Disease: Kawasaki's Disease

    Kawasaki's disease facts

    • Kawasaki's disease is a syndrome of unknown cause that mainly strikes young children.
    • Signs of the disease include fever and redness of the eyes, hands, feet, mouth, and tongue.
    • The disease can be treated with high doses of aspirin (salicylic acid) and gammaglobulin.
    • Kawasaki's disease usually resolves on its own within a month or two.
    • Some children with Kawasaki's disease suffer damage to the coronary arteries.

    What is Kawasaki's disease, and how is it diagnosed?

    Kawasaki's disease is an uncommon illness in children that is characterized by high fever of at least five days' duration together with at least four of the following five findings:

    1. Inflammation with reddening of the whites of the eyes (conjunctivitis) without pus
    2. Redness or swelling of the hands or feet, or generalized skin peeling
    3. Rash
    4. Lymph node swelling in the neck
    5. Cracking, inflamed lips or throat, or red "strawberry" tongue

    The above criteria are used to make a diagnosis of Kawasaki's disease. The terminology "incomplete Kawasaki's disease" is sometimes used for patients with only some features of classical Kawasaki's disease. Most patients are under 5 years of age.

    What is mucocutaneous lymph node syndrome?

    Mucocutaneous lymph node syndrome is the original name for Kawasaki's disease. The original name was quite descriptive because the disease is characterized by the typical changes in the mucous membranes that line the lips and mouth and by the enlarged and tender lymph nodes. Kawasaki's disease is also sometimes referred to as Kawasaki disease.

    What are the usual symptoms and signs of Kawasaki's disease?

    The usual symptoms and signs of Kawasaki's disease include

    • fever,
    • reddening of the eyes without pus,
    • cracked and inflamed lips and mucous membranes of the mouth with an inflamed "strawberry" tongue,
    • ulcerative gum disease (gingivitis),
    • swollen lymph nodes in the neck (cervical lymphadenopathy),
    • joint pain often on both sides of the body,
    • irritability,
    • cough and runny nose,
    • and a rash that is raised and bright red, especially on the palms and soles.

    The rash appears in a glove-and-sock fashion over the skin of the hands and feet. The rash becomes hard, swollen (edematous), and then peels off.

    What are the less common findings?

    Most of the common symptoms described above will resolve without complications, even if untreated. Less common findings include inflammation of the lining of the heart (pericarditis), joints (arthritis), or covering of the brain and spinal cord (meningitis), and the other heart complications listed below in the section on serious complications. The outlook (prognosis) for an individual affected is primarily determined by the seriousness of any heart complications.

    What is the difference between Kawasaki's disease and Kawasaki's syndrome?

    They are the same. Kawasaki's disease is also referred to as Kawasaki's syndrome. It was first described in the late 1960s in Japan by the pediatrician Tomisaku Kawasaki.

    What causes Kawasaki's disease?

    The cause is not known. Microorganisms and toxins have been suspected, but none has been identified to date. Kawasaki's disease is not felt to be a contagious illness. Genetic factors and the immune system seem to play roles in the disease, which is characterized by immune activation.

    Who develops Kawasaki's disease?

    Kawasaki disease typically affects children under 5 years of age. Only rarely does it affect children over 8 years of age, in which case it is sometimes referred to as atypical Kawasaki's disease.

    How can Kawasaki's disease cause serious complications?

    Children with Kawasaki's disease can develop inflammation of the arteries of various parts of the body. This inflammation of the arteries is called vasculitis. Arteries that can be affected include the arteries that supply blood to the heart muscle (the coronary arteries). Vasculitis can cause weakening of the blood vessels and lead to areas of vessel widening (aneurysms). Coronary aneurysms have been reported in some with Kawasaki's disease.

    Because of the potential for heart injury and coronary aneurysms, special tests are performed to examine the heart. Children are typically evaluated with an electrocardiogram (EKG) and an ultrasound test of the heart (echocardiogram). Other arteries that can become inflamed include the arteries of the lungs, neck, and abdomen. These effects can lead to breathing problems, headaches, and pain in the belly, respectively. Blood tests to measure the degree of inflammation (such as C-reactive protein, or CRP, and erythrocyte sedimentation rate, or ESR) are often used to monitor the activity of the disease.

    What is the treatment for Kawasaki's disease?

    Children affected by Kawasaki's disease are hospitalized. Kawasaki's disease is treated with high doses of aspirin (salicylic acid) to reduce inflammation and to mildly thin the blood to prevent blood clot formation. Also used in treatment is gammaglobulin administered through the vein (intravenous immunoglobulin or IVIG), together with fluids. This treatment has been shown to decrease the chance of developing aneurysms in the coronary arteries. Sometimes cortisone medications are given. Persisting joint pains are treated with anti-inflammatory drugs, such as ibuprofen (Advil) or naproxen (Aleve).

