胸腺切除术并类固醇疗法治疗伴胸腺瘤的重症肌无力的远期疗效观察.docVIP

胸腺切除术并类固醇疗法治疗伴胸腺瘤的重症肌无力的远期疗效观察 　　摘要　目的　评价胸腺切除术并皮质类固醇疗法对伴胸腺瘤的重症肌无力患者的远期疗效。方法　对采用胸腺切除术并类固醇疗法治疗的52例伴胸腺瘤的重症肌无力患者作回顾性研究，经术后1～20年，平均(4.5±3.6)年的长期随访，评价其远期有效率和存活率。结果　远期有效率为88.5%(46/52)，病死率11.5%(6/52)。在46例远期存活者中，8例获完全缓解(15.4%)，29例获药物缓解(55.8%)，9例明显改善(17.3%)。5年存活率86.4%，10年存活率33.3%。结论　本研究结果提示胸腺切除术并皮质类固醇疗法治疗伴胸腺瘤的重症肌无力患者的远期疗效良好，该疗法对降低术后病死率和提高缓解率有重要作用。 　　Objective　To evaluate the long-term outcome of thymectomy combined with corticosteroid therapy for myasthenia gravis (MG) with thymoma. Methods　Fifty-two MG Patients with thymoma treated with thymectomy combined with corticosteroid therapy underwent a retrospective study during a follow-up period of 1 to 20 years (mean4.5±3.6years) after thymectomy in order to evalute the long-term effective rate and survival rate. Results　The long-term effective rate was 88.5% (46/52),mortality was 11.5%(6/52).In 46 survivors,8 of them obtained complete remission (15.4%)，29 of them pharmacological remission (55.8%)，9 of them marked improvement (17.3%).The overall 5-year survival rateamp; nbsp;was 86.4%，the overall 10-year survival rate was 33.3%. Conclusions　The long-term therapeutic results of thymectomy combined corticosteroid therapy were good for MG patients with thymoma.This therapeutic measure played an important role in decreasing mortality and increasing remission rate. 　　Key words　　myasthenia gravis;thymoma;thymectomy;corticosteroid therapy;long-term outcome 　　有数份报告指出胸腺切除术对伴胸腺瘤的重症肌无力(myasthenia gravis,MG)的疗效不佳，而且有增加术后病死率的危险［1，2］。本文报告胸腺切除术并类固醇疗法治疗52例伴胸腺瘤MG的远期预后的回顾性研究结果。 　　1　对象和方法 　　1.1　患者　本组52例MG患者均经新斯的明或滕喜龙试验阳性确诊。其伴有的胸腺瘤均经胸片、纵隔CT或纵隔MRI检查后作出诊断并经手术证实。其中男35例和女17例，MG的发病年龄8～61岁，平均(38.5±12.7)岁。眼型5例，全身型47例(90.3%)。按Osserman的标准，其中ⅡA型10例，ⅡB型1例，Ⅲ型10例，Ⅳ型26例。 　　1.2　方法　手术方法均采用胸骨纵切的扩大的胸腺和胸腺瘤切除术，手术前1～2个月后给予类固醇疗法，开始通常用强的松30～60 mg/d，晨1次顿服(小儿按每日1 mg/kg)，症状明显改善后即行胸腺切除术，术后临床好转，则逐渐减量，获最大改善后用维持量(强的松10～20 mg/d)继续维持治疗1～2年。在减量过程中若有轻微复发则暂停减量，或适当加量数月。若症状严重恶化则重新给予每日大剂量。服用维持量强的松的MG患者若6个月以上仍无任何症状可停药。本组患者用