Clinical outcome of juvenile myasthenia gravis after extended transsternal thymectomy in a chinese cohort.
Clinical outcome of juvenile myasthenia gravis after extended transsternal thymectomy in a chinese cohort.
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DOI：
10.1016/j.athoracsur.2012.11.074
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发表时间：
2013-03
期刊：
影响因子：
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通讯作者：
Chao Cheng;Zhenguo Liu;Fenghua Xu;Zhensheng Deng;H. Feng;Yiyan Lei;Jianyong Zou;S. Yeung
中科院分区：
文献类型：
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作者：
Chao Cheng;Zhenguo Liu;Fenghua Xu;Zhensheng Deng;H. Feng;Yiyan Lei;Jianyong Zou;S. Yeung
手术治疗在青少年重症肌无力（MG）中的作用尚不清楚。在这里，我们进行了一项回顾性研究，以评估预测的临床结果与青少年MG治疗延长经胸骨thymectomy.METHODSA共141个连续的青少年MG患者进行了延长经胸骨胸腺切除术在学术医院超过20年的时间进行了审查。对吡啶斯的明治疗耐药、全身症状或对吡啶斯的明治疗有部分反应超过2年的眼部MG患者进行胸腺切除术。使用Kaplan-Meier分析和考克斯回归模型评价可能影响扩大经胸骨胸腺切除术反应的变量。完全稳定缓解（Complete stable remission，CSR）定义为无症状且未服药12个月以上。眼型MG 96例，全身型MG 45例，发病中位年龄6岁，手术中位年龄12岁。在完成术后随访的135例患者中，34例（25.2%）达到CSR，28例（20.7%）出现药物缓解，61例（45.2%）改善，5例（3.7%）保持稳定，7例（5.2%）恶化。结果表明，发病年龄大于6岁和手术年龄大于12岁都与CSR反应呈正相关。眼MG术后类固醇治疗和全身性MG术前病程（>12个月）与CSR respons.CONCLUSIONSExtended transsternal胸腺切除术对中国青少年MG患者的疗效与其他种族的报道具有可比性。发病年龄大于6岁、手术年龄大于12岁、全身性MG病程较短的青少年患者与良好的临床结局相关。
BACKGROUNDThe role of surgical treatment for juvenile myasthenia gravis (MG) remains unclear. Here, we performed a retrospective study to evaluate the predictors of clinical outcome of juvenile MG treated with extended transsternal thymectomy.METHODSA total of 141 consecutive juvenile MG patients underwent extended transsternal thymectomy at an academic hospital over a 20-year period were reviewed. Thymectomy was performed in patients resistant to pyridostigmine therapy, with generalized symptoms or ocular MG with partial response to pyridostigmine for more than 2 years. Variables potentially affecting responses to extended transsternal thymectomy were evaluated using Kaplan-Meier analysis and Cox regression modeling. Complete stable remission (CSR) is defined as asymptomatic without medication for more than 12 months.RESULTSThere were 96 patients with ocular MG and 45 generalized MG, the median age at disease onset was 6 years and that at operation was 12 years. Among 135 patients with complete postoperative follow-up, 34 (25.2%) achieved CSR, 28 (20.7%) experienced pharmacologic remission, 61 (45.2%) improved, 5 (3.7%) remained stable, and 7 (5.2%) deteriorated. The results indicated the disease-onset age greater than 6 years and age at operation greater than 12 years were both positively associated with CSR responses. Postoperative steroid treatments in ocular MG and preoperative disease duration in generalized MG (>12 months) were negatively associated with CSR responses.CONCLUSIONSExtended transsternal thymectomy for Chinese juvenile MG patients has an efficacy comparable with reports from other ethnicities. Juvenile patients with disease-onset age greater than 6 years, age at operation greater than 12 years, and shorter disease duration of generalized MG are associated with favorable clinical outcomes.