背景:川崎病(KD)是一种中小动脉血管炎综合征,具有典型的发热等临床特征,皮疹,颈淋巴结病,结膜炎,粘膜改变.心脏表现,包括冠状动脉瘤,心肌炎,心肌梗塞,和心源性猝死,是KD中观察到的最严重的并发症。在极少数情况下,它可能伴随着收缩性低血压导致的器官灌注减少,一种称为川崎病休克综合征(KDSS)的疾病。KDSS是一种严重的并发症,当尚未检测到KD的典型临床症状时,可以将其作为初始特征提供给急诊科。
方法:我们报告一例12岁男孩因长期发烧入院,双侧非化脓性结膜炎,和休克的迹象,如低血压和心动过速。实验室结果显示炎症标志物升高,低蛋白血症,和无菌脓尿。考虑到可能诊断为中毒性休克综合征,他最初接受静脉注射头孢噻肟和万古霉素治疗。而治疗无效。随后的胸部计算机断层扫描和超声发现肺实变和多浆液性积液。超声心动图显示轻度双心房扩张和轻度瓣膜反流,左心室功能保留。
方法:经过多学科咨询,诊断为KDSS。
方法:为了预防冠状动脉病变和其他严重并发症,患者立即接受了免疫球蛋白,皮质激素,和乙酰水杨酸。
结果:不久之后,他表现出显著的进步,静脉注射免疫球蛋白治疗后约24小时,体温降至正常,低血压得到纠正。多浆液性积液在出院前也消失了。随访超声心动图检查结果正常。
结论:临床医生应保持对KD的高度怀疑,并将肺部受累和多浆液性积液视为潜在的并发症。对于患有KD的儿童,任何指向感染的症状都应仔细考虑。当没有病因学证据时,应谨慎使用抗生素。我们的病例还强调了将KDSS作为长期发烧和休克儿童的鉴别诊断的重要性。早期识别,及时治疗,密切监测是预防严重并发症和确保KDSS患者良好预后的关键.
BACKGROUND: Kawasaki disease (KD) is a vasculitis syndrome of small to medium-sized arteries that has typical clinical characteristics such as fever, rash, cervical lymphadenopathy, conjunctivitis, and mucosal changes. Cardiac manifestations, including coronary artery aneurysms, myocarditis, myocardial infarction, and sudden cardiac death, are the most serious complications observed in KD. On rare occasions, it may accompanied with reduced organ perfusion due to systolic hypotension, a condition known as Kawasaki disease shock syndrome (KDSS). KDSS is a serious complication that can be presented to the emergency department as an initial feature when typical clinical symptoms of KD have not be detected.
METHODS: We report the case of a 12-year-old boy admitted with prolonged fever, bilateral non-purulent conjunctivitis, and signs of shock such as hypotension and tachycardia. Laboratory findings showed elevated inflammatory markers, hypoalbuminemia, and sterile pyuria. He was initially treated with intravenous cefotaxime and vancomycin considering the possible diagnosis of toxic shock syndrome, while the treatment was not effective. Subsequent chest computerized tomography and ultrasound identified pulmonary consolidation and polyserous effusion. Echocardiography revealed mild biatrial dilatation and mild valvular regurgitation with preserved left ventricular function.
METHODS: After a multidisciplinary consultation, a diagnosis of KDSS was made.
METHODS: To prevent coronary artery lesions and other severe complications, the patient immediately received immunoglobulin, corticoid, and acetylsalicylic acid.
RESULTS: Soon afterwards, he showed significant improvement, with the temperature dropped to normal and hypotension corrected about 24 hours post-intravenous immunoglobulin therapy. Polyserous effusions also disappeared before discharge. Follow-up echocardiography revealed normal results.
CONCLUSIONS: Clinicians should maintain a high index of suspicion for KD and consider pulmonary involvement and polyserous effusions as potential complications. For children with KD, any symptoms pointing to infection should be carefully considered. When there is no etiologic evidence, antibiotics should be used with caution. Our case also highlights the importance of considering KDSS as a differential diagnosis in children presenting with prolonged fever and shock. Early recognition, timely treatment, and close monitoring are key to preventing severe complications and ensuring favorable outcomes in patients with KDSS.