Kawasaki Disease

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Axillary aneurysm in Kawasaki disease

What is Kawasaki Disease?

Kawasaki Disease is an autoimmune disorder wherein the sufferer’s blood vessels are inflamed. This condition is rare but it mostly affects infants and children. Since the inflammation of blood vessels occurs all throughout the body, not only one organ system is affected. The main problem is the cardiovascular system since the blood vessels are inflamed. Its other name, mucocutaneous lymp node syndrome, suggests that the mucus membranes, skin, and lymph nodes are also affected.

The disease is named after Japanese pediatrician Tomisaku Kawasaki who discovered this case in 50 patients. The first victim was a four-year-old child who presented with fever and rashes. That was in January 1961. In 1967, the first report about KD was written in Japanese. When the disease has spread to Northern America, an English report was written by Kasawaki in 1974. [1]

Kawasaki Disease Causes

Kawasaki Disease has an idiopathic cause, meaning the exact cause is unknown or obscure. It is somehow pointed to bacterial or viral cause, though the causative agent is not identified. Hereditary genes may also be a factor in acquiring this disease. [2]

Risk Factors

Though the exact cause of KD is not known, physicians and scientists have observed that the enumerated risk factors below are common in the patients who have been diagnosed to have KD. [3]

  • Below 5 years of age
  • More cases in males than in females
  • Asian descent, particularly Japanese and Korean

Signs and Symptoms of Kawasaki Disease

Below are two illustrations that show you how painful it is for a child (or an adult) to have a Kawasaki disease.

Kawasaki Disease symptoms

Picture 1 :  Kawasaki disease signs and symptoms

Source: physio-pedia.com

 Kawasaki Disease signs and symptoms pictures

Picture 2 : Signs of Kawasaki Disease

(A) Conjunctival injection.
(B) Strawberry tongue.
(C) Rashes starting from the genital area.
(D) Edematous erythema of the hands.
(E) Edematous erythema of the feet.
(F) Desquamation or peeling off of fingers.
(G) Redness and induration around the injection site of Bacillus Calmette-Guerin (BCG).
(H) Erythematous desquamation around the anus.

Source: eymj.org

A person with Kawasaki disease does not only suffer for days. They suffer for approximately six weeks, depending on the progress of the treatment. The signs and symptoms experienced by a KD patient are subdivided into three phases namely, acute, subacute, and convalescent phases. Let us tackle each phase with the corresponding signs and symptoms that are expected in a KD patient for each week.

Kawasaki Disease clinical manifestations

Picture 3 : Kawasaki Disease clinical manifestations

Source: Medscape

Phase 1: Acute (Weeks 1-2)

Fever

The foremost sign in a KD patient is a high fever ranging from 38C (100.4F) to 40C (104F). This lasts for 5 days or more. This fever cannot be treated by the mainstream paracetamol or ibuprofen, which should at least lower the temperature. But in this case of KD patients, these become ineffective. [4]

Conjunctival Injection

This may be likened to conjunctivitis because of the eye color KD may cause. Eyes swell and they appear reddish. But unlike conjunctivitis, conjunctival injection is painless and it does not cause excessive tearing.

Strawberry Tongue

In a patient with KD, even the tongue is inflamed. It appears red, swollen, and with lumps, likened to a strawberry. Along with that the lips are dry and cracked.

Swollen Lymph Glands

If you notice a tender lump on the patient’s neck, that’s the swollen lymph glands. They swell because they overwork, trying to defend our body from infection.

Rashes

The rashes start in the perineum and spreads to the trunk, back, extremities, and face [4].

Swollen Hands and Feet

A patient with KD has edematous hands and feet. They appear reddish and they are painful to touch. Imagine the pain that a child (or adult) experiences. Do not urge them to keep on moving because even their extremities cause them pain.

Phase 2: Subacute (Weeks 2-4)

This is the stage where the symptoms in the acute phase are very slowly disappearing, though the patient is still in pain. This is also the critical phase where complications are likely to occur, so monitor the patient very closely. The following will be observed in KD patients under Phase 2: [4]

  • Headache
  • Jaundice (yellowish color of the skin and eyes)
  • Desquamation or peeling of hands and feet
  • Abdominal pain
  • Nausea and vomiting
  • Diarrhea
  • Pyuria (urine with pus)
  • Arthritis (inflammation of joints)
  • Lack of energy

Phase 3: Convalescent (Weeks 4-6)

This is the phase where the signs and symptoms subside. The patient feels better compared to the previous stages, however he/she may still feel sick because of lack of energy. Imagine everything that the patient has gone through. So if you are the one taking care of a KD patient, you must be patient enough to cater his/her needs.

Is Kawasaki Disease Contagious?

