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      Aetiological Significance of Infectious Stimuli in Kawasaki Disease

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          Kawasaki disease (KD) is a pediatric vasculitis syndrome that is often involves coronary artery lesions (e. g., coronary artery aneurysms). Although its causal factors and entire pathogenesis remain elusive, the available evidence indicates that the pathogenesis of KD is closely associated with dysregulation of immune responses to various viruses or microbes. In this short review, we address several essential aspects of the etiology of KD with respect to the immune response to infectious stimuli: 1) the role of viral infections, 2) the role of bacterial infections and the superantigen hypothesis, 3) involvement of innate immune response including pathogens/microbe-associated molecular patterns and complement pathways, and 4) the influence of genetic background on the response to infectious stimuli. Based on the clinical and experimental evidence, we discuss the possibility that a wide range of microbes and viruses could cause KD through common and distinct immune processes.

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          Most cited references 132

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          [Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children].

           B Kawasaki (1967)
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            ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms.

            Kawasaki disease is a pediatric systemic vasculitis of unknown etiology for which a genetic influence is suspected. We identified a functional SNP (itpkc_3) in the inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) gene on chromosome 19q13.2 that is significantly associated with Kawasaki disease susceptibility and also with an increased risk of coronary artery lesions in both Japanese and US children. Transfection experiments showed that the C allele of itpkc_3 reduces splicing efficiency of the ITPKC mRNA. ITPKC acts as a negative regulator of T-cell activation through the Ca2+/NFAT signaling pathway, and the C allele may contribute to immune hyper-reactivity in Kawasaki disease. This finding provides new insights into the mechanisms of immune activation in Kawasaki disease and emphasizes the importance of activated T cells in the pathogenesis of this vasculitis.
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              Staphylococcal and streptococcal superantigen exotoxins.

              SUMMARY This review begins with a discussion of the large family of Staphylococcus aureus and beta-hemolytic streptococcal pyrogenic toxin T lymphocyte superantigens from structural and immunobiological perspectives. With this as background, the review then discusses the major known and possible human disease associations with superantigens, including associations with toxic shock syndromes, atopic dermatitis, pneumonia, infective endocarditis, and autoimmune sequelae to streptococcal illnesses. Finally, the review addresses current and possible novel strategies to prevent superantigen production and passive and active immunization strategies.

                Author and article information

                Front Pediatr
                Front Pediatr
                Front. Pediatr.
                Frontiers in Pediatrics
                Frontiers Media S.A.
                28 June 2019
                : 7
                1Central Research Laboratory, Graduate School of Medical Science, Kyoto Prefectural University of Medicine , Kyoto, Japan
                2Department of Pediatrics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine , Kyoto, Japan
                3Pediatric Cardiology and Kawasaki Disease Center, Uji-Tokushukai Medical Center , Kyoto, Japan
                4Faculty of Life and Medical Sciences, Doshisha University , Kyoto, Japan
                Author notes

                Edited by: Hiromichi Hamada, Tokyo Women's Medical University Yachiyo Medical Center, Japan

                Reviewed by: Ying-Hsien Huang, Kaohsiung Chang Gung Memorial Hospital, Taiwan; Hiroyuki Suzuki, Wakayama Medical University, Japan

                *Correspondence: Akihiro Nakamura nakam993@

                This article was submitted to Pediatric Immunology, a section of the journal Frontiers in Pediatrics

                Copyright © 2019 Nakamura, Ikeda and Hamaoka.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                Figures: 2, Tables: 0, Equations: 0, References: 132, Pages: 9, Words: 7596
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