背景:川崎病(KD)是一种主要发生在5岁以下婴幼儿的自限性急性系统性血管炎。尽管使用乙酰水杨酸(AAS)联合静脉注射免疫球蛋白(IVIG)仍然是KD的标准治疗方法,病因,KD的遗传易感基因和致病因素仍未阐明。
目的:目前治疗KD的障碍包括缺乏用于早期诊断的标准临床和遗传标记,AAS(Reye综合征)可能的严重副作用,以及对IVIG治疗耐药的难治性KD病例,因此,这篇综述重点介绍了遗传易感基因鉴定的最新进展,环境因素,KD的诊断标志物和辅助药物干预。
结果:随着不同方面KD发展的全面更新,我们目前的生物信息学数据提示CASP3、CD40和TLR4可能是KD的致病因子或诊断标志物。此外,还总结了一系列可能通过靶向不同的KD分子靶标作为KD辅助治疗的草药。
结论:借助现代药理研究和技术,预计新的治疗方法,特别是针对KD的精确临床标志物的活性草药可以被开发用于疾病的准确诊断和治疗。
BACKGROUND: Kawasaki disease (KD) is a self-limiting acute systemic vasculitis occur mainly in infants and young children under 5 years old. Although the use of acetylsalicylic acid (AAS) in combination with intravenous immunoglobulin (IVIG) remains the standard therapy to KD, the etiology, genetic susceptibility genes and pathogenic factors of KD are still un-elucidated.
OBJECTIVE: Current obstacles in the treatment of KD include the lack of standard clinical and genetic markers for early diagnosis, possible severe side effect of AAS (Reye\'s syndrome), and the refractory KD cases with resistance to IVIG therapy, therefore, this review has focused on introducing the current advances in the identification of genetic susceptibility genes, environmental factors, diagnostic markers and adjuvant pharmacological intervention for KD.
RESULTS: With an overall update in the development of KD from different aspects, our current bioinformatics data has suggested CASP3, CD40 and TLR4 as the possible pathogenic factors or diagnostic markers of KD. Besides, a list of herbal medicines which may work as the adjunct therapy for KD via targeting different proposed molecular targets of KD have also been summarized.
CONCLUSIONS: With the aid of modern pharmacological research and technology, it is anticipated that novel therapeutic remedies, especially active herbal chemicals targeting precise clinical markers of KD could be developed for accurate diagnosis and treatment of the disease.