Signs and symptoms

Symptoms

Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal doses of paracetamol (acetaminophen) or ibuprofen. The fever may persist steadily for up to two weeks and is normally accompanied by irritability. Affected children develop red eyes, red mucous membranes in the mouth, red cracked lips, a “strawberry tongue“, iritis, keratic precipitates (detectable by an ophthalmologist but usually too small to be seen by the unaided eye), and swollen lymph nodes. Skin rashes occur early in the disease, and peeling of the skin in the genital area, hands, and feet (especially around the nails and on the palms and soles) may occur in later phases. Some of these symptoms may come and go during the course of the illness. If left untreated, the symptoms will eventually relent, but coronary artery aneurysms will not improve, resulting in a significant risk of death or disability due to myocardial infarction (heart attack). If treated in a timely fashion, this risk can be mostly avoided and the course of illness cut short.^{[citation needed]}

Pediatrics, Kawasaki disease. Note the appearance of the hand and lips. Photo courtesy of Sam Richardson, MD.

BAYI DENGAN KAWAZAKI

DermNet.com is developed & maintained by Alan N. Binnick & Thomas P. Habif, Dartmouth Medical School, New Hampshire.           DermNet.com is developed & maintained by Alan N. Binnick & Thomas P. Habif, Dartmouth Medical School, New Hampshire.

Complications

The cardiac complications are the most important aspect of the disease. Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms. These aneurysms can lead to myocardial infarction (heart attack) even in young children. Overall, about 10–18% of children with Kawasaki disease develop coronary artery aneurysms with much higher prevalence among patients who are not treated early in the course of illness. Kawasaki disease and rheumatic fever are most common causes of acquired heart disease among children in the United States.

Causes

Like all autoimmune diseases, the cause of Kawasaki disease is presumably the interaction of genetic and environmental factors, possibly including an infection. The specific cause is unknown,^[6][7][8] but current theories center primarily on immunological causes for the disease. Evidence increasingly points to an infectious etiology,^[9] but debate continues on whether the cause is a conventional antigenic substance or a superantigen.^[10] Children’s Hospital Boston reports that “[s]ome studies have found associations between the occurrence of Kawasaki disease and recent exposure to carpet cleaning or residence near a body of stagnant water; however, cause and effect have not been established.”

An association has been identified with a SNP in the ITPKC gene, which codes an enzyme that negatively regulates T-cell activation.An additional factor that suggests genetic susceptibility is the fact that regardless of where they are living, Japanese children are more likely than other children to contract the disease.The HLA-B51 serotype has been found to be associated with endemic instances of the disease.

Diagnosis

Kawasaki disease can only be diagnosed clinically (ie. by medical signs and symptoms). There exists no specific laboratory test that for this condition. It is normally difficult to establish the diagnosis, especially early in the course of illness, and frequently children are not diagnosed until they have seen several health care providers, or visited a number of different health care providers. Many other serious illnesses can cause similar symptoms, and must be considered in the differential diagnosis, including scarlet fever, toxic shock syndrome, juvenile idiopathic arthritis, and childhood mercury poisoning (acrodynia).

Classically, five days of fever^[plus four of five diagnostic criteria must be met in order to establish the diagnosis. The criteria are: (1) erythema of the lips or oral cavity or cracking of the lips; (2) rash on the trunk; (3) swelling or erythema of the hands or feet; (4) red eyes (conjunctival injection) (5) swollen lymph node in the neck of at least 15 millimeters.

Many children, especially infants, eventually diagnosed with Kawasaki disease do not exhibit all of the above criteria. In fact, many experts now recommend treating for Kawasaki disease even if only three days of fever have passed and at least three diagnostic criteria are present, especially if other tests reveal abnormalities consistent with Kawasaki disease. In addition, the diagnosis can be made purely by the detection of coronary artery aneurysms in the proper clinical setting.

Investigations

A physical examination will demonstrate many of the features listed above.

