Kawasaki disease.
Kawasaki disease.
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DOI：
10.1148/radiology.203.1.218
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发表时间：
1994
期刊：
影响因子：
--
通讯作者：
L. Jonides;C. Rudy;S. Walsh
中科院分区：
文献类型：
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作者：
L. Jonides;C. Rudy;S. Walsh
川崎是一种病因不明的急性发热性皮肤粘膜感染综合征，主要累及5岁以下婴幼儿。诊断川崎的主要体征和症状有6种，包括：（a）发热持续5天以上，（B）外周改变，如初发期掌跖发红、硬结性水肿，恢复期指端膜性脱沉，（c）点状渗出，（d）双侧结膜充血，（e）双侧结膜充血，（f）双侧结膜充血，（（e）嘴唇的变化如发红，口腔的变化如草莓舌和口腔及咽粘膜的弥漫性充血;和（f）急性非化脓性颈部淋巴结病。此外，其他临床上重要的症状或发现也涉及诊断。川崎病的诊断至少应具备6项检查中的5项，但如果有4项体征或症状，则可通过二维超声心动图或冠状动脉造影发现冠状动脉瘤而作出诊断。如果检测到四种体征或症状，则诊断为非典型川崎病;在北美，如果冠状动脉造影超声心动图显示动脉炎引起的冠状动脉改变，则三种或更少的发现被认为是非典型川崎病。婴儿结节性阴茎动脉炎（现称为婴儿多触角炎）的罕见尸检结果不能与川崎的尸检结果区分开来。因此，川崎病并不是一种新疾病;然而，在其被确立之前，在临床环境中没有方法来识别这种疾病。自1970年以来，在日本全国范围内进行了12次调查; 1992年底，报告的川崎病例数为116，848例。自1970年以来，病例数增加; 1979年、1982年和1986年发生流行病。自1987年以来，没有发生任何流行病。每年的发病率恒定在5，000 - 6，000例。据调查，复发率为3%-5%。1994年，在一项对4，560例病例的研究中，发现复发率为每1，000人年5.21例。发现2岁以下患者复发的风险较高;静脉注射丙种球蛋白治疗未发现是风险因素。当考虑川崎病的病因时，复发是一个重要因素;因此，该疾病可能介于胶原血管病和感染性疾病之间。川崎病的实验室检查结果和临床特征提示细菌感染。然而，细菌还没有被发现。此外，抗生素的使用一直是无效的，并且使用糖皮质激素的临床试验（其最初被认为是有效的）已被发现可能增加冠状动脉瘤发展的风险。类固醇可引起动脉瘤内血小板聚集。阿司匹林因其抗炎和抗血栓作用而成为治疗川崎最合适的药物，在开始使用大剂量丙种球蛋白治疗之前，阿司匹林是最常用的药物。目前，治疗主要包括阿司匹林和大剂量丙种球蛋白。之前，给予的丙种球蛋白剂量为400 mg/kg！一天4或5天。然而，由于在某些情况下住院时间缩短，并且由于丙种球蛋白更有效，因此目前的治疗方案是一次给药2 g/kg。它还没有确定为什么丙种球蛋白是有效的;然而，它...
Kawasaki disease is an acute, febnile, mucocutaneous-affecting syndrome with no known cause and affects mainly infants and children younger than 5 years. There are six principal signs and symptoms involved in the diagnosis of Kawasaki disease, which include (a) fever that persists for 5 days or longer;(b) changes in the peripheral extremities such as reddening of palms and soles and indurative edema in the initial stage and membranous desqua-mation from the fingertips in the convalescent stage;(c) potymomphous exanthema;(d) bilateral conjunctivat congestion;(e) changes in the lips such as redden-ing, and changes in the oral cavity such as strawberry tongue and diffuse injection of oral and pharyngeal mucosa; and (f) acute nonpurulent cervical lymphadenopathy. In addition, other clinically important symptoms or findings are involved in diagnosis. For a diagnosis of Kawasaki disease to be made, a minimum of five of the six findings should be pres-ent. The diagnosis can be made if four signs or symp-toms are present, however, when coronary aneurysm is detected at two-dimensional echocardiography or coronary angiography. If four signs or symptoms are detected, the diagnosis is atypical Kawasaki disease; in North America, the presence of three or fewer findings is considered atypical Kawasaki disease if echocardiography on coronary angiography reveal coronary artery changes caused by artenitis. Rare autopsy findings of infantile peniarteritis nodosa, now termed infantile polyantenitis, cannot be distinguished from those of Kawasaki disease. Therefore, Kawasaki disease is not new; however, there were previously no means to identify this dis-ease in a clinical setting until it was established. Since 1970, 12 surveys have been performed nationwide in Japan; at the end of 1992, the number of cases of Kawasaki disease reported was 116,848. The number of cases increased since 1970; epidemics occurred in 1979, 1982, and 1986. Since 1987, no epidemics have occurred. The annual incidence is constant at 5,000-6,000 cases. According to surveys, the recurrence rate is 3%-5%. In 1994, in a study of 4,560 cases, the recurrence rate was found to be 5.21 per 1,000 person-years. The risk of recurrence was found to be high in patients younger than 2 years; treatment with intravenous administration of gamma globulin was not found to be a risk factor. When the cause of Kawasaki disease is considered, recurrence is an important factor; thus the disease may be clas-sified somewhere between a collagen vascular dis-ease and an infectious disease. Laboratory findings and clinical features of Ka-wasaki disease suggest a bacterial infection. How-ever, bacteria have not been discovered. Further-more, use of antibiotics has been ineffective, and clinical trials with use of glucocorticoids, which were originally believed to be effective, have been found to possibly increase the risk of development of coronary artery aneurysms. Steroids may cause aggregation of thrombocytes within an aneurysm. Aspirin has been the most suitable agent for treatment of Kawasaki disease because of the antiinflammatomy and antithrombotic effects associated with it. Aspirin was the most common agent used before treatment with high-dose gamma globulin was initiated. At present, therapy involves mainly aspirin and high-dose gamma globulin. Previously, the dose of gamma globulin administered was 400 mg/kg! day for 4 or 5 days. However, since hospital stays are shortened in some cases, and because gamma globulin is more effective, the current regimen is 2 g/kg administered in one dose. It has not been de-termined why gamma globulin is effective; however, it …