Pathogenesis and management of Kawasaki disease.
Pathogenesis and management of Kawasaki disease.
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DOI：
10.1586/eri.09.109
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发表时间：
2010-02
影响因子：
5.7
通讯作者：
Shulman ST
中科院分区：
文献类型：
--
作者：
Rowley AH;Shulman ST
川崎是一种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，在以前健康的儿童。临床和流行病学特征支持一种感染性病因，但在Tomisaku川崎首次发现KD后的40年里，其病因仍不清楚。寻找KD的病因是儿科研究的优先事项。我们回顾了KD独特的免疫病理学，并描述了目前的治疗方法。新的研究已经导致在急性KD组织中鉴定出病毒样细胞质包涵体;这一发现可能导致鉴定难以捉摸的病原体，并导致KD诊断和治疗的重大进展。目前对急性KD的治疗是基于静脉注射免疫球蛋白（IVIG）与大剂量阿司匹林的前瞻性、多中心治疗试验。最佳治疗是在急性发热期诊断后尽快给予2 g/kg IVIG和高剂量阿司匹林，随后给予低剂量阿司匹林，直至随访超声心动图显示无冠状动脉异常。增加一个剂量的静脉脉冲类固醇尚未被证明是有益的。对于10-15%的难治性KD患者，几乎没有对照数据可用。选择包括重复IVIG（我们的首选），静脉内脉冲甲基强的松龙，或英夫利昔单抗（Remicade®）的3天疗程。轻度至中度冠状动脉异常的患者应接受抗血小板药物，如低剂量阿司匹林（3-5 mg/kg/天）或氯吡格雷（1 mg/kg/天，最高75 mg），巨大（直径约8 mm）或多发性冠状动脉瘤患者应接受抗血小板药物与抗凝剂，如华法林或低分子量肝素。急性冠状动脉阻塞需要通过外科手术或经皮介入治疗进行急性溶栓治疗。
Kawasaki disease (KD) is an acute systemic inflammatory illness of young children that can result in coronary artery aneurysms, myocardial infarction and sudden death in previously healthy children. Clinical and epidemiologic features support an infectious cause, but the etiology remains unknown four decades after KD was first identified by Tomisaku Kawasaki. Finding the cause of KD is a pediatric research priority. We review the unique immunopathology of KD and describe the current treatment. New research has led to identification of viral-like cytoplasmic inclusion bodies in acute KD tissues; this finding could lead to identification of the elusive etiologic agent and result in significant advances in KD diagnosis and treatment. Current management of acute KD is based upon prospective, multicenter treatment trials of intravenous immunoglobulin (IVIG) with high-dose aspirin. Optimal therapy is 2 g/kg IVIG with high-dose aspirin as soon as possible after diagnosis during the acute febrile phase of illness, followed by low-dose aspirin until follow-up echocardiograms indicate a lack of coronary abnormalities. The addition of one dose of intravenous pulse steroid has not been shown to be beneficial. For the 10–15% of patients with refractory KD, few controlled data are available. Options include repeat IVIG (our preference), a 3-day course of intravenous pulse methylprednisolone, or infliximab (Remicade®). Patients with mild-to-moderate coronary abnormalities should receive an antiplatelet agent such as low-dose aspirin (3–5 mg/kg/day) or clopidogrel (1 mg/kg/day up to 75 mg), and those with giant (~8 mm diameter) or multiple coronary aneurysms should receive an antiplatelet agent with an anticoagulant such as warfarin or low-molecular-weight heparin. Acute coronary obstruction requires acute thrombolytic therapy with a surgical or percutaneous interventional procedure.
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影响因子：
4.7
作者：
Nakamura Y;Yashiro M;Uehara R;Oki I;Watanabe M;Yanagawa H
通讯作者：
Yanagawa H
影响因子：
168.9
作者：
ENRIA, DA;BRIGGILER, AM;MAIZTEGUI, JI
通讯作者：
MAIZTEGUI, JI
影响因子：
3.6
作者：
Jibiki, T;Terai, M;Kohno, Y
通讯作者：
Kohno, Y
影响因子：
3.6
作者：
Miura, M;Garcia, FL;Rowley, AH
通讯作者：
Rowley, AH
影响因子：
158.5
作者：
Newburger, Jane W.;Sleeper, Lynn A.;Sundel, Robert P.
通讯作者：
Sundel, Robert P.