Sudden Death in Patients with a History of Kawasaki Disease under School Supervision

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

A case of incomplete Kawasaki disease with extremely high serum ferritin and interleukin-18 levels

Abstract Background The clinical features and laboratory parameters of patients with Kawasaki disease (KD) and systemic juvenile idiopathic arthritis (sJIA) occasionally overlap. Therefore, serum levels of cytokine and ferritin are used as markers to distinguish between KD and sJIA. KD patients have a high level of interleukin (IL)-6, low level of IL-18, and no elevation of the level of serum ferritin. Conversely, sJIA patients have a low level of IL-6 and high levels of IL-18 and ferritin in the serum. However, to the best of our knowledge, no case report of KD with a low serum level of IL-6 and extremely high levels of IL-18 and ferritin is found. Case presentation A 6-year-old boy presented with a history of fever for 9 days and a rash that appeared 7 days from the onset. He was diagnosed with incomplete KD because of fever, skin rash, oral cavity erythematous changes, and erythema and edema of the hands with laboratory findings of serum albumin level < 3.0 g/dL, elevated alanine aminotransferase level and leukocyturia. Intravenous immunoglobulin and prednisolone and oral aspirin were introduced on the 10th day. Fever subsided 1 day after initiating the treatment, but arthritis of both knees appeared in addition to hepatosplenomegaly. We suspected sJIA, as the serum level of ferritin was 19,740 ng/mL, IL-6 was < 3 pg/mL, and IL-18 was 132,000 pg/mL. Skin desquamation of the fingertips was observed 18 days from the onset; thus, he was finally diagnosed with incomplete KD with arthritis. At 32 days from the onset, we stopped the prednisolone therapy and no symptoms of relapse were observed afterwards. In the follow-up at 16 months from the onset, he had neither signs of active joint or skin involvement, nor cardiac involvement. Conclusions Although patients with sJIA generally have high serum levels of IL-18 and ferritin, this was a case of incomplete KD with extremely high serum levels of IL-18 and ferritin. Serum cytokine and ferritin are often used for the differential diagnosis of KD and sJIA. We need to recognize the existence of KD with high serum levels of IL-18 and ferritin

Sex Disparities in Receipt of Bystander Interventions for Students Who Experienced Cardiac Arrest in Japan

Matsui S, Kitamura T, Kiyohara K, et al. Sex Disparities in Receipt of Bystander Interventions for Students Who Experienced Cardiac Arrest in Japan. JAMA Netw Open. 2019;2(5):e195111. doi:10.1001/jamanetworkopen.2019.511

Analysis of Coronary Arterial Aneurysm Regression in Patients With Kawasaki Disease by Aneurysm Severity: Factors Associated With Regression

Background Coronary arterial aneurysms (CAAs) associated with Kawasaki disease (KD) significantly affect prognosis. However, the clinical course of CAAs and factors associated with CAA regression have not been well analyzed. Methods and Results The cohort of the Z‐Score 2nd Project Stage study, a multicenter, retrospective, cohort study involving 44 institutions in Japan including 1006 patients with KD, was examined. CAAs were classified by the z score of their internal diameter in the acute phase: small (z<5), medium (5≤z<10), and large (z≥10). The lower limit of small CAA was based on the Japanese Ministry of Health, Labour and Welfare criteria. In the right coronary artery, the CAA regression rates 10 years after diagnosis were 95.5% for small, 83.2% for medium, and 36.3% for large. In the proximal left anterior descending artery, the regression rates 10 years after diagnosis were 95.3% for small, 80.1% for medium, and 28.8% for large. Cox regression analysis showed that diagnosis under the age of 1 year and onset of KD in 2010 to 2012 for the right coronary artery and the left anterior descending artery, and female for the right coronary artery were significantly associated with a high regression rate, whereas large CAAs for the right coronary artery and the left anterior descending artery were significantly associated with a low regression rate. Conclusions The current study, the largest Japanese study of its kind, found that small aneurysm, recent onset, and diagnosis under the age of 1 year predict regression, and that even giant aneurysms could regress. These data may contribute to long‐term management of coronary aneurysms. Registration URL: https://www.umin.ac.jp/ctr/; Unique identifier: UMIN000010606