Recent advances in the understanding and management of kawasaki disease.
Recent advances in the understanding and management of kawasaki disease.
复制标题
DOI：
10.1007/s11908-010-0091-6
复制
发表时间：
2010-03
影响因子：
3.1
通讯作者：
Shulman ST
中科院分区：
文献类型：
--
作者：
Rowley AH;Shulman ST
川崎病（Kawasaki disease， KD）是一种儿童期急性全身性炎症性疾病，可导致冠状动脉瘤、心肌梗死和猝死。临床和流行病学数据表明，病因是一种未知的感染因子。我们发现急性KD患者动脉组织中存在寡克隆IgA免疫应答。KD动脉壁中普遍存在的IgA抗体的合成版本可识别急性KD纤毛支气管上皮和其他炎症KD组织中的细胞质包涵体。光镜和电镜研究表明，包涵体与病毒蛋白和RNA的聚集体一致，可能是由KD病原体形成的。KD易感性可能是多基因的。对丙种球蛋白无反应的治疗通常包括额外的静脉注射免疫球蛋白、甲基强的松龙和/或英夫利昔单抗。目前迫切需要更多关于KD发病机制的数据，以便为那些发生冠状动脉异常风险最高的患者提供其他治疗靶点。
Kawasaki disease (KD) is an acute systemic inflammatory illness of childhood that can result in coronary artery aneurysms, myocardial infarction, and sudden death. Clinical and epidemiologic data point to an unknown infectious agent as the cause. We discovered that an oligoclonal IgA immune response is present in arterial tissue in acute KD. Synthetic versions of prevalent IgA antibodies in the KD arterial wall identify cytoplasmic inclusion bodies in acute KD ciliated bronchial epithelium and other inflamed KD tissues. Light and electron microscopic studies show that the inclusion bodies are consistent with aggregates of viral protein and RNA, and are likely formed by the KD etiologic agent. KD susceptibility is likely to be polygenic. Treatment of gammaglobulin non-responders usually consists of additional intravenous immunoglobulin, methylprednisolone, and/or infliximab. Additional data regarding KD pathogenesis are urgently needed to provide other targets for therapy for those patients at highest risk of developing coronary artery abnormalities.