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菲律宾总医院胸腺切除术后与未行胸腺切除术的重症肌无力患者的临床特征及预后:一项6年回顾性研究

Clinical Profile and Outcome of Postthymectomy versus Non-Thymectomy Myasthenia Gravis Patients in the Philippine General Hospital: A 6-Year Retrospective Study.

作者信息

De Roxas Ranhel C, Bagnas Marjorie Anne C, Baldonado Jobelle Joyce Anne R, Rivera Jonathan P, Roxas Artemio A

机构信息

Department of Neurosciences, Philippine General Hospital , Manila , Philippines.

Department of Surgery, Philippine General Hospital , Manila , Philippines.

出版信息

Front Neurol. 2016 Jun 21;7:96. doi: 10.3389/fneur.2016.00096. eCollection 2016.

Abstract

BACKGROUND

Myasthenia gravis is an autoimmune neuromuscular disorder characterized by the production of abnormal autoantibodies directed against the receptors present in the neuromuscular junction. It has been the standard practice to offer thymectomy in all generalized myasthenia gravis patients despite the lack of robust evidence.

OBJECTIVES

The objectives of this study are to describe the clinical profile and differentiate the clinical outcomes of thymectomy versus non-thymectomy and thymomatous versus non-thymomatous myasthenia gravis patients in the Philippine General Hospital.

METHODOLOGY

Between 2009 and 2014, a total of 69 postthymectomy and 16 non-thymectomy patient records were successfully retrieved. The demographic characteristics, surgical approach, and histopathologic results were obtained. The clinical outcome after 6 months or 1 year-follow-up was also determined and grouped according to the following: (1) complete remission, (2) pharmacological remission, (3) no clinical change, (4) worsening symptoms, and (5) mortality.

RESULTS

Majority of the patients were females (68.0%) with a mean age of 39.8 years and a mean duration of myasthenic symptoms of 21 months. Using the Myasthenia Gravis Foundation of America classification, 54.1% of patients fell under Class II and 48.2% of them presented with generalized weakness. In this study, 60.8% of postthymectomy myasthenia gravis patients had either complete remission or pharmacologic remission compared with 12.5% among non-thymectomy patients (p-value <0.001). No significant difference in the clinical outcome was found between thymomatous and non-thymomatous myasthenia gravis after thymectomy (p-value = 0.29).

CONCLUSION

This study showed that both thymomatous and non-thymomatous myasthenia gravis patients who underwent thymectomy had a higher incidence of complete stable remission and pharmacologic remission as compared with myasthenia gravis patients who did not undergo thymectomy.

摘要

背景

重症肌无力是一种自身免疫性神经肌肉疾病,其特征是产生针对神经肌肉接头处受体的异常自身抗体。尽管缺乏有力证据,但对所有全身型重症肌无力患者进行胸腺切除术一直是标准做法。

目的

本研究的目的是描述菲律宾总医院中重症肌无力患者的临床特征,并区分胸腺切除术与非胸腺切除术以及胸腺瘤型与非胸腺瘤型重症肌无力患者的临床结局。

方法

2009年至2014年期间,成功检索到69例胸腺切除术后患者和16例非胸腺切除术患者的记录。获取了人口统计学特征、手术方式和组织病理学结果。还确定了6个月或1年随访后的临床结局,并根据以下情况进行分组:(1)完全缓解,(2)药物缓解,(3)无临床变化,(4)症状恶化,(5)死亡。

结果

大多数患者为女性(68.0%),平均年龄39.8岁,重症肌无力症状的平均持续时间为21个月。根据美国重症肌无力基金会的分类,54.1%的患者属于II类,其中48.2%表现为全身无力。在本研究中,胸腺切除术后的重症肌无力患者中有60.8%实现了完全缓解或药物缓解,而非胸腺切除术患者中这一比例为12.5%(p值<0.001)。胸腺切除术后,胸腺瘤型与非胸腺瘤型重症肌无力患者的临床结局无显著差异(p值=0.29)。

结论

本研究表明,与未接受胸腺切除术的重症肌无力患者相比,接受胸腺切除术的胸腺瘤型和非胸腺瘤型重症肌无力患者完全稳定缓解和药物缓解的发生率更高。

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