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目的:总结儿童巨噬细胞活化综合征并肝衰竭的临床特点,为临床提供诊疗经验。方法:对青岛市儿童医院风湿免疫科2011年至2012年诊治的2例巨噬细胞活化综合征并肝衰竭患儿的临床资料进行分析、总结,并复习相关文献。结果:2例患儿均继发于全身型幼年特发性关节炎,肝衰竭为突出临床表现,经环孢素、大剂量甲泼尼龙、丙种球蛋白联合治疗后,病情缓解,随访超过6个月无复发。结论:巨噬细胞活化综合征并肝衰竭患儿病情凶险,病死率高,积极治疗可改善预后。环孢素、大剂量甲泼尼龙、丙种球蛋白联合治疗可作为一线治疗方案。
Objective: To summarize the clinical features of macrophage activation syndrome in children with liver failure and provide clinical experience. Methods: The clinical data of 2 cases of macrophage activation syndrome and liver failure diagnosed and treated by Department of Rheumatology, Qingdao Children’s Hospital from 2011 to 2012 were analyzed and summarized, and the related literatures were reviewed. Results: Two cases of children were all secondary to systemic juvenile idiopathic arthritis. Liver failure was the most prominent clinical manifestation. After treatment with cyclosporine, high dose of methylprednisolone and gamma globulin, the condition was relieved and the follow-up was over 6 No recurrence for a month. Conclusion: Macrophage activation syndrome and liver failure in children with dangerous conditions, high mortality, aggressive treatment can improve prognosis. Cyclosporine, high-dose methylprednisolone, gamma globulin combination therapy can be used as a first-line treatment.