No, it is not contagious. There is no scientific evidence that KD can be transmitted from one person to another. [2]

Diagnostic Criteria of Kawasaki Disease

The table below serves as a guide to physicians to determine whether or not their patient’s case is Kawasaki Disease.

Kawasaki Disease diagnositc criteria

Differential Diagnosis

A prompt diagnosis is needed by patients who show signs and symptoms of Kawasaki disease. Delay in providing treatment in patients with positive KD can cause complications, particularly in the cardiovascular system, which may lead to the patient’s death.

Kawasaki Disease differential diagnosis

Source: physio-pedia.com

Treatment for Kawasaki Disease

A pediatric cardiologist might be your best option with regards to who gives the proper treatment. Kawasaki disease should be treated promptly in order to relieve the painful symptoms as soon as possible, thereby alleviating the patient’s suffering, and to prevent further complications such as aneurysm. The standard treatment is discussed below.

Intravenous Immunoglobulin (IVIG)

Intravenous means the medication is administered via a vein. So the patient needs to be hospitalized. An immunoglobulin is given to minimize the inflammation along the blood vessel walls. It is a medication containing antibodies which fight off infection [5]. Administration of IVIG before the 10th day of fever steadfastly improves the patient’s condition. Usually the first dose is enough. But if the fever and inflammation does not improve, a second dose will be administered [6].

If a patient has undergone this treatment, his/her vaccination on chicken pox and measles, mumps, rubella (MMR) must be delayed for up to 11 months because IVIG decreases their effectiveness [6].

Aspirin

Initially, aspirin is given to KD patients in high doses because as discussed earlier, paracetamol and ibuprofen can’t cure the fever. Furthermore, aspirin has an antipyretic (anti-fever), anti-inflammatory, and analgesic (pain reliever) effect [5], which are drug properties that are certainly needed by patients suffering from KD.

After the fever is gone, aspirin is given in low doses because of its antiplatelet property. This prevents blood clots from forming, which may stick to the inflamed blood vessels and will soon accumulate, then it will travel into the major arteries, which will lead to coronary problems.

Anticoagulants

This may be prescribed by the doctor to prevent further formation of blood clot. [6]

Follow Up Treatment

Follow up treatment is concentrated on the heart. If the coronary arteries were inflicted during the course of the disease, ongoing and long term blood thinning medication may be prescribed by the physician. [7]

Cardiac tests such as echocardiogram, electrocardiogram, and stress test are performed to ensure the optimal status of the heart.

Complications

Even with treatment, complications of Kawasaki disease may be inevitable because blood vessels are involved. Blood vesssels run all throughout our body and if it functions abnormally, our entire body may be in danger, particularly our heart.

The most common complication is aneurysm. Since the blood vessels are inflamed, the arteries may become weak overtime. If this happens, it “loosens” and protrudes outward of the blood vessel. Blood clots may lodge onto it and accumulate. As the clot becomes thicker, it may obstruct the blood flow, decreasing the blood supply to the heart. It may also travel onto the major arteries and cause blockage, killing the muscle cells. When the myocardium (heart muscle) becomes deprived of oxygen (which the blood carries), myocardial infarction (heart attack) happens. [8]

Axillary aneurysm in Kawasaki disease

Picture : Axillary aneurysm in Kawasaki disease.

Image Source: kawasakidiseaseindia.org

Coronary artery aneurysm and thrombosis

Picture : Coronary artery aneurysm and thrombosis.

Source: kawasakidiseaseindia.org

Kawasaki Disease Prognosis

Kawasaki disease is a self-limiting disease, meaning the patient will recover as the time passes by. However, serious cardiac complications are more likely to occur if it is left untreated. You might regret it if you do not take your child immediately to the hospital because with prompt and early treatment, the signs and symptoms manifested could go away quickly. Though all KD patients are prone to future complications, those who underwent treatment are less likely to be affected. [9]

References

  1. http://www.pediatricsdigest.mobi/content/106/2/e27.full
  2. http://www.nhlbi.nih.gov/health/health-topics/topics/kd/causes.html
  3. http://www.mayoclinic.com/health/kawasaki-disease/DS00576/DSECTION=risk-factors
  4. http://www.nhs.uk/Conditions/Kawasaki-disease/Pages/Symptoms.aspx
  5. http://www.nhs.uk/Conditions/Kawasaki-disease/Pages/Treatment.aspx
  6. http://www.webmd.com/heart-disease/tc/kawasaki-disease-treatment-overview
  7. http://www.nhlbi.nih.gov/health/health-topics/topics/kd/treatment.html
  8. http://www.nhs.uk/Conditions/Kawasaki-disease/Pages/Complications.aspx
  9. http://www.news-medical.net/health/Kawasaki-Disease-Prognosis.aspx

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