川崎病(KD)是一种影响5岁以下儿童的系统性血管炎。生命的早期阶段以快速的体细胞生长和细胞增殖以及显性先天免疫系统的免疫不成熟为特征。KD中的冠状动脉并发症是儿童最常见的获得性心脏病,然而KD的诊断仍取决于临床诊断标准.有光泽的红唇和结膜注射是儿科医生初步诊断KD的特征性体征;然而,鲜为人知的是为什么这些是如此的特点。KD的诊断标准似乎分散在看似无关紧要的身体系统中,例如眼睛,嘴唇,皮肤,和心脏。KD被归类为结缔组织疾病。最近,红细胞(RBC)已成为先天免疫应答的重要调节剂。据报道,红细胞参与真皮成纤维细胞的细胞外基质重塑和基质金属蛋白酶(MMP)表达上调。此外,与纤维化相关的成纤维细胞生长因子和microRNAs在KD中引起关注。KD的主要体征出现在粘膜皮肤交界处。在经历上皮-间质转化(EMT)的组织中头颈部区域丰富。间质性心脏炎和瓣膜功能不全以及冠状动脉病变可能会使KD复杂化。这些病变存在于通过EMT起源于心外膜祖细胞的组织中。回顾了最近对KD的研究,我们推测,KD的征象存在于角化和非角化复层鳞状上皮之间的边界,在这些上皮中,EMT仍在进行中,因为在快速的体细胞生长中,RBC被招募作为先天免疫应答并防止粘膜过度纤维化.KD在完成体细胞生长和免疫成熟的成年人中几乎不存在。在这次审查中,我们试图解释KD临床表现的原因,并在KD儿童的躯体生长和免疫系统成熟过程中从EMT的角度寻找诊断线索之间的联系。
Kawasaki disease (KD) is a systemic vasculitis affecting children younger than 5 years of age. Early period in life is marked by rapid somatic growth with cell proliferation and immaturity of the immunity with dominant innate immune system. Coronary complications in KD are the most common acquired heart disease in children, yet the diagnosis of KD still depends on the clinical diagnostic criteria. Glossy red lips and conjunctival injection are characteristic signs enabling pediatricians to make the initial diagnosis of KD; however, little is known why these are so characteristic. The diagnostic criteria of KD seem to be scattered in seemingly irrelevant body systems such as the eyes, lips, skin, and heart. KD is classified as a connective tissue disease. Recently, red blood cells (RBCs) have emerged as important modulators in innate immune response. RBCs are reported to participate in extracellular matrix remodeling and upregulating matrix metalloproteinase (MMP) expression in dermal fibroblasts. Also, fibroblast growth factors and microRNAs associated with fibrosis are drawing attention in KD. The cardinal signs of KD appear at the border of muco-cutaneous junction. Head and neck regions are abundant in tissues undergoing epithelial-to-mesenchymal transition (EMT). Interstitial carditis and valve insufficiency as well as coronary arterial lesions may complicate KD, and these lesions present in tissues that originated from epicardial progenitor cells by EMT. Having reviewed the recent research on KD, we presume that the signs of KD present at borders between keratinized and non-keratinized stratified squamous epithelium where the EMT is still ongoing for the rapid somatic growth where RBCs are recruited as an innate immune response and to prevent excessive fibrosis in mucosa. KD presents scarcely in adults with somatic growth and immune maturation completed. In this
review, we attempted to explain the reasons for the clinical manifestations of KD and to search for a link among the diagnostic clues in the perspective of EMT during the somatic growth and immune system maturation in children with KD.