Kawasaki disease
(ka‵wă-sa'kē 
)
[Tomisaku Kawasaki, Japanese pediatrician, 1925–2020]
An acute febrile vasculitis of children, marked acutely by fever, rashes, lymphadenopathy, and irritability and chronically by late cardiac complications, including coronary artery aneurysms and myocardial infarction. Fever is present on the first day of the illness and may last from 1 to 3 weeks. The child with Kawasaki disease is irritable, lethargic, and has bilateral congestion of the conjunctivae. The oral mucosa may turn deep red, and the lips often become dry and cracked. It is common for the affected child to have unilateral cervical lymphadenopathy. A strawberry tongue is a prominent sign, as is redness and peeling of the skin of the hand s and feet. The disease is rarely fatal in the acute phase, but children may die suddenly from coronary artery disease some years later. This disease was previously called mucocutaneous lymph node syndrome. Cervical lymph nodes are most often the ones enlarged.
SEE: toxic shock syndrome.
Epidemiology: In 80% of children, diagnosis takes place before age 5, and usually the younger the age at onset, the more severe the disease. The disease is rare after age 8. It occurs more commonly in Asian children than in other ethnic groups. In Japan, there are about 5000 to 6000 cases annually. By comparison, about 4000 cases occur annually in the U.S.
Diagnosis: Because of the similarities of this disease to others, e.g., scarlet fever and toxic shock syndrome, diagnostic criteria are strict. There must be fever and at least four of the following five findings: conjunctivitis; oral lesions like those described above; redness, swelling, and peeling of the fingers and toes; rash similar to that described above; cervical lymphadenopathy. The erythrocyte sedimentation rate (ESR) is elevated.
Etiology: The disease appears to be linked to infection with human coronaviruses and some other infectious agents.
Complications: Formation of giant aneurysms of the coronary artery (esp. in infants and very young children) is the major complication and can lead to sudden death or myocardial infarction later in life. Mortality from the disease is about 1%. Other findings may include arthritis, otitis media, diarrhea, uveitis, pyuria (sterile), and hepatic dysfunction.
Treatment: If given within 10 days of onset of fever, high-dose intravenous immunoglobulin (IVIG) therapy over 12 to 24 hr can dramatically relieve the symptoms and prevent coronary artery dilation. Daily aspirin therapy has traditionally also been given to decrease the risk of coronary artery dilation, but its safety (given the risk of Reye’s syndrome) and its effectiveness have been questioned. Neither antibiotics nor high-dose corticosteroids are effective. Frequent follow-up care, including repeat evaluations to detect or monitor heart disease, is essential.
Patient Care: Medications are administered as prescribed, and the child is observed for salicylate toxicity. Both child and family benefit from psychological support during the acute period of illness and require continued support through the chronic phase. Parents learn about the importance of following the prescribed regimen and early signs of toxicity. The child requires careful monitoring during the acute phase and conscientious follow-up thereafter. The child's progress is monitored; parents must understand the importance of normal activity, sound nutrition, and good hygiene. Referral to a mental health practitioner, support groups such as the Kawasaki Disease Foundation, or spiritual counselor may be helpful for patients and families.