川崎病合并巨噬细胞活化综合征1例并文献复习

One Case of Kawasaki Disease Complicated with Macrophage Activation Syndrome and Literature Review

摘要: 目的：总结1例川崎病合并巨噬细胞活化综合征患儿的临床特点及诊疗经过，提高对该疾病的认识。方法：回顾性分析2023年1月山东大学附属威海市立医院儿科收治的1例川崎病合并巨噬细胞活化综合征患儿的临床特点及诊疗经过，并进行相关文献复习。结果：患儿以发热、皮疹及结膜充血为突出表现，在诊断为川崎病后使用静注人免疫球蛋白、阿司匹林治疗，仍高热。加用糖皮质激素后体温降至正常，结合相关检查，诊断KD合并MAS。结论：对于川崎病患儿，若伴有三系减少或静注人免疫球蛋白治疗无效时，应考虑是否合并MAS，及时加用糖皮质激素，降低死亡率。

Abstract: Objective: To summarize the clinical characteristics and treatment outcome of 1 cases of Kawasaki disease complicated by macrophage activation syndrome so as to improve clinician’s awareness of the disease. Methods: Retrospective analysis of the clinical characteristics and diagnosis and treatment process of a child with Kawasaki disease complicated with macrophage activation syndrome admitted to the pediatrics department of Weihai Municipal Hospital affiliated with Shandong University in January 2023, and relevant literature review. Results: The child presented with prominent symptoms of fever, rash, and conjunctival congestion. After diagnosis of Kawasaki disease, intravenous immunoglobulin and aspirin were used for treatment, but the fever persisted. After adding glucocorticoids, the body temperature returned to normal, and combined with relevant examinations, KD was diagnosed with MAS. Conclusions: For children with Kawasaki disease, if there is a reduction in the three lineages or if intravenous immunoglobulin therapy is ineffective, consideration should be given to whether to combine MAS and timely use of glucocorticoids to reduce mortality.