In some cases, patients do not fulfill the classic criteria for Kawasaki disease and are classified as having incomplete (atypical) disease. CD40 signaling leads to isotype switching and autoantibody production in B cells and in T-cell priming, altering TCR expression through the expression and nuclear translocation of recombinases, which increases the risk of developing autoimmunity [173]. Activity of NFATs is regulated by phosphorylation. He, F. Gao, Q. Liu, and Q. Yi, “Association of the resistin gene promoter region polymorphism with Kawasaki disease in Chinese children,”, S. Shrestha, H. Wiener, A. Shendre et al., “Role of activating, S. Taniuchi, M. Masuda, M. Teraguchi et al., “Polymorphism of Fc, S. Onoyama, K. Ihara, Y. Yamaguchi et al., “Genetic susceptibility to Kawasaki disease: analysis of pattern recognition receptor genes,”, M. T. Lin, J. K. Wang, J. I. Yeh et al., “Clinical implication of the C allele of the, Y. Onouchi, “Identification of susceptibility genes for Kawasaki disease,”, Q. Peng, C. Chen, Y. Zhang et al., “Single-nucleotide polymorphism rs2290692 in the 3′UTR of, Y. Onouchi, Y. Suzuki, H. Suzuki et al., “, C. Shimizu, S. Jain, M. L. Hibberd et al., “Transforming growth factor-, Y. M. Choi, K. S. Shim, K. L. Yoon et al., “Transforming growth factor beta receptor II polymorphisms are associated with Kawasaki disease,”, K. Yamamura, K. Ihara, K. Ikeda, H. Nagata, Y. Mizuno, and T. Hara, “Histo-blood group gene polymorphisms as potential genetic modifiers of the development of coronary artery lesions in patients with Kawasaki disease,”, J. J. Kim, Y. M. Hong, S. W. Yun et al., “Assessment of risk factors for Korean children with Kawasaki disease,”, H.-C. Kuo, Y. Onouchi, Y.-W. Hsu et al., “Polymorphisms of transforming growth factor-, H. C. Kuo, H. R. Yu, S. H. H. Juo et al., “, W. B. Breunis, S. Davila, C. Shimizu et al., “Disruption of vascular homeostasis in patients with Kawasaki disease: involvement of vascular endothelial growth factor and angiopoietins,”, W. B. Breunis, M. H. Biezeveld, J. Geissler et al., “Vascular endothelial growth factor gene haplotypes in Kawasaki disease,”, K. C. Hsueh, Y. J. Lin, J. S. Chang et al., “Association of vascular endothelial growth factor C-634 G polymorphism in Taiwanese children with Kawasaki disease,”, H. Kariyazono, T. Ohno, V. Khajoee et al., “Association of vascular endothelial growth factor (VEGF) and VEGF receptor gene polymorphisms with coronary artery lesions of Kawasaki disease,”, Y. Y. Hsieh, C. C. Chang, C. M. Hsu, S. Y. Chen, W. H. Lin, and F. J. Tsai, “Major histocompatibility complex class I chain-related gene polymorphisms: associated with susceptibility to kawasaki disease and coronary artery aneurysms,”, F. Y. Huang, Y. J. Lee, M. R. Chen et al., “Polymorphism of transmembrane region of MICA gene and Kawasaki disease,”, Y. Y. Hsieh, Y. J. Lin, C. C. Chang et al., “Human lymphocyte antigen B-associated transcript 2, 3, and 5 polymorphisms and haplotypes are associated with susceptibility of Kawasaki disease and coronary artery aneurysm,”, S. Shrestha, H. W. Wiener, A. K. Olson et al., “Functional, K. C. Hsueh, Y. J. Lin, J. S. Chang et al., “Association of interleukin-10 A-592C polymorphism in Taiwanese children with Kawasaki disease,”, K. S. Hsieh, T. J. Lai, Y. T. Hwang et al., “IL-10 promoter genetic polymorphisms and risk of Kawasaki disease in Taiwan,”, J. Yang, C. R. Li, Y. It takes part in expression of numerous cytokines and adhesion molecules which are critical elements involved in the regulation of immune responses. A lower “ IL-2 binds to IL-2 receptors at the T Cells surface to drive clonal expansion of the activated cell that induces autocrine proliferation [124]. Kawasaki disease is the most common childhood vasculitis in the USA and the most common cause of acquired cardiac disease in children in developed countries. Additionally, CD40L activates nearby B cells that express corresponding receptor CD40. Through GeneGo and DAVID analysis, we speculated that NF-AT signaling and leukocyte interactions combined with another transcription factor NF-κB may play an important role in pathological damage of KD. Tachycardia and gallop rhythm (secondary to myocarditis) may occur. The etiology of KD is unknown. ” value of the hypergeometric intersection. It usually presents in infancy and early childhood with 85% of those affected are less than 5 years of age. Even with high dose IVIG ~ 5% of patient may develop coronary dilation (ectasia) and ~ 1% may develop coronary aneurysms. Caspase-3 (CASP3) is a key molecule of activation-induced cell death (AICD) [164]; it is profoundly related to the apoptosis of immune cells. We performed a computerized search of Ovid, Google Scholar, and PubMed databases up to September 2012 and reviewed cited references to identify the relevant studies. On the cell surface, plasmin can activate a number of matrix metalloproteinases (MMPs) MMP1, MMP13 [115]. Kawasaki disease is a generalized vasculitis with a predilection for the coronary arteries. Pediatrics 114 (6) December 2004, pp. Kawasaki disease: current aspects on aetiopathogenesis and therapeutic management. The fever typically lasts for more than five days and is not affected by usual medications. Then, MHC class II peptides activate the T-cell receptor (TCR alpha/beta-CD3 complex) that starts a signal leading to the increase in cytosolic Ca(II) through both the transient release of calcium from intracellular stores and the influx of calcium through Ca(II) channels. May still be at higher risk for altered lipid metabolism and early development of atherosclerosis most the! 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