Kawasaki disease is a febrile condition affecting primarily children who are 5 yr of age or younger. It is notable for its association with vasculits of the large coronary blood vessels. It has surpassed rheumatic fever as the leading cause of acquired heart disease in children in the United States, has been recognized worldwide, and appears to be increasing in frequency. Kawasaki disease has been described in all racial groups, but appears to be a predilection for Japanese.
There is no evidence for person-to-person transmission.The cause remains unknown,but bacterial toxins similar the staphylococcal toxins of the toxic shock syndrome may be involved in its pathogenesis.There is no evidence that autoimmunity plays a role in pathogenesis; the finding of increased members of T-cell subset is consistent with toxin superantigen stimulation.
Diagnostic rests on the demonstration of characteristic clinical signs:
A.Fever lasting for at least 5 days unresponsive to antibiotic therapy.
B.Presence of four of the following conditions:
1.Bilateral nonpurulente conjunctival injection
2.Changes of the mucosa of the oropharynx, including infected pharynx, infected and/or dry fissured lips, strawberry tongue.
3.Changes of the peripheral extremities,such as edema and/or erythema of the hands or feet,desquamation,usually beginning periungually.
4.Rash,primarily truncal,polymorphous but nonvesicular.
5.Cervical lymphadenopathy.
C.Illness not explained by other known disease process.
The disease can be subdivided into four stages:
1.Acute phase:
Lasts about 10 days
2.Subacute phase:
3.Convalescent phase:
4.Chronic phase:
Atypical cases manifesting few early signs but later caricaturists coronary artery lesions have been reported. Atypical patients, often younger than 1 yr of age, may have incorrect admitting diagnoses[gastroenteritis, viral syndrome, sepses], and a high morbidity rate.
Laboratory Findings
There are no diagnostic tests.Leukocytosis with a predominance of immature forms and thrombocytosis[in the 2nd-3rd wk] can be streaking; anemia is also common. Sedimentation rates and C-reactive proteins levels are usually greatly elevated. Test results for autoantibodies including ANA and rheumatoid factors are negative,and hemolytic complement levels are normal or high.Mild proteinuria and pyuria may be present,as may cerebrospinal fluid pleocytosis. Serum levels of hepatic transaminases and bilirubin may be slightly elevated.
Cardiac involvement is the most important manifestation of the disease. Two-dimensional echocardiogram should be performed in all children with known or suspected Kawasaki disease at the time of presentation and again during the first 2 wk of disease.
Diagnosis rests on the clinical features. There are no diagnostic laboratory tests, although demonstration of coronary artery involvement revealed by echocardiogram is highly suggestive of this condition.The differential diagnoses includes scarlet fever, toxic shock syndrome, leptospirosis, Epstein-Barr virus infection, juvenile rheumatoid arthritis, measles, acrodynia, Rocky Mountain spotted fever, drug reactions, Stevens-Johnson syndrome, and vasculitis syndromes.
Recovery is usually complete in patients who do not have detectable coronary vasculitis;second attacks occur only rarely.A few reportes detail the later occurrence of aneurysms of large vessels than the coronary arteries.
Duration of fever, presumably reflecting the severity of ongoing vasculitis, has been confirmed as a powerful predictor of these lesions.
Kawasaki disease responds dramatically to therapy with intravenous gamma globulin given during the period of active febrile disease. Fever and other attendant systemic manifestations often abate within 24 hs of initial therapy. Furthermore, controlled studies show that intravenous gamma globulin therapy given early in disease prevents coronary vascular involvement.The recommended regimen consists of an intravenous infusion of 2g/kg given as a single dose over 10-12hr. Therapy given within 10 days of onset appears to be effective in preventing coronary vascular damage. Side effects of intravenous gamma globulin therapy are rare and include anaphylaxis, chills, fever, headache, and myalgia.
Salicylate therapy is also indicated during the febrile phase; therapeutic serum concentrations of 20-30mg/dl are desirable but may be difficult to achieve, even whit doses of salicylates as high as 100mg/kg/24hr. Continuance of low,single-dose[5mg/kg/24hr]salicylate therapy for its antithrombotic effects has been advocated for 6-8wk after the period of active disease subsides. Low-dose aspirin with or without dipyridamole should be continued until coronary lesions resolve.
Corticosteroids therapy is rarely used in Kawasaki disease, and some consider it contraindicated.
Thrombolytic therapy with agents such as streptokinase has been used for patients with thrombosis or peripheral artery ischemia. Arterial bypass surgery may be appropriate for rare patients with severe coronary occlusion.
1.Nelson:Textbook of Pediatrics
2.Peter C.Rowe,Angela Quinlan and Brian K. H. Luke:Value of degenerative change in neutrophils as a diagnostic test for Kawasaki disease:J PEDIATR 1991:vol 119:370-374
3.Robert P. Sundel,Jane C.Burns,Annette Baker,Alexa Beiser and Jane W. Newburger:Gamma globulin re-treatment in Kawasaki disease:J PEDIAT 1993.vol 123:657-659
Back to PEDIATRICS
Back to SPECIALTIES
Back to MEDSTUDENTS' HOMEPAGE