Abstract:
Kawasaki disease (KD) is an acute vasculitis with a particular tropism for the coronary arteries. KD mainly affects male children between 6 months and 5 years of age. The diagnosis is clinical, based on the international American Heart Association criteria. It should be systematically considered in children with a fever, either of 5 days or more, or of 3 days if all other criteria are present. It is important to note that most children present with marked irritability and may have digestive signs. Although the biological inflammatory response is not specific, it is of great value for the diagnosis. Because of the difficulty of recognising incomplete or atypical forms of KD, and the need for urgent treatment, the child should be referred to a paediatric hospital as soon as the diagnosis is suspected. In the event of signs of heart failure (pallor, tachycardia, polypnea, sweating, hepatomegaly, unstable blood pressure), medical transfer to an intensive care unit (ICU) is essential. The standard treatment is an infusion of IVIG combined with aspirin (before 10 days of fever, and for a minimum of 6 weeks), which reduces the risk of coronary aneurysms. In case of coronary involvement, antiplatelet therapy can be maintained for life. In case of a giant aneurysm, anticoagulant treatment is added to the antiplatelet agent. The prognosis of KD is generally good and most children recover without sequelae. The prognosis in children with initial coronary involvement depends on the progression of the cardiac anomalies, which are monitored during careful specialised cardiological follow-up.
摘要:
川崎病(KD)是一种急性血管炎,对冠状动脉具有特殊的嗜性。KD主要影响6个月至5岁的男性儿童。诊断是临床的,根据国际美国心脏协会的标准。应该系统地考虑发烧的儿童,5天或更长时间,如果所有其他标准都存在,则为3天。重要的是要注意,大多数儿童表现出明显的易怒,并可能有消化迹象。虽然生物炎症反应不是特异性的,这对诊断有很大的价值。由于难以识别不完整或非典型形式的KD,以及紧急治疗的需要,一旦怀疑诊断,孩子应该被转诊到儿科医院。如果出现心力衰竭的迹象(苍白,心动过速,呼吸息肉,出汗,肝肿大,血压不稳定),医疗转移到重症监护病房(ICU)是必不可少的。标准治疗是输注IVIG联合阿司匹林(在发烧10天之前,和至少6周),这降低了冠状动脉瘤的风险。在冠状动脉受累的情况下,抗血小板治疗可以维持终身。如果是巨大的动脉瘤,抗血小板剂中加入抗凝治疗。KD的预后总体良好,大多数儿童恢复无后遗症。初次冠状动脉受累儿童的预后取决于心脏异常的进展,在仔细的专业心脏病随访期间进行监测。