Clinical features and analysis of 10 children with Kawasaki disease shock syndrome combined with macrophage activation syndrome
10.3760/cma.j.issn.1673-4912.2025.03.008
- VernacularTitle:十例川崎病休克综合征合并巨噬细胞活化综合征患儿临床特征分析
- Author:
Zhihui ZHAO
1
;
Jia NA
1
;
Zhen ZHEN
1
;
Wen YU
1
;
Yue YUAN
1
;
Yanyan XIAO
1
Author Information
1. 国家儿童医学中心 首都医科大学附属北京儿童医院心脏内科 100045
- Publication Type:Journal Article
- Keywords:
Kawasaki disease shock syndrome;
Macrophage activation syndrome;
Clinical features;
Children
- From:
Chinese Pediatric Emergency Medicine
2025;32(3):202-206
- CountryChina
- Language:Chinese
-
Abstract:
Objective:Kawasaki disease shock syndrome（KDSS）and macrophage activation syndrome（MAS）are both severe forms of Kawasaki disease. The coexistence of these two critical illnesses is extremely rare，which can be life-threatening in severe cases. The purpose of this study is to summarize the clinical features of children with KDSS complicated with MAS，and provide a basis for precise diagnosis and treatment.Methods:A retrospective analysis was conducted on the clinical manifestations，laboratory tests，imaging characteristics，treatment，and prognosis of 10 children with KDSS and MAS admitted to Beijing Children's Hospital，Capital Medical University from January 2021 to June 2024.Results:Among the 10 children，six were male and four were female，and the age of onset was three months to eleven years old. Acute kidney injury was observed in five patients. Laboratory tests revealed significant increases in serum ferritin，C-reactive protein，B-type natriuretic peptide，aspartate aminotransferase，alanine aminotransferase，creatinine，triglycerides，and interferon-γ，while platelet count and albumin were significantly decreased. Six patients had cardiac enlargement，three had reduced ejection fraction，seven had pericardial effusion，and seven had coronary artery damage. All children were treated with immunoglobulin and methylprednisolone pulse therapy，as well as vasoactive drug infusion to improve circulation and maintain blood pressure. All children were discharged after clinical improvement，and most had a good prognosis.Conclusion:Children with KDSS may develop MAS，which present a complex and rapidly progressing condition often accompanied by a significant increase in ferritin levels. Early diagnosis and treatment can lead to a favorable prognosis.