    Learn more about: aspirin

    Plasma exchange (plasmapheresis) has been reported as effective in patients who were not responding to aspirin and gammaglobulin. Plasmapheresis is a procedure whereby the patient's plasma is removed from the blood and replaced with protein-containing fluids. By taking out portions of the patient's plasma, the procedure also removes antibodies and proteins that are felt to be part of the immune reaction that is causing the inflammation of the disease. Kawasaki's disease that is not responding to the traditional aspirin and gammaglobulin treatments can be deadly. Medications that block the effects of TNF (tumor necrosis factor), one of the messenger molecules in the inflammatory response, are being studied for use in these situations. Examples of TNF-blocking drugs are infliximab (Remicade) and etanercept (Enbrel). Further research is needed to design treatment programs for those who are failing conventional treatments. Pentoxifylline (Trental) is also being studied as a possible treatment for Kawasaki's disease.

    Learn more about: Remicade | Enbrel | Trental

    What are the usual symptoms and signs of Kawasaki's disease?

    The usual symptoms and signs of Kawasaki's disease include

    • fever,
    • reddening of the eyes without pus,
    • cracked and inflamed lips and mucous membranes of the mouth with an inflamed "strawberry" tongue,
    • ulcerative gum disease (gingivitis),
    • swollen lymph nodes in the neck (cervical lymphadenopathy),
    • joint pain often on both sides of the body,
    • irritability,
    • cough and runny nose,
    • and a rash that is raised and bright red, especially on the palms and soles.

    The rash appears in a glove-and-sock fashion over the skin of the hands and feet. The rash becomes hard, swollen (edematous), and then peels off.

    What are the less common findings?

    Most of the common symptoms described above will resolve without complications, even if untreated. Less common findings include inflammation of the lining of the heart (pericarditis), joints (arthritis), or covering of the brain and spinal cord (meningitis), and the other heart complications listed below in the section on serious complications. The outlook (prognosis) for an individual affected is primarily determined by the seriousness of any heart complications.

    What is the difference between Kawasaki's disease and Kawasaki's syndrome?

    They are the same. Kawasaki's disease is also referred to as Kawasaki's syndrome. It was first described in the late 1960s in Japan by the pediatrician Tomisaku Kawasaki.

    What causes Kawasaki's disease?

    The cause is not known. Microorganisms and toxins have been suspected, but none has been identified to date. Kawasaki's disease is not felt to be a contagious illness. Genetic factors and the immune system seem to play roles in the disease, which is characterized by immune activation.

    Who develops Kawasaki's disease?

    Kawasaki disease typically affects children under 5 years of age. Only rarely does it affect children over 8 years of age, in which case it is sometimes referred to as atypical Kawasaki's disease.

    How can Kawasaki's disease cause serious complications?

    Children with Kawasaki's disease can develop inflammation of the arteries of various parts of the body. This inflammation of the arteries is called vasculitis. Arteries that can be affected include the arteries that supply blood to the heart muscle (the coronary arteries). Vasculitis can cause weakening of the blood vessels and lead to areas of vessel widening (aneurysms). Coronary aneurysms have been reported in some with Kawasaki's disease.

    Because of the potential for heart injury and coronary aneurysms, special tests are performed to examine the heart. Children are typically evaluated with an electrocardiogram (EKG) and an ultrasound test of the heart (echocardiogram). Other arteries that can become inflamed include the arteries of the lungs, neck, and abdomen. These effects can lead to breathing problems, headaches, and pain in the belly, respectively. Blood tests to measure the degree of inflammation (such as C-reactive protein, or CRP, and erythrocyte sedimentation rate, or ESR) are often used to monitor the activity of the disease.

    What is the treatment for Kawasaki's disease?

    Children affected by Kawasaki's disease are hospitalized. Kawasaki's disease is treated with high doses of aspirin (salicylic acid) to reduce inflammation and to mildly thin the blood to prevent blood clot formation. Also used in treatment is gammaglobulin administered through the vein (intravenous immunoglobulin or IVIG), together with fluids. This treatment has been shown to decrease the chance of developing aneurysms in the coronary arteries. Sometimes cortisone medications are given. Persisting joint pains are treated with anti-inflammatory drugs, such as ibuprofen (Advil) or naproxen (Aleve).

    Learn more about: aspirin

    Plasma exchange (plasmapheresis) has been reported as effective in patients who were not responding to aspirin and gammaglobulin. Plasmapheresis is a procedure whereby the patient's plasma is removed from the blood and replaced with protein-containing fluids. By taking out portions of the patient's plasma, the procedure also removes antibodies and proteins that are felt to be part of the immune reaction that is causing the inflammation of the disease. Kawasaki's disease that is not responding to the traditional aspirin and gammaglobulin treatments can be deadly. Medications that block the effects of TNF (tumor necrosis factor), one of the messenger molecules in the inflammatory response, are being studied for use in these situations. Examples of TNF-blocking drugs are infliximab (Remicade) and etanercept (Enbrel). Further research is needed to design treatment programs for those who are failing conventional treatments. Pentoxifylline (Trental) is also being studied as a possible treatment for Kawasaki's disease.

    Learn more about: Remicade | Enbrel | Trental

    Source: http://www.rxlist.com

    They are the same. Kawasaki's disease is also referred to as Kawasaki's syndrome. It was first described in the late 1960s in Japan by the pediatrician Tomisaku Kawasaki.

    Source: http://www.rxlist.com

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