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To assess the longitudinal incidence of Kawasaki disease (KD) within the well-defined predominantly White population of Olmsted County, MN. This retrospective cohort study used a population-based medical record linkage system and manual chart reviews to identify children with KD in Olmsted County, MN between January 1, 1979-December 31, 2016. Age- and gender-adjusted incidence rates were calculated using the 2010 U.S. White population. 124 children with KD were confirmed during the study period (median age 3.5, 61% male, 85% White, 9% Asian). The overall age- and gender-adjusted incidence rates for all ages and < 5 years old were 9.8 and 21.4 per 100,000 person-years, respectively. There was an overall increase in incidence up to 1994 followed by plateau, except among children between the ages of 1–5 years. There was also an overall increase in incidence among females compared to males. 24% of children had cardiac complications. While the overall incidence of KD in Olmsted County appears to be stable since 1994, the incidence of KD in subgroups of children 1–5 years old and females seems to have increased. Given the rising trends and one-quarter of children developing cardiac complications, further studies identifying factors driving these trends are warranted. [ABSTRACT FROM AUTHOR]

A giant coronary artery (CA) aneurysm is a potentially fatal cardiac complication resulting from Kawasaki disease (KD). We aimed to identify epidemiologic characteristics and potential risk factors associated with giant CA aneurysms identified after acute KD. We analyzed 90,252 patients diagnosed with KD from 2011 to 2018, using data obtained in nationwide KD surveys conducted in Japan. Multivariable logistic regression analyses were performed to evaluate potential risk factors associated with subsequent giant CA aneurysm complications (defined as lumen size ≥ 8 mm), adjusting for all potential factors. Giant CA aneurysms were identified in 144 patients (0.16%) after acute KD. The annual prevalence ranged from 0.07 to 0.20% during the study period. In the multivariate analyses, male sex (adjusted odds ratio 2.09 [95% confidence interval 1.41–3.11], recurrent KD (1.90 [1.09–3.33]), IVIG administration at 1–4 days of illness (1.49 [1.04–2.15]) and ≥ 8 days after KD onset (2.52 [1.38–4.60]; reference, 5–7 days), detection of CA dilatations and aneurysms at initial echocardiography (4.17 [1.85–5.41] and 46.5 [28.8–74.8], respectively), and resistance to IVIG treatment (6.09 [4.23–8.75]) were significantly associated with giant CA aneurysm complications identified after acute KD. The annual prevalence of giant CA aneurysms identified after acute KD did not increase during the study period. Patients with larger CA abnormalities detected at initial echocardiography were independently associated with progression to giant CA aneurysm complications after acute KD regardless of the number of days from onset at treatment initiation. [ABSTRACT FROM AUTHOR]

This study aimed to assess the outcome of cardiovascular diseases for patients with chronic active Epstein–Barr virus infection (CAEBV). The study enrolled 15 patients (7 boys and 8 girls) who fulfilled the diagnostic criteria for CAEBV, including 10 patients with T-cell type and 3 patients with natural killer (NK)-cell type. The median age at the CAEBV onset was 6.3 years (range, 1.2–17.8 years). Regular cardiologic studies were performed during the median follow-up period of 8 years (range, 2–20 years). Nine patients (60%) had cardiac diseases including coronary artery lesion (CAL) ( n = 4, 44%), decreased left ventricular ejection fraction and pericardial effusion in ( n = 3, 33%), complete atrioventricular block ( n = 1), and sudden arrest ( n = 1). The frequency of fever (78%, p = 0.04) or cytopenias (100%, p = 0.01), as the major symptom among patients with cardiac complications, was higher than among those without complications. The median time from disease onset to detection of CAL was 3.4 years (range, 1.8–8.6 years). The mean z-score increased to 3.98. Seven patients (78%) with cardiac complications died of disease progression, hematopoietic stem cell transplantation–related events, or both. In two patients, CAL regressed after allogeneic cord blood transplantation. Among CAEBV patients, CAL was the most common cardiac complication and could not be controlled without the eradication of EBV-infected T- and NK-cells. [ABSTRACT FROM AUTHOR]