Blood tests

• Complete blood count (CBC) may reveal normocytic anemia and eventually thrombocytosis
• Erythrocyte sedimentation rate (ESR) will be elevated
• C-reactive protein (CRP) will be elevated
• Liver function tests may show evidence of hepatic inflammation and low serum albumin

Other tests (may or may not be performed)

• Electrocardiogram may show evidence of ventricular dysfunction or, occasionally, arrhythmia due to myocarditis
• Echocardiogram may show subtle coronary artery changes or, later, true aneurysms.

Echocardiogram in parasternal short axis view showing coronary aneurysm in Kawasaki disease 

• Ultrasound or computerized tomography may show hydrops (enlargement) of the gallbladder
• Urinalysis may show white blood cells and protein in the urine (pyuria and proteinuria) without evidence of bacterial growth
• Lumbar puncture may show evidence of aseptic meningitis
• Angiography was historically used to detect coronary artery aneurysms and remains the gold standard for their detection, but is rarely used today unless coronary artery aneurysms have already been detected by echocardiography.

Treatment

Children with Kawasaki disease should be hospitalized and cared for by a physician who has experience with this disease. When in an academic medical center, care is often shared between pediatric cardiology and pediatric infectious disease specialists (although no specific infectious agent has been identified yet^[5]). It is imperative that treatment be started as soon as the diagnosis is made to prevent damage to the coronary arteries.

Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease and is administered in high doses with marked improvement usually noted within 24 hours. If the fever does not respond, an additional dose may have to be considered. IVIG by itself is most useful within the first 7 days of onset of fever, in terms of preventing coronary artery aneurysm.

Salicylate therapy, particularly aspirin, remains an important part of the treatment (though questioned by some)^[15] but salicylates alone are not as effective as Intravenous immunoglobulin. Aspirin therapy is started at high doses until the fever subsides, and then is continued at a low dose when the patient returns home, usually for 2 months to prevent blood clots from forming. Except for Kawasaki disease and a few other indications, aspirin is otherwise normally not recommended for children due to its association with Reye’s syndrome.

Corticosteroids have also been used, especially when other treatments fail or symptoms recur, but in a randomized controlled trial, the addition of corticosteroid to immune globulin and aspirin did not improve outcome.

There are also treatments for iritis and other eye symptoms.

Epidemiology

By far the highest incidence of Kawasaki disease occurs in Japan (175 per 100,000), though its incidence in the United States is increasing. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than 5 years of age. The disease affects boys more than girls. Kawasaki was extremely uncommon in caucasians until the last few decades. Approximately 2000-4000 cases are identified in the United States each year.

Prognosis

With early treatment, rapid recovery from the acute symptoms can be expected and the risk of coronary artery aneurysms greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited (i.e. the patient will recover eventually), but the risk of coronary artery involvement is much greater. Overall, about 2% of patients die from complications of coronary vasculitis. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every 1–2 years to screen for progression of cardiac involvement.

It is also not uncommon that a relapse of symptoms may occur soon after initial treatment with IVIG. This usually requires re-hospitalization and retreatment. Treatment with IVIG can cause allergic and non-allergic acute reactions, aseptic meningitis, fluid overload and, rarely, other serious reactions. Aspirin may increase the risk of bleeding from other causes and may be associated with Reye’s syndrome. Overall, life-threatening complications resulting from therapy for Kawasaki disease are exceedingly rare, especially compared with the risk of non-treatment.

