Gupta-Malhotra Monesha, Rao P Syamasundar
University of Texas Houston Medical School & Memorial Hermann Children's Hospital, Houston, Texas, USA.
Indian J Pediatr. 2005 Jul;72(7):621-9. doi: 10.1007/BF02724189.
The etiology of Kawasaki disease (KD) remains unknown despite several years of dedicated research in this direction. Recently coronavirus infection and genetic polymorphisms have been implicated. Since first description of the disease there have been few changes in the diagnostic criteria except for newer recommendations of fever of at least 4 instead of 5 days duration. Recently, Echocardiography Criteria and Laboratory Criteria have been added to aid in the diagnosis of incomplete KD where all the historical diagnostic criteria are not present; this is now called the "incomplete form of KD" as opposed to "atypical form of KD". The word "atypical" is reserved for unusual presentations of KD such as those with hemophagocytic syndrome or nerve palsy. The treatment of KD includes infusion of high dose immunoglobulin. Patients non-responsive to immunoglobulin therapy are labeled as having "immunoglobulin resistant KD". The treatment of immunoglobulin resistant KD can be challenging and new therapies that have tried with some success. Late outcomes after 4 decades of treating these patients have recently been published. There has been some concern about increased risk for premature atherosclerosis in patients with childhood KD who had coronary artery abnormalities.
尽管在这一方向上进行了数年的专门研究，但川崎病（KD）的病因仍然不明。最近，冠状病毒感染和基因多态性被认为与之有关。自该疾病首次被描述以来，除了将发热持续时间至少4天而非5天作为新的推荐标准外，诊断标准几乎没有变化。最近，超声心动图标准和实验室标准被补充进来，以帮助诊断不具备所有既往诊断标准的不完全KD；现在这被称为“不完全KD型”，以区别于“非典型KD型”。“非典型”一词用于描述KD的不寻常表现，如伴有噬血细胞综合征或神经麻痹的情况。KD的治疗包括输注高剂量免疫球蛋白。对免疫球蛋白治疗无反应的患者被标记为患有“免疫球蛋白抵抗性KD”。免疫球蛋白抵抗性KD的治疗可能具有挑战性，一些新疗法已取得了一定成功。在对这些患者进行了40年的治疗后，近期公布了其晚期预后情况。人们一直担心，患有冠状动脉异常的儿童KD患者发生过早动脉粥样硬化的风险会增加。
