assessed and the patient should be monitored closely with serial echocardiograms until KD is diagnosed or until the patient is afebrile and well appearing for 48 hours TABLE 101.19 ECHOCARDIOGRAPHIC CRITERIA SUGGESTIVE OF KAWASAKI DISEASE Echocardiogram considered positive if any of the three criteria are met I z score of LAD or RCA of ≥2.5 II Coronary arteries meet Japanese Ministry of Health criteria for aneurysms III If three or more suggestive features are present Lack of tapering Decreased LV function Mitral regurgitation Pericardial effusion z scores in LAD or RCA of 2–2.5 LAD, left anterior descending coronary artery; RCA, right coronary artery; LV, left ventricle Management IVIG If the clinical criteria are fulfilled, or partial clinical criteria are met with supportive supplemental laboratory criteria, then treatment should be initiated Recommended initial therapy includes IVIG and aspirin within the first 10 days of the illness; treatment with IVIG within this time frame significantly shortens disease duration and minimizes the incidence of complications Overall, prompt diagnosis and appropriate therapy prevent aneurysm formation in approximately 95% of children and result in rapid symptomatic improvement in about 90% Studies in Japan were the first to suggest relative protection from coronary artery aneurysms when IVIG is administered early in the course of KD Since then, further trials in the United States and Japan have confirmed this finding and documented the safety of high-dose infusions of immunoglobulin At present, a single large infusion of IVIG (2 g/kg) administered over to 12 hours is the standard of care for KD This is somewhat more effective than multiple smaller infusions, and it also significantly shortens the duration of hospitalization Therapy with IVIG also has other benefits Treatment results in a reduced prevalence of giant aneurysms, the most serious form of coronary abnormality caused by the disease It also accelerates normalization of abnormalities of left ventricular systolic function and contractility Finally, high-dose IVIG reduces fever and laboratory indices of inflammation, suggesting a rapid, generalized anti-inflammatory effect in addition to specific cardioprotective effects Despite its advantages, IVIG is an expensive and potentially toxic intervention The greatest long-term concern is the possible