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非典型川崎病

ATYPICAL KAWASAKI DISEASE.

作者信息

Ristovski Ljiljana, Milankov Olgica, Vislavski Melanija, Savić Radojica, Bjelica Milena

出版信息

Med Pregl. 2016 Jan-Feb;69(1-2):53-7. doi: 10.2298/mpns1602053r.

DOI:10.2298/mpns1602053r
PMID:27498535
Abstract

INTRODUCTION

Kawasaki disease is an acute vasculitis which occurs primarily in children under the age of 5. The etiology of the disease is still unknown. Diagnostic criteria for Kawasaki disease are fever and at least four of the five additional clinical signs. Incomplete Kawasaki disease should be taken into consideration in case of all children with unexplained fever for more than 5 days, associated with 2 or 3 of the main clinical findings of Kawasaki disease. The diagnosis of incomplete Kawasaki disease is based on echocardiographic findings indicating the involvement of the coronary arteries. Cardiac complications, mostly coronary artery aneurysm, can occur in 20% to 25% of untreated patients and in 4% of treated patients. CASE REPORT. In this report we present a case of atypical Kawasaki disease in a 3.5-month-old infant. As soon as the diagnosis was made, the patient received high doses of intravenous immunoglobulin, with the initial introduction of ibuprofen, then aspirin with a good clinical response. Due to the presence of aneurysm of coronary arteries, further therapy involved aspirin and clopidogrel over the following 3 months, and then only aspirin for 2 years. There was a gradual regression of the changes in the coronary blood vessels to the normalization of the echocardiographic findings after 2 years.

CONCLUSION

Kawasaki disease is the second most common vasculitis of childhood, so it should be included in the differential diagnosis for any child with a prolonged unexplained fever. Atypical Kawasaki disease should be taken into consideration in cases when not all clinical criteria are present but coronary abnormalities are documented.

摘要

引言

川崎病是一种主要发生在5岁以下儿童的急性血管炎。该病的病因仍不清楚。川崎病的诊断标准为发热以及其他五项临床体征中的至少四项。对于所有不明原因发热超过5天且伴有川崎病两项或三项主要临床表现的儿童,应考虑不完全川崎病。不完全川崎病的诊断基于超声心动图显示冠状动脉受累。未经治疗的患者中20%至25%会出现心脏并发症,主要是冠状动脉瘤,而接受治疗的患者中这一比例为4%。病例报告。在本报告中,我们呈现了一例3.5个月大婴儿的非典型川崎病病例。一旦确诊,患者即接受大剂量静脉注射免疫球蛋白治疗,最初使用布洛芬,随后使用阿司匹林,临床反应良好。由于存在冠状动脉瘤,后续治疗在接下来的3个月中使用阿司匹林和氯吡格雷,之后仅使用阿司匹林治疗2年。2年后冠状动脉血管的变化逐渐消退,超声心动图结果恢复正常。

结论

川崎病是儿童期第二常见的血管炎,因此对于任何不明原因长期发热的儿童,都应将其纳入鉴别诊断。当并非所有临床标准都存在但有冠状动脉异常记录时,应考虑非典型川崎病。

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