Oxygen Radicals Activity as a Predictor of Coronary Artery Lesions in Kawasaki Disease

Abstract

Background: Coronary aneurysms (CAA) appear around Day 10 of Kawasaki syndrome (KS). Enhanced perivascular echo brightness (PEB) is not widely accepted as a marker of vasculitis. To explore its utility in early diagnosis of KS, we examined interobserver agreement (IOA) and prevalence. Working definition: Appearance of bright broad echoes surrounding the coronary lumen extending for at least 1 cm along the artery, as compared to thin parallel echoes representing normal coronary artery walls distinct from the surrounding. Methods: IOA: 20 randomly mixed PEB positive and negative studies were reviewed blindly by 6 pediatric cardiologists after they were coached on the definition. Mean % IOA and 95% confidence intervals (CI) were calculated for presence/absence of PEB in right and left coronary arteries (RCA, LCA). Prevalence: 50 consecutive KS pts' echoes were reviewed for PEB and concomitant or subsequent coronary ectasia or aneurysm (CAE or CAA). Results: IOA: Overall IOA was 79.2% (95% CI: 74.4, 84.0). For RCA and LCA, IOAs were 76.7% (70.2, 83.1) and 81.7 (74.6, 88.7), respectively. Prevalence: 28/50 pts (56%) showed PEB in one or both CAs in the first echo. 13 and 4 of those (26% and 8%) showed CAE and CAA, respectively. PEB without CAE/CAA was noted on Day 7.2 ϩ 4.24 (m ϩ sd); PEB with CAE or CAA was noted on Day 9.29 ϩ 5.77 (NS). PEB was noted in 11/18 (61%) typical KS pts (TKS), in 11/14 (79%) atypical KS pts (AKS) and in 4/14 (29%) possible KS pts (PKS) (pϭ0.025). All pts who later developed CAA or CAE initially showed PEB. All PEB-negative pts remained free of CAA or CAE. Conclusions: (1) PEB is a sufficiently objective finding. (2) PEB is detectable in majority of pts before Day 10. (3) PEB precedes CAE or CAA in some pts. Background: Severe myocarditis leading to heart failure is a specific entity complicating the acute phase of Kawasaki disease (KD) in children. Clinical course and response to therapy appear to differ in those children with myocarditis requiring inotropic treatment. Objective: To characterize specific clinical, laboratory and imaging findings in patients with severe myocarditis and delineate their response to therapeutic intervention. Methods: A 3 year retrospective chart review of all patients seen at the HSC (1998HSC ( -2001 with the diagnosis of KD plus severe myocarditis was performed. The demographic features, clinical, lab and imaging findings and response to treatment were analyzed. Results: 5 patients (3female/ 2male) with a mean age at onset of 3.4years (range 1.5 to 4.4yrs) met diagnostic criteria for KD. In addition all children had significant fatigue, severe tachycardia (mean HR161/min) and hypotension (5/5 Ͻ10.perc.). Lab testing revealed elevated ESR (mean 105mm), anemia (mean HGB 112g/l), hypocalcemia (mean ion.Ca 0.99mmol/l) and hypoalbuminemia (mean 26g/l). All children received 2 doses of IVIG (2g/kg/dose). In addition to non-responsiveness to IVIG clinical signs of heart failure were aggravated (tachycardia: mean HR 195/min) supported by diagnostic findings of hem...