erythema of the oral mucosa or oropharynx)

Other diagnostic approaches include:

1. Imaging tests

• Echocardiogram: Kawasaki Disease can result in severe heart problems. This test uses ultrasound images to determine how well the heart is performing and can aid in diagnosing cardiac issues.

• Electrocardiogram (ECG): An ECG is a gadget that records electrical activity in the heart. It is performed in Kawasaki disease to monitor for potential heart involvement and detect any abnormal heart rhythms or changes in heart function.

2. Blood tests

• Complete blood cell count (CBC): Kawasaki's disease is identified by low red blood cell count, inflammation, and elevated levels of white blood cells. 
  
  

• Erythrocyte sedimentation rate (ESR): ESR can assist evaluate whether or not inflammation is present. ESR levels in the blood are high in the case of Kawasaki disease.
  
  

• C-reactive protein (CRP): In response to inflammation, your liver creates C-reactive protein, which is then released into the bloodstream. High CRP levels in the blood can be the reason for Kawasaki disease.
  
  

• Serum electrolytes: This test detects abnormalities in blood components such as low sodium, low serum protein, and albumin which can be seen in Kawasaki disease.
  
  

• Liver function test (LFT): Evaluating liver enzymes in Kawasaki disease helps assess the potential involvement of the liver due to inflammation and aids in monitoring the disease's impact on the body's organs.

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4. Urinalysis

• It is conducted in Kawasaki disease to assess kidney function and detect any abnormalities or signs of inflammation.

5. Cerebrospinal fluid evaluation

Typically, increased lymphocytes (pleocytosis) with normal protein and glucose levels are observed in the cerebrospinal fluid in the case of Kawasaki disease.

Prevention of Kawasaki Disease

Currently, there's no known way to prevent Kawasaki disease. However, there are certain things you can do that will ensure a healthy lifestyle and can help manage or prevent the disease. These include:

1. Maintain Good Hygiene

• Encourage regular handwashing, especially before meals and after using the restroom. 

• This helps reduce the risk of infections that may trigger Kawasaki disease or worsen its symptoms.

2. Stay Up-To-Date With Vaccinations

• Ensuring that your child is up-to-date with their recommended vaccinations can help prevent certain infections that have been associated with Kawasaki disease. 

• However, it's important to note that the exact relationship between vaccines and Kawasaki disease is still under study.

Specialist To Visit

Specialists that can help to treat Kawasaki disease include:

• General physician

• Pediatrician

• Pediatric cardiologist

• Pediatric rheumatologist

• Pediatric infectious diseases specialist

A general physician can assist by recognizing early symptoms of Kawasaki disease, and referring the patient to a specialist for diagnosis and treatment.

A pediatrician is a child specialist who recognizes and diagnoses Kawasaki disease early, initiating appropriate treatment.

A pediatric cardiologist treats the potential heart complications that can arise from Kawasaki disease.

A pediatric infectious disease specialist is responsible for diagnosing and treating a variety of infections. They ensure appropriate management to prevent potential complications and long-term effects of Kawasaki disease.

A pediatric rheumatologist can assist in managing the inflammatory response seen in Kawasaki disease, providing specialized care to alleviate symptoms and prevent complications.

If your child shows symptoms of Kawasaki disease, such as cracked and red lips, swelling, and redness in certain areas, and a persistent high fever that does not improve with common medications, it is important to seek medical attention from a specialist.
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Treatment Of Kawasaki Disease

The treatment of Kawasaki disease typically involves a combination of medications aimed at reducing inflammation and preventing complications. The primary treatment options include:

• Intravenous immunoglobulin (IVIG): IVIG is a concentrated dose of antibodies obtained from donated blood plasma. It is given through a vein (intravenously) and works to decrease inflammation in the blood vessels. 

• Aspirin: High-dose aspirin is usually given during the acute phase of Kawasaki disease to reduce inflammation and decrease the risk of blood clot formation. Once the fever subsides, the dosage of aspirin is typically reduced to a low dose.

• Anticoagulants: These are blood thinners that may be prescribed if there is a high risk of blood clot formation. Heparin is the most commonly used drug.