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MILLER’S_REVIEW_OF_ORTHOPEDICS.pdf
Modern Neuromuscular Techniques (Modern Neuromuscular Techniques (WCD)) (2003).ISO
MP Van De Graaff Human Anatomy.pdf
Musculoskeletal.Diseases.DIAGNOSTIC IMAGING AND INTERVENTIONAL TECHNIQUES.pdf
Nelson.Textbook.of.Pediatrics.17th.ed – Behrman.pdb
Nervous System and Sensory Organs – Color Atlas and Textbook of Human Anatomy – Volume 3.pdf
Neuroanatomy – Atlas of Structures Sections Systems 6ed.pdf
Neuroscience – D.Purves, et al.pdf
Neurotransmitters, Drugs and Brain Function Wiley (2001).pdf
Nurses manual of laboratory and diagnostic tests 4th Ed 2003.pdf
Nursing practice in multiple sclerosis – a core curriculum.pdf
Occupational Health Practice – Arnold.Publishers.Fourth.EditionISBN0750627204.pdf
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Outpatient and Primary Care Medicine, 2005 Edition (2004).pdf
Oxford Textbook Of Rheumatology, 2nd ed (1998).pdb
Oxford_Dictionary_of_Medical_Quotations.pdf
Oxford_textbook_of_public_health_4th_ed.pdb
Pediatric Musculoskeletal Diseases.pdf
Pediatric Radiography.pdf
Pain and Depression – An Interdisciplinary Patient-centered Approach.pdf
Pain.Medicine.and.Management.Just.the.Facts.pdf
Pain.Psychological.Perspectives.eBook-DDU.pdf
PATHOPHYSIOLOGY OF DISEASE.pdf
Pediatric Orthopedics for Primary Care Physicians.pdf
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Physical Medicine and Rehabilitation Board Review.pdf
Pocket Atlas of Radiographic Anatomy 2nd ed, 2000.pdf
Pocket Guide to Diagnostic Tests-0838581358.pdf
Pocket.Guide.to.Musculoskeletal.Diagnosis.3HAXAP.pdf
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Rosen’s Emergency Medicine.chm
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Screening Notes Rehabilitation Specialists Pocket Guide (Davis Notes S) ISBN0803615736.pdf
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SPORTS MEDICINE Just the Facts.pdf
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Super Joints Russian Longevity Secrets for Pain-Free Movement, Maximum Mobility & Flexible Strength (2001).pdf
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The Clinical Science of Neurologic Rehabilitation (Contemporary Neurology Series, 66) (2003).pdf
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The Handbook of Brain Theory and Neural Networks – ISBN 0262511029.pdf
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Understanding Human Anatomy and Physiology 5th edition.pdf
Using Alternative Therapies – A Qualitative Analysis.pdf
Visions of the Future. Chemistry and Life Science.pdf
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WHO manuals of diagnostic imaging – Radiographic Anatomy and Interpretation of the Musculoskeletal System.pdf
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Copyright © 2009, Clinical Pediatric Online Information Education Network. All rights reserved.

Supported  by
CLINICAL PEDIATRIC ONLINE

Yudhasmara Foundation

JL Taman Bendungan Asahan 5 Jakarta Indonesia 102010

phone : 62(021) 70081995 – 5703646

email : judarwanto@gmail.com,

http://clinicalpediatric.wordpress.com/

Clinical and Editor in Chief :

DR WIDODO JUDARWANTO

Copyright © 2009, Clinical Pediatric Online Information Education Network. All rights reserved.

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Office ; JL Taman Bendungan Asahan 5 Jakarta Indonesia 10210

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email : judarwanto@gmail.com

Copyright © 2009, Clinic For Children Information Education Network. All rights reserved.

Supported  by
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Office ; JL Taman Bendungan Asahan 5 Jakarta Indonesia 10210

phone : 62(021) 70081995 – 5703646

email : judarwanto@gmail.com,

http://childrenclinic.wordpress.com/

Editor in Chief :

Dr WIDODO JUDARWANTO

email : judarwanto@gmail.com

Copyright © 2009, Clinic For Children Information Education Network. All rights reserved.

Supported  by
CLINIC FOR CHILDREN

Yudhasmara Foundation

Office ; JL Taman Bendungan Asahan 5 Jakarta Indonesia 10210

phone : 62(021) 70081995 – 5703646

email : judarwanto@gmail.com,

http://childrenclinic.wordpress.com/

Editor in Chief :

Dr WIDODO JUDARWANTO

email : judarwanto@gmail.com

Copyright © 2009, Clinic For Children Information Education Network. All rights reserved.

Supported  by
CLINIC FOR CHILDREN

Yudhasmara Foundation

Office ; JL Taman Bendungan Asahan 5 Jakarta Indonesia 10210

phone : 62(021) 70081995 – 5703646

email : judarwanto@gmail.com,

http://childrenclinic.wordpress.com/

Editor in Chief :

Dr WIDODO JUDARWANTO

email : judarwanto@gmail.com

Copyright © 2009, Clinic For Children Information Education Network. All rights reserved.