Kawasaki DiseasePub Date : 2023-06-01DOI: 10.59492/kd.2023.1.1.e5
Jihye You
{"title":"Differentiation between Lymph-Node-First Presentation of Kawasaki Disease from Bacterial Cervical Lymphadenitis","authors":"Jihye You","doi":"10.59492/kd.2023.1.1.e5","DOIUrl":"https://doi.org/10.59492/kd.2023.1.1.e5","url":null,"abstract":"\u0000\u0000Kawasaki disease (KD) is the leading cause of acquired heart disease in children. If the patients are left untreated, coronary artery aneurysms may occur. Among the major criteria for KD, lymphadenopathy is the least common. Thus, lymph node-first presentation KD (NFKD) may be overlooked, resulting in coronary artery dilatation. Pediatric patients with fever and cervical lymphadenopathy should be cautiously evaluated for an accurate diagnosis and to minimize complications. Laboratory findings and imaging studies may help make a diagnosis in these patients. We discussed eight previous studies and compared the results of laboratory and imaging findings to differentiate between NFKD from bacterial cervical lymphadenitis (BCL).\u0000","PeriodicalId":208326,"journal":{"name":"Kawasaki Disease","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126567292","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kawasaki DiseasePub Date : 2023-06-01DOI: 10.59492/kd.2023.1.1.e2
Ji Whan Han
{"title":"Historical Background of Kawasaki Disease in Korea","authors":"Ji Whan Han","doi":"10.59492/kd.2023.1.1.e2","DOIUrl":"https://doi.org/10.59492/kd.2023.1.1.e2","url":null,"abstract":"\u0000\u0000The first 50 cases of Kawasaki disease (KD) were reported in 1967 by Dr. Tomisaku Kawasaki. In Korea, Park et al. reported the first 5 cases of KD in 1973. In 1970, the Japanese Kawasaki Disease Research Committee was established with support from the Ministry of Health, Labour and Welfare of Japan and conducted the first Japanese nationwide epidemiological survey on KD that same year. In Korea, the first epidemiological study on KD was conducted by Lee et al. in 1982. The first nationwide epidemiological survey on KD in Korea was conducted by Yoon et al. from 1991 through 1993 and has been subsequently conducted every three years. The Korean Kawasaki Disease Study Group was founded in 2003 under the Korean Pediatric Heart Association. The Korean Society of Kawasaki Disease was established in 2015 and has since been conducting the triennial nationwide epidemiological survey.\u0000","PeriodicalId":208326,"journal":{"name":"Kawasaki Disease","volume":"98 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115958635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kawasaki DiseasePub Date : 2023-06-01DOI: 10.59492/kd.2023.1.1.e8
H. Jun, E. Shin, J. Baek, J. Yu, Young-Hwue Kim, J. Ko
{"title":"Plasma Exchange Treatment in Five Kawasaki Disease Patients Unresponsive to Intravenous Immunoglobulin","authors":"H. Jun, E. Shin, J. Baek, J. Yu, Young-Hwue Kim, J. Ko","doi":"10.59492/kd.2023.1.1.e8","DOIUrl":"https://doi.org/10.59492/kd.2023.1.1.e8","url":null,"abstract":"\u0000\u0000\u0000\u0000The efficacy of high-dose intravenous immunoglobulin (IVIG) treatment for acute-stage Kawasaki disease patients has become widely recognized. However, in the case of refractory Kawasaki disease, alternative treatment should be considered to prevent coronary artery abnormality (CAA). Here, we present several cases of Kawasaki disease in children treated with plasma exchange (PE).\u0000\u0000\u0000\u0000We retrospectively reviewed the medical records of 5 subjects who had been unresponsive to IVIG treatments and treated with PE consecutively.\u0000\u0000\u0000\u0000The fever subsided and the other clinical manifestations were improved after PE in all cases. In two cases, giant coronary artery aneurysms were detected on echocardiographic follow-up study.\u0000\u0000\u0000\u0000PE should be a method with promising efficacy and might be considered in refractory Kawasaki disease patients for whom other treatment options have failed.\u0000\u0000","PeriodicalId":208326,"journal":{"name":"Kawasaki Disease","volume":"28 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133489535","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kawasaki DiseasePub Date : 2023-06-01DOI: 10.59492/kd.2023.1.1.e9
Min Seob Song, Hye Su Hwang
{"title":"Successful Treatment with Infliximab in Intravenous Immunoglobulin-Resistant Kawasaki Disease with High Procalcitonin Level: A Case Report","authors":"Min Seob Song, Hye Su Hwang","doi":"10.59492/kd.2023.1.1.e9","DOIUrl":"https://doi.org/10.59492/kd.2023.1.1.e9","url":null,"abstract":"\u0000\u0000Procalcitonin (PCT) is modulated by sepsis-related cytokines such as tumor necrosis factor (TNF)-α and interleukins. Therefore, TNF-α blockade, infliximab may be useful for intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) with high PCT level. We report a case of IVIG-resistant KD with elevated PCT level who was successfully treated with infliximab. This case report suggests that the treatment of infliximab may be useful for IVIG-resistant KD with high PCT level.\u0000","PeriodicalId":208326,"journal":{"name":"Kawasaki Disease","volume":"20 4-5 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116651818","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kawasaki DiseasePub Date : 2023-06-01DOI: 10.59492/kd.2023.1.1.e6
