以重症肝炎为首发症状的巨噬细胞活化综合征6例临床分析及回顾

The macrophage activation syndrome with severe hepatitis as the first symptom:clinical analysis of 6 cases and review

目的:提高对以重症肝炎为首发症状的巨噬细胞活化综合征的认识。方法:对6例以重症肝炎为首发症状的巨噬细胞综合征的临床表现及实验室资料进行回顾性分析。结果:6例以重症肝炎为首发症状的巨噬细胞活化综合征的患儿均有幼年特发性关节炎(全身型)病史,MAS发病时均无发热,6例患儿的肝功能ALT平均为1 927.33 U/L,TBIL平均为179.82μmol/L,6例经治疗后肝功能均好转出院,无死亡病例。结论:对于重症肝炎的患儿,应综合分析,追问有无风湿性疾病的病史,尤其是幼年特发性关节炎(全身型)病史,提高对巨噬细胞活化综合征的认识,早期诊断,早期治疗。

Objective：To improve the understanding of macrophage activation syndrome with severe hepatitis as the first symptom.Methods：The clinical manifestations of 6 cases of macrophage activation syndrome with severe hepatitis as the first symptom and laboratory data were retrospectively analyzed.Results：6 cases of severe hepatitis as the first symptom of macrophage activation syndrome in children had Systemic-onset juvenile idiopathic arthritis medical history,MAS incidence had no fever,an average of 6 cases of children with liver function ALT of as 1 927.33 U/L TBIL and average the for 179.82 μmol/L,6 cases of liver function were improved and discharged,with no deaths.Conclusion：For children with severe hepatitis,a comprehensive analysis should be needed,questioning whether a history of rheumatic diseases,especially the history of Systemic-onset juvenile idiopathic arthritis,might improve the understanding of macrophage activation syndrome,early diagnosis,early treatment.