环磷酰胺冲击治疗儿童难治性斯蒂尔病的疗效

Efficiency of Pulse Cyclophosphamide in the Treatment of Severe Still' s Disease in Children

目的观察环磷酰胺冲击治疗儿童难治性斯蒂尔病的疗效。方法环磷酰胺每次700～750mg/m2,每2～4周1次静脉滴注,疗程3～6月,总剂量150mg/kg,予以疗效评估,并随访6月～3年。结果10例难治性斯蒂尔病患儿均完成疗程,其中显效者4例,有效者5例,无效者1例,总有效率达90%。治疗过程中3例出现不良反应。结论环磷酰胺冲击治疗儿童难治性斯蒂尔病具有较好的疗效。

Objective To investigate the effect of pulse cyclophosphamide treatment in severe Still' s disease children. Methods The total dose of intravenous cyclophosphamide was 150 mg/kg and 700 - 750 mg/m2 per course. The median interval between the courses treatment for cyclophosphamide was 3 weeks (range, 2 to 4). Each total course was 3 to 6 months. We assessed the efficacy of cyclophosphamide and reported the 6 months to 3 years follow-up investigation. Results Ten severe Still's disease patients finished the whole course of treatment. Four patients achieved satisfaction response, and five partial response, and one no response. The overall effective rate was 90% . Three cases had toxic reaction response. Conclusion Cyclophosphamide is effective in the treatment of severe Still's disease in children.