kawasaki syndrome

Classification and external resources Kawasaki syndrome ICD-10 M30.3 ICD-9 446.1 OMIM 611775 DiseasesDB 7121 MedlinePlus 000989 eMedicine ped/1236 MeSH D009080

Kawasaki disease (also known as lymph node syndrome, mucocutaneous node disease, infantile polyarteritis and Kawasaki syndrome) is an inflammation (vasculitis) of the middle-sized arteries. It affects many organs, including the skin, mucous membranes, lymph nodes, and blood vessel walls, but the most serious effect is on the heart. Without treatment, mortality may approach 1%, usually within 6 weeks of onset. With treatment, mortality is <0.01% in the U.S.[1]

It was first described in 1967 by Dr. Tomisaku Kawasaki in Japan.[2] Contents [hide] 1 Incidence and risk factors 2 Causes 3 Presentation 4 Symptoms 5 Signs and tests 6 Diagnosis 7 Treatment 8 Prognosis 9 References 10 External links //

Incidence and risk factors

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. Approximately 2000-4000 cases are identified in the United States each year.[3][4]

Causes

The causative agent of Kawasaki disease is still unknown,[5][6][7] but current theories center primarily on immunological causes for the disease. Evidence increasingly points to an infectious etiology,[8] but debate continues on whether the cause is a conventional antigenic substance or a superantigen.[9] 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."[4]

An association has been identified with a SNP in the ITPKC gene, which codes an enzyme that negatively regulates T-cell activation.[10] 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.[4] The HLA-B51 serotype has been found to be associated with endemic instances of the disease.[11]

Presentation

The cardiac complications are, by far, the most important aspect of the disease.[citation needed] 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.

Symptoms

Kawasaki