Maximal thymectomy in children with myasthenia gravis

Maximal thymectomy in children with myasthenia gravis
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DOI:
10.1016/s1010-7940(03)00299-9
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发表时间:
2003-08-01
影响因子:
3.4
通讯作者:
Al-Kattan, K
Al-Kattan, K
中科院分区:
医学2区
文献类型:
--
作者:
Essa, M;El-Medany, Y;Al-Kattan, K

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目的:我们进行这项研究,以评估胸腺切除术在儿童重症肌无力(MG)的好处。研究方法:从1986年到2001年,我们收集了30例MG患儿的资料,并回顾性分析了最大胸腺切除术的结果。结果:有23名女性和7名男性,平均年龄为13.2岁(范围4-16)。平均病程为19.3个月(范围2-144)。根据Osserman分类,11班有14名儿童; 111班有12名儿童;四班有4名儿童。1例IV级患儿需要术后通气,1例再次探查以引流继发于中心管泄漏的心包积液。发现异位胸腺组织10例(33.3%)。在平均53.5个月的随访期间(范围9-180),13名儿童(43.4%)完全缓解,14名儿童(46.6%)改善。其余3名儿童(10%)在手术后没有改善。单因素分析(P < 0.05)显示异位胸腺组织是影响预后的重要因素。结论:最大胸腺切除术似乎提供了一个高的缓解率和改善儿童MG。然而,异位胸腺组织的存在有不良的预后价值。(C)2003 Elsevier Science B. V.保留所有权利。
Objectives: We performed this study to evaluate the benefit of thymectomy in children with myasthenia gravis (MG). Methods: Over a period of 15 years from 1986 to 2001, we collected data on 30 children with MG and retrospectively reviewed the outcome of maximal thymectomy. Results: