We investigated the incidence of sudden death in students with a history of Kawasaki disease (KD) while under school supervision. Reports of sudden death in students with a history of KD during 1990–1999 and 2000–2009 were retrieved from the mutual aid system data. The student’s grade, sex, circumstances at the time of sudden death, final diagnosis, recommended restrictions on school activities, and intensity of physical activity at the time of sudden death were investigated. There were 11 cases from 1990 to 1999 and 3 from 2000 to 2009; KD was complicated with coronary artery aneurysm (CAA) in nine and one cases, respectively. The incidence of sudden death decreased by approximately 50% for KD history and 80% for KD with CAA between the two decades; however, the difference was not statistically significant. Of the 14 cases, 12 occurred during moderate-to-strenuous exercise; the restriction on exercise for students with KD complicated with CAA was not followed in at least five cases during 1990–1999, while three cases during 2000–2009 occurred without recommended restriction. Cases of sudden cardiac death decreased during 2000–2009, compared with those during 1990–1999. Special attention is required for students with a history of KD, particularly when complicated with CAA. Full article

Background: Kawasaki disease (KD) is a form of systemic vasculitis that mainly affects children under the age of five years old. Limb swelling and redness are among the primary symptoms of KD. Previous studies have reported that wireless optical monitoring systems can identify limb indurations characteristics in patients with KD. Therefore, we conducted this study to monitor the dynamic changes in different stages of KD and the disease outcome of coronary artery lesions (CAL).Methods: KD patients who were admitted for intravenous immunoglobulin (IVIG) treatment and controls with or without fever were enrolled in this study. Near infrared spectroscopy data were collected for KD patients at different stages, including before (within one day before IVIG treatment, KD1) and shortly after IVIG treatment (within three days, KD2), at least 21 days after IVIG (KD3), 6 months later (KD4), 1 year later (KD5), 2 years later (KD6), and 3 years later (KD7).Results: This study included a total of 350 pieces of data, including data from 20 healthy controls, 64 fever controls, 53 KD1, 67 KD2, 58 KD3, 28 KD4, 25 KD5, 15 KD6, and 20 KD7. The relative HbO₂ of the KD1 group were significantly lower than those of the healthy group (0.298 ± 0.01 vs. 0.304 ± 0.05, p = 0.028) but no significant differences were found with the fever group. The HbT concentrations of KD1 group showed significantly lower than health group (0.632 ± 0.019 vs. 0.646 ± 0.021, p = 0.001) but no significant difference with fever control. Relative levels of HbO₂, HbT and Hb showed significant difference between KD1 and health control while StO₂ and H₂O showed difference between KD1 and fever control. The relative H₂O concentration was significantly higher in KD patients with CAL formation than without (p < 0.005). Conclusion: This report is the first to use near infrared spectroscopy to detect changes in tissue hemoglobin and water levels at different stages of KD in patients and showed that water content was significantly associated with CAL formation. This non-invasive device may benefit physicians by serving for early identification of KD from fever illness. Full article

Background: Under the Coronavirus disease 2019 (COVID-19) pandemic, manifestations in children with Kawasaki disease (KD) are different between the Western and the Eastern countries. Particularly, there has not been a report comparing a series of KD in Japan, where KD was originally discovered and has a large number of registered cases. Methods: We compared patients with KD under the period of the COVID-19 pandemic in Japan with the report from Italy during its reported period by a retrospective, cohort, observational study in a Japanese single center. Results: Thirty-two patients with typical KD were treated during the study period, while the Italian study reported 10 patients with the signs of KD. Concerning the proof of severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) infection, none (0%) of our KD cases showed a positive result and one and no patients developed the macrophage activation syndrome (MAS) and Kawasaki disease shock syndrome (KDSS), respectively; however, eight (80%) patients in the Italian series were confirmed with SARS-CoV-2 infection. MAS and KDSS developed in six and five patients, respectively. Conclusions: Cases reported as COVID-19 pandemic-related KD in Italy showed significantly different clinical characteristics from the typical KD symptoms known in Japan. Although they show KD-like manifestations, we cannot conclude that SARS-CoV-2 has the same etiology of our ‘classic’ KD at the present stage. Full article

Considerable advances have occurred in the understanding of Kawasaki disease, with a substantial drop in morbidity and mortality following the infusion of gamma globulin during the acute phase. Nevertheless, major complications may still occur. A 27-year-old male presented as an infant of 11 weeks when he was diagnosed as having Kawasaki disease. He was appropriately treated with aspirin and a gamma globulin infusion following his diagnosis 5 days after the onset of his illness. Despite that, he went on to develop coronary aneurysms. He represented a few weeks later with a history of inconsolable crying associated with pallor, suggestive of ischaemic chest pain. A repeat echocardiogram revealed infarction of the apex of the left ventricle with localised thrombus formation. There were persistent aneurysms within both coronary artery systems. A further infusion of gamma globulin was given. In view of the thrombus formation, he was started on warfarin. The thrombus gradually resolved with the development of a clearly defined left ventricular apical aneurysm. He has remained on warfarin, aiming for an international normalised ratio (INR) level of 2 to 2.5. He developed mild left ventricular dysfunction during late childhood, which improved following the commencement of an angiotensin-converting enzyme (ACE) inhibitor. Despite his ventricular aneurysm, there has been no documented evidence of ventricular tachycardia over the years. Repeated testing initially by nuclear perfusion scans and then by stress echocardiograms failed to show any inducible ischaemia apart from the apical ventricular aneurysm. A recent computed tomography (CT) coronary angiogram revealed an ectatic origin of the left main and the right coronary arteries with mild calcification involving the mid-portion of the latter and slight calcification of the former. His raised cholesterol level has responded well to a statin. Despite the persistence of the ventricular aneurysm, he continues to be managed conservatively, as he has remained well. The question arises as to what the long-term implications are of his left ventricle apical aneurysm. Should it be excised? Is he at risk for ventricular tachycardia and sudden death? In addition, although the coronary aneurysms have resolved, the CT coronary angiogram shows calcium plaques in both coronary arteries at the site of the earlier aneurysms. This finding raises the question as to whether all children who develop coronary artery aneurysms following Kawasaki disease should have a CT coronary angiogram performed in adulthood. Full article