Jongmin Seo
{"title":"Advancing Risk Stratification of Coronary Artery Aneurysms Caused by Kawasaki Disease Using Hemodynamics Analysis and Computational Fluid Dynamics","authors":"Jongmin Seo","doi":"10.59492/kd.2023.1.1.e6","DOIUrl":"https://doi.org/10.59492/kd.2023.1.1.e6","url":null,"abstract":"\u0000\u0000This article reviews previous studies on the risk assessment of coronary artery aneurysms (CAA) caused by Kawasaki disease (KD) using the computational fluid dynamics (CFD) simulation. Patient-specific CFD is an emerging tool that provides detailed three-dimensional hemodynamic information, not available from current standard imaging techniques or invasive measures. The reviewed CFD studies on KD CAA used Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) images to reconstruct the three-dimensional anatomical geometry consisting of the aorta and coronary arteries. On the inlet and outlet of the anatomical model, physiologic boundary conditions were prescribed to match the measured patient-specific blood pressure or flow data, supplemented by physiologic rules informed by population studies. Hemodynamic variables such as the blood velocity, wall shear stress, oscillatory shear index, fractional flow reserve, and particle residence time, were then quantified from the simulation results. The studies reviewed revealed strong correlations between these hemodynamic parameters and clinical outcomes, suggesting that hemodynamic metrics could be useful for treatment planning for KD patients. We discuss the limitations of the reviewed studies including small sample sizes, a narrow focus on thrombosis, and the use of different imaging modalities. The article suggests future research directions for CFD studies of KD that address these limitations.\u0000","PeriodicalId":208326,"journal":{"name":"Kawasaki Disease","volume":"75 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121259850","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kawasaki DiseasePub Date : 2023-06-01DOI: 10.59492/kd.2023.1.1.e4
Eun Jung Shin
{"title":"Kawasaki Disease and Multisystem Inflammatory Syndrome in Children","authors":"Eun Jung Shin","doi":"10.59492/kd.2023.1.1.e4","DOIUrl":"https://doi.org/10.59492/kd.2023.1.1.e4","url":null,"abstract":"\u0000\u0000Kawasaki disease is a vasculitis that presents with fever and affects medium-sized arteries throughout the body. Although the etiology and pathophysiology of Kawasaki disease remain unknown, the emergence of the multisystem inflammatory syndrome in children (MIS-C) in 2020 is of significant interest due to its similarity in symptoms of Kawasaki disease and its association with SARS-CoV-2. Research on the pathophysiology of MIS-C is still ongoing and is expected to contribute to the explanation of the symptoms of Kawasaki disease in the future. The diagnosis and definition, clinical features, and treatment methods of Kawasaki disease and MIS-C, which may have such an important relationship, will be summarized.\u0000","PeriodicalId":208326,"journal":{"name":"Kawasaki Disease","volume":"29 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116635446","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kawasaki DiseasePub Date : 2023-06-01DOI: 10.59492/kd.2023.1.1.e10
J. Oh
{"title":"A Case of a Third Attack of Recurrent Node-First Kawasaki Disease with Retropharyngeal Edema","authors":"J. Oh","doi":"10.59492/kd.2023.1.1.e10","DOIUrl":"https://doi.org/10.59492/kd.2023.1.1.e10","url":null,"abstract":"\u0000\u0000Kawasaki disease (KD) is currently the leading cause of acquired heart disease in children. Since the diagnosis of KD still depends on clinical features excluding underlying diseases, it remains a challenge if clinical signs of KD and other infections overlap. We experienced a case of a 6-year-old boy who initially presented with a severe suppurative neck infection. The patient repeatedly showed similar clinical findings three times, with severe inflammation of the skin on the enlarged cervical lymph nodes, but remained refractory to antibiotics treatment. Cervical computed tomography revealed retropharyngeal edema, and in the operative field, the patient showed a serous discharge from the edematous retropharyngeal wall. All underlying microbial pathogen tests were negative. The patient subsequently presented typical features of KD on the 6th day from the onset of fever. He was treated with intravenous immunoglobulin and aspirin, and he antibiotics were discontinued. Here, we report a case of a third attack of recurrent node-first KD with a literature review.\u0000","PeriodicalId":208326,"journal":{"name":"Kawasaki Disease","volume":"2 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114337101","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kawasaki DiseasePub Date : 2023-06-01DOI: 10.59492/kd.2023.1.1.e7
