胸腺手术治疗儿童重症肌无力的疗效随访及影响因素分析  

Response to thymectomy and analysis of influencing factors in the treatment of children withmyasthenia gravis

作　　者：李言[1] 刘卫彬[1] 罗传铭[1] 冯慧宇[1] 黄鑫[1] 王海燕[1] 邱力[1] 黄如训[1] 

机构地区：[1]中山大学附属第一医院神经内科,广州510080

出　　处：《中华医学杂志》2012年第17期1170-1173,共4页National Medical Journal of China

基　　金：国家自然科学基金（30870850,81071002）;广东省科技专项基金（200713016002039）;中山大学临床研究5010计划（2010003）

摘　　要：目的通过长期的随访评价胸腺扩大切除术治疗儿童重症肌无力的疗效与相关影响因素。方法回顾性分析2003年1月至2009年8月在中山大学附属第一医院行胸腺扩大切除术的59例儿童重症肌无力患儿临床资料及长期随访结果，疗效评价分为完全稳定缓解、药物缓解、好转、无变化、恶化（包括死亡）。对性别、手术年龄、临床分型、术前病程、异位胸腺等因素进行统计分析。结果（1）术后总随访人数53例，中位随访时间35个月，无恶化和死亡病例。总缓解率（完全缓解＋药物缓解）69．8％，有效率（完全缓解＋药物缓解＋好转）90．6％。16例完全缓解患者无复发。眼肌型患者术后无1例转化为全身型，而16例全身型手术患者是由眼肌型转化而来。（2）单因素分析和多因素分析显示术前病程影响手术疗效（P〈0．05）。生存分析眼肌型和全身型患者术后2年、4年的总缓解率分别为56％、88％和42％、75％，Log-rank法比较两者之间总缓解率无差别。结论严格挑选的重症肌无力儿童手术治疗安全、有效，特别是术前病程较短者。总缓解率随术后时间延长而升高。Objective To evaluate the efficacy of thymectomy and relevant influencing factors in the treatment of children with myasthenia gravis through a long-term follow-up. Methods The clinical records of 59 patients undergoing expanded thymectomy for the treatment of myasthenia gravis （MG） between January 2003 and August 2009 were reviewed retrospectively. Their postoperative outcomes were categorized into complete stable remission （ CSR）, pharmacological remission （ PR）, improvement, no change and deterioration （including mortality）. Results During a median follow-up period of 35 months, none of them died or deteriorated clinically among 53 patients with a postoperative follow-up. The overall remission rate was 69.8% and the effective rate 90.6%. No symptomatic relapse occurred among 16 patients in CSR. None of the ocular patients progressed to generalized MG while 16 thymectomized generalized MG developed from ocular MG. Both univariate and logistic regression analyses revealed that the preoperative duration of illness influenced the surgical curative effect（ P 〈0.05 ）. Survival analysis indicated that the rates of overall remission were 56% or 88% at 24 months and 42% or 75% at 48 months among ocular MG and generalized MG respectively. According to Log-rank analysis, no difference in remission existed between two types of MG. Conclusion Thymeetomy is an effective and safe treatment in selected MG children, especially in those with a shorter illness duration.

关 键 词：重症肌无力 儿童 胸腺切除术 

分 类 号：R746.1[医药卫生—神经病学与精神病学]