Note: Ciclosporine A and methotrexate are potential treatments for Kawasaki disease, but only cyclosporine A is occasionally used, while methotrexate lacks clear AHA recommendations due to limited understanding.

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Home Care for Kawasaki Disease

While Kawasaki disease requires medical treatment and supervision by healthcare professionals, there are some measures you can take at home to support your child's comfort and recovery. Here are some home care tips for Kawasaki disease: 

1. Provide comfort measures

• Help your child feel more comfortable by providing a cool, quiet, and restful environment. 

• Dress them in loose, comfortable clothing and use lightweight bedding. 

• Keep the room well-ventilated and at a comfortable temperature.

2. Keep them hydrated

• Encourage your child to drink plenty of fluids to stay hydrated. Offer water, diluted fruit juices, or electrolyte solutions. 

• If your child has a decreased appetite, offer small, frequent meals and snacks.

3. Give rest and activity importance

• Rest is essential for recovery, so ensure your child gets adequate sleep and avoids strenuous activities. 

• However, gentle physical activity, such as short walks or light play, can be beneficial as tolerated and approved by the doctor.

4. Focus on skincare

• If your child's skin starts peeling on the hands and feet during recovery, you can use moisturizers to help with dryness. 

• This will soothe the discomfort and improve skin healing.

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Complications Of Kawasaki Disease

Kawasaki disease, if left untreated or inadequately managed, can lead to various complications, particularly affecting the coronary arteries and the heart. Some of the potential complications include:

1. Heart complications

• Coronary artery abnormalities: This includes the formation of aneurysms, which are weakened areas in the walls of the coronary arteries.

• Myocarditis: Kawasaki disease can cause inflammation of the heart muscle (myocarditis), leading to reduced heart function. 

• Heart valve problems: In some cases, Kawasaki disease can affect the heart valves, leading to valvular regurgitation (leaking valves) or stenosis (narrowing of the valves). 

• Pericarditis: Kawasaki disease can cause inflammation of the pericardium, which is the thin sac that surrounds the heart. This condition is known as pericarditis.

• Blood vessel inflammation: In addition to the coronary arteries, Kawasaki disease can affect blood vessels in other parts of the body, leading to inflammation and potential complications in various organs, including the kidneys, liver, and lungs.

2. Neurological complications

• These include irritability, headache, seizures, and changes in consciousness. Other rare complications include facial paralysis and stroke.

3. Renal complications

• It can involve inflammation of the kidneys, leading to abnormal kidney function and sometimes causing protein or blood in the urine.

4. Hematological complications

• These include decreased platelet levels (thrombocytopenia) or elevated white blood cell counts. 

Alternative Therapies For Kawasaki Disease

Certain complementary therapies have shown good results along with conservative treatment in managing the symptoms of Kawasaki disease. These include:

I. Corticosteroid therapy

• These can be used for persistent fever or inflammation despite IVIG treatment. Methylprednisolone is used in case of severe inflammation.

II. Chinese herbal medicine

• It is a plant-based medicine that has been studied for the treatment of Kawasaki disease.

•  It can be used as part of conventional therapy. 

• Though it is safe, more study is required to identify its exact efficacy.

Living With Kawasaki Disease

Though Kawasaki disease is an acute infection, kids who suffer from it may require lifelong monitoring. The American Heart Association (AHA) recommendations for the monitoring of Kawasaki disease include:

Baseline and follow-up echocardiography

• Initial echocardiography is recommended during the acute phase.

• Repeat echocardiograms are advised at week 2 and weeks 6–8 after onset.

• More frequent imaging is needed for patients with high-risk factors or slow recovery.
  
  

In low-risk children

• Follow-up for at least 10–20 years, even without coronary abnormalities.

In moderate-risk children

• If the aneurysm regresses by weeks 6–8, treat it as low-risk.

• Cardiac evaluations every 3–5 years are advised.

In high-risk children

• High chance of coronary artery stenosis (narrowing or blockage of the coronary artery, which is the main artery that supplies blood to the heart) progression.

• Comprehensive cardiac exams twice a year; yearly stress tests and perfusion studies are suggested.

• Physical activity should be limited based on the risk of bleeding.

• Invasive tests like angiography can be performed if needed.