Jong-Won Choi, G. Kim
{"title":"Epidemiology and Clinical Outcomes of Refractory Kawasaki Disease in South Korea","authors":"Jong-Won Choi, G. Kim","doi":"10.59492/kd.2023.1.1.e7","DOIUrl":"https://doi.org/10.59492/kd.2023.1.1.e7","url":null,"abstract":"\u0000\u0000\u0000\u0000Refractory Kawasaki disease (KD) is defined as KD resistant to initial intravenous immunoglobulin (IVIG) therapy. The proportion of refractory KD is reported to be 10%−20%, and the risk of coronary arterial aneurysm (CAA) is increased in refractory KD. We analyzed data of Korean nationwide surveys on KD to know the epidemiology, treatment modalities, and clinical outcomes of refractory KD in South Korea.\u0000\u0000\u0000\u0000We analyzed the data of three triennial Korean nationwide surveys on KD from 2009 to 2017, and selected patients with refractory KD who had persistent or recrudescent fever at least 36 hours after completion of initial IVIG therapy.\u0000\u0000\u0000\u0000Among a total of 42,705 KD patients from 2009 through 2017, 5,581 (13.1%) had refractory KD. The annual proportions of refractory KD were between 10.1% and 18.7%, which showed significant changes with the nadir in 2013 and increased proportions in 2015−2017 (P < 0.05). Treatment modalities for refractory KD were second IVIG in 86.2%, third or more IVIG in 3.8%, corticosteroids in 34.4%, infliximab in 3.7%, methotrexate in 1.0%, plasmapheresis in 0.1%, and cyclosporine in 0.02%. The use of third or more IVIG showed a decreasing trend, and the use of corticosteroids and infliximab showed an increasing trend. The incidence of CAA was significantly higher in refractory KD patients than in total KD patients (5.5% vs 1.8%; P < 0.05).\u0000\u0000\u0000\u0000The overall proportion of refractory KD was 13.1% from 2009 through 2017 in South Korea. The annual proportions of refractory KD showed significant changes with increased proportions in recent years. Treatment modalities for refractory KD showed a decreasing trend in the use of third or more IVIG and an increasing trend in the use of corticosteroids and infliximab. The incidence of CAA was significantly higher in refractory KD patients than in total KD patients.\u0000\u0000","PeriodicalId":208326,"journal":{"name":"Kawasaki Disease","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133387055","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kawasaki DiseasePub Date : 2023-06-01DOI: 10.59492/kd.2023.1.1.e3
D. Jeong
{"title":"Kawasaki Disease and Innate Immunity","authors":"D. Jeong","doi":"10.59492/kd.2023.1.1.e3","DOIUrl":"https://doi.org/10.59492/kd.2023.1.1.e3","url":null,"abstract":"\u0000\u0000Kawasaki disease (KD) is a self-limited febrile illness diagnosed based on various clinical manifestations, including fever. The major complication of KD is systemic vasculitis, particularly involving the coronary artery. The etiology of KD is not clear, but the pathogenesis may be related to excessive activation of the immune system. Immune modulation for KD treatment, including intravenous immunoglobulin (IVIG), is based on the control of immune hyperactivation. Many studies have reported a genetic susceptibility to KD, which is related to immune cell activation (ITPKC, CAPS3, BLK, FCGR2A, etc.). The innate immunity of humans begins to recognize and react through pattern recognition receptors against pathogen-associated molecular patterns (PAMPs) or damage-associated molecular patterns (DAMPs). Among receptors, the nucleotide-binding domain and leucine-rich repeat-containing receptor (NLRP) or NOD-like receptor (NLRs) in the cytosol contribute to inflammation as a key component. Cytosolic protein complexes called inflammasomes, assembled by NLRP, activate proinflammatory caspase 1 and 11, which ultimately produce IL-1β and IL-18 or induce cell death. IL-1 serves as an initiator for the recruitment of immune cells and the inflammatory response. Systemic vasculitis, such as Behcet disease, may be related to IL-1 polymorphism, and the expression of NLRP3. Coronary arteritis in KD is associated with an innate immune response, including IL-1. The suggested evidence of innate immunity in KD is related to increased neutrophils and monocytes, high levels of γδT cells, macrophage infiltration in coronary arteries and skin, elevated DAMPs such as S100 or HMGB1, and is sometimes associated with hyperactive innate immunity. The gene of the IL-1 pathway may be related to IVIG-resistant KD, and a clinical trial with IL-1 antagonist is currently ongoing. Adjunctive therapy in KD consists of various strategies, including second IVIG, steroids, and TNF inhibitors. The rationale for adjunctive therapy is based on immune hyperactivation with hypercytokinemia or immune modulation. Further efforts are needed to understand innate immunity and KD, especially in IVIG-resistant cases.\u0000","PeriodicalId":208326,"journal":{"name":"Kawasaki Disease","volume":"14 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123815056","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}