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

A combination therapy for Kawasaki disease with severe complications: a case report

Kawasaki disease (KD) is a form of acute multisystem vasculitis that presents with various complications, including coronary artery aneurysm. Heart failure and brain damage are rare, but life-threatening complications are associated with KD. Here, we describe a 4-year-old girl who developed intravenous immunoglobulin-resistant KD with both left ventricular failure and acute encephalopathy. On day 8 of the illness, the low left ventricular ejection fraction, mitral regurgitation, and low blood pressure, which required continuous administration of dobutamine, were observed during the treatments for KD, including intravenous immunoglobulin. She also appeared unconscious, where the electroencephalogram showed slow waves of activity in all regions of the brain. The cardiac performance improved after she received plasma exchange for three days. However, her unconsciousness with slow waves of activity on electroencephalogram and fever continued after the plasma exchange. Therefore, she was treated with methylprednisolone pulse, followed by prednisolone, as well as intravenous immunoglobulin. Finally, she recovered without any cardiac or neurological sequelae not only at the time she was discharged, but also throughout the follow-up period. The combination therapy using plasma exchange and methylprednisolone pulse may be a treatment option for severe KD with left ventricular failure and acute encephalopathy complications

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

Epidemiology of Pediatric Out-of-Hospital Cardiac Arrest at School ― An Investigation of a Nationwide Registry in Japan ―

Kosuke Kiyohara, Junya Sado, Tetsuhisa Kitamura, Mamoru Ayusawa, Masahiko Nitta, Taku Iwami, Ken Nakata, Yasuto Sato, Noriko Kojimahara, Naohito Yamaguchi, Tomotaka Sobue, Yuri Kitamura, for the SPIRITS Investigators, Epidemiology of Pediatric Out-of-Hospital Cardiac Arrest at School　― An Investigation of a Nationwide Registry in Japan ―, Circulation Journal, 2018, Volume 82, Issue 4, Pages 1026-1032, Released March 23, 2018, [Advance publication] Released February 15, 2018, Online ISSN 1347-4820, Print ISSN 1346-9843, https://doi.org/10.1253/circj.CJ-17-1237, https://www.jstage.jst.go.jp/article/circj/82/4/82_CJ-17-1237/_article/-char/e