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儿童期起病的重症肌无力患者在长期随访观察中受益于胸腺切除术。

Childhood-Onset Myasthenia Gravis Patients Benefited from Thymectomy in a Long-Term Follow-up Observation.

机构信息

Department of Neurology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.

Department of Radiology, Zhongnan Hospital, Wuhan University, Wuhan, Hubei Province, China.

出版信息

Eur J Pediatr Surg. 2022 Dec;32(6):543-549. doi: 10.1055/s-0042-1744150. Epub 2022 Mar 9.

Abstract

INTRODUCTION

The effect of thymectomy on the treatment of childhood-onset myasthenia gravis (CMG) remains debatable. The objective of this study was to evaluate the clinical outcome and relevant prognostic factors of thymectomy for CMG patients.

MATERIALS AND METHODS

A total of 32 CMG patients who underwent thymectomy before 18 years of age were included in this retrospective study. Clinical state following thymectomy was assessed by quantified myasthenia gravis (QMG) scores, myasthenia gravis-related activities of daily living (MG-ADL) scores, and Myasthenia Gravis Foundation of America postintervention status. Repeated-measures analysis of variance (ANOVA) examined the changes in postoperative scores during the 5-year follow-up. Univariate logistic regression was applied to identify factors associated with short-term (1-year postoperation) and long-term (5-year postoperation) clinical outcomes.

RESULTS

Repeated-measures ANOVA showed that QMG scores ( = 6.737,  < 0.001) and MG-ADL scores ( = 7.923,  < 0.001) decreased gradually with time. Preoperative duration (odds ratio [OR] = 0.85, 95% confidence interval [CI]: 0.73-1.00,  = 0.043), gender (OR = 0.19, 95% CI: 0.04-0.94,  = 0.041), and MG subgroup (OR = 13.33, 95% CI: 1.43-123.99,  = 0.023) were predictors for 1-year postoperative prognosis. Shorter disease duration (OR = 0.82, 95% CI: 0.70-0.97,  = 0.018) and generalized CMG (OR = 6.11, 95% CI: 1.06-35.35,  = 0.043) were found to have more favorable long-term results.

CONCLUSION

Our results suggest that thymectomy is effective in treating CMG. Thymectomy could be recommended for CMG patients, especially for patients in the early course of GMG.

摘要

简介

胸腺切除术对儿童期起病重症肌无力(CMG)的治疗效果仍存在争议。本研究旨在评估胸腺切除术治疗 CMG 患者的临床疗效和相关预后因素。

材料与方法

本回顾性研究纳入了 32 例在 18 岁之前接受胸腺切除术的 CMG 患者。术后临床状态采用定量重症肌无力(QMG)评分、重症肌无力日常生活活动(MG-ADL)评分和美国重症肌无力基金会干预后状态进行评估。采用重复测量方差分析(ANOVA)检验 5 年随访期间术后评分的变化。单因素 logistic 回归分析用于识别与短期(术后 1 年)和长期(术后 5 年)临床结局相关的因素。

结果

重复测量 ANOVA 显示 QMG 评分( = 6.737, < 0.001)和 MG-ADL 评分( = 7.923, < 0.001)随时间逐渐降低。术前病程(比值比 [OR] = 0.85,95%置信区间 [CI]:0.73-1.00, = 0.043)、性别(OR = 0.19,95% CI:0.04-0.94, = 0.041)和 MG 亚组(OR = 13.33,95% CI:1.43-123.99, = 0.023)是术后 1 年预后的预测因素。病程较短(OR = 0.82,95% CI:0.70-0.97, = 0.018)和全身型 CMG(OR = 6.11,95% CI:1.06-35.35, = 0.043)提示预后更好。

结论

我们的研究结果表明胸腺切除术对 CMG 有效。建议对 CMG 患者行胸腺切除术,尤其是对 GMG 早期患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/90fc/9666056/0ba5bab7c7f3/10-1055-s-0042-1744150-i216093oa-1.jpg

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