Pediatric emergency medicine trisk 3013 3013

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
8 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