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他克莫司改善对泼尼松治疗抵抗的儿童重症肌无力的症状。

Tacrolimus Improves Symptoms of Children With Myasthenia Gravis Refractory to Prednisone.

机构信息

Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

出版信息

Pediatr Neurol. 2017 Dec;77:42-47. doi: 10.1016/j.pediatrneurol.2017.08.014. Epub 2017 Sep 5.

Abstract

BACKGROUND

Myasthenia gravis tends to affect children in China. Oral pyridostigmine and prednisone could effectively improve the symptoms, but multiple side effects become a major concern after long-term oral prednisone. To avoid the long-term complications of prednisone therapy and to obtain more satisfactory improvement, we tested the efficacy and safety of tacrolimus in children with myasthenia gravis.

METHODS

Children with myasthenia gravis who had not achieved satisfactory improvement or who experienced severe side effects after prednisone therapy were recruited between January 2015 and December 2016 at Tongji Hospital. All the children were treated with tacrolimus 1 mg to 2 mg daily and the dose was adjusted on the basis of the clinical response and the serum concentration. The dosage of prednisone, the severity of symptoms, blood samples, the serum concentration of tacrolimus, and titers of antiacetylcholine receptor antibodies were evaluated every four weeks.

RESULTS

Fourteen children were enrolled. One child withdrew two weeks after the enrollment. Thirteen children have completed the therapy for one year. At the end point, the dosage of prednisone was significantly decreased (P < 0.05), the symptoms were evaluated by the quantitative myasthenia gravis score, and myasthenia gravis-specific manual muscle testing and myasthenia gravis-activities of daily living scores were significantly improved (P < 0.05, P < 0.05, and P < 0.01, respectively). More importantly, ten (76.9%) patients had completely discontinued prednisone, and the major side effects were nearly reversed. The mean titer of antiacetylcholine receptor antibodies significantly dropped from 1.96±2.62 nmol/L to 0.70±1.04 nmol/L (P < 0.05). No severe adverse events were reported.

CONCLUSIONS

Our results suggest that tacrolimus is a promising agent for children with refractory myasthenia gravis. Randomized clinical trials are needed to confirm the observation.

摘要

背景

重症肌无力在中国常影响儿童。口服吡啶斯的明和泼尼松可有效改善症状,但长期口服泼尼松会引起多种副作用,这是一个主要关注点。为了避免长期泼尼松治疗的并发症,并获得更满意的改善效果,我们测试了他克莫司在重症肌无力儿童中的疗效和安全性。

方法

2015 年 1 月至 2016 年 12 月,在同济大学附属同济医院招募未达到满意改善或泼尼松治疗后出现严重副作用的重症肌无力患儿。所有患儿均给予他克莫司 1mg 至 2mg/d 治疗,根据临床反应和血清浓度调整剂量。每四周评估泼尼松剂量、症状严重程度、血样、他克莫司血清浓度和乙酰胆碱受体抗体滴度。

结果

共纳入 14 例患儿,1 例患儿入组后 2 周退出。13 例患儿完成了 1 年的治疗。在终点时,泼尼松剂量显著降低(P<0.05),定量重症肌无力评分、重症肌无力特定手工肌肉测试和重症肌无力日常生活活动评分评估的症状显著改善(P<0.05、P<0.05 和 P<0.01)。更重要的是,10 例(76.9%)患者完全停用了泼尼松,主要副作用几乎逆转。乙酰胆碱受体抗体滴度的平均值从 1.96±2.62 nmol/L 显著下降至 0.70±1.04 nmol/L(P<0.05)。未报告严重不良事件。

结论

我们的结果表明,他克莫司是治疗难治性重症肌无力儿童的一种有前途的药物。需要进行随机临床试验来证实这一观察结果。

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