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Autoimmune Conditions

Kawasaki Disease

Other Names: Mucocutaneous Lymph Node Syndrome, Infantile Polyarteritis

Kawasaki's Disease is a rare, childhood autoimmune that attacks the walls of blood vessels causing them to inflame and reduce the amount of blood flow. It can attack any blood vessel: veins, arteries and capillaries. In most cases children can make a full recovery with proper treatment, however Kawasaki's can cause serious complications when main vessels and coronary arteries are affected.

Symptoms of Kawasaki's might include a high fever, swollen lymph nodes in the neck, rashes in the genital area and across the trunk, swollen tongue, red and dry, cracked lips and redness in the eyes. Kawasaki's can also affect the lymph nodes, skin and mucous membranes (mouth, nose and throat).

According to the Mayo Clinic* there are three stages to Kawasaki Disease.

First phase
Signs and symptoms of the first phase may include:

Fever, which often is higher than 101.3 F (38.5 C), and lasts one to two weeks
Extremely red eyes (conjunctivitis) without thick discharge
A rash on the main part of the body (trunk) and in the genital area
Red, dry, cracked lips and an extremely red, swollen tongue ("strawberry" tongue)
Swollen, red skin on the palms of the hands and the soles of the feet
Swollen lymph nodes in the neck and perhaps elsewhere

Second phase
In the second phase of the disease, your child may develop:

Peeling of the skin on the hands and feet, especially the tips of the fingers and toes, often in large sheets
Joint pain
Diarrhea
Vomiting
Abdominal pain

Third phase
In the third phase of the disease, signs and symptoms slowly go away unless complications develop.

Symptoms:

Bright red mouth, also known as strawberry mouth
Fever
General redness and inflammation of the skin
Irritability
Lips that are red and cracked
Red eyes
Skin peeling on fingers, hands,feet or genital area
Swelling in ankles
Swelling in hands and fingers
Swelling in legs
Swollen eyes
Swollen lymph nodes

Diagnosis:

Typically Kawasaki's is diagnosed through a process of eliminating other possible causes of the symptoms, a review of symptoms, medical history and risk factors. Chest X-rays may be performed to help rule out other causes, and a urinalysis may show some abnormalities as well. EKG's and Echocardiograms are used to determine heart function to help in the diagnosis. Blood tests may show:

Elevated C-Reactive Protein (CRP)
Elevated Erythrocyte Sedimentation Rate (ESR)
Low Albumin
Low Total Serum Iron


Treatment:

The goal of treatment is to lower the immediate fever (if present) and lower the inflammation to reduce heart complications and improve the quality of life for the patient. Gamma Globulin infusion is a common form of therapy used to lower the risk of coronary artery problems. Aspirin is also used to prevent blotting.

Close monitoring of the heart is a long-term part of the treatment process to detect any signs of complications as early as possible.

Prognosis:

With early detection and treatment, the progress is very good with most making a full recovery. However, there is a 1% death rate when the condition is not properly treated or complications become sever enough to cause inflammation of the heart muscle (myocarditis), heart valve problems (mitral regurgitation), abnormal heart rhythm (arrhythmia) or inflammation of blood vessels (vasculitis), usually the coronary arteries, which supply blood to the heart. When these complications are not identified early enough to be treated, the prognosis is poorer.

 

 
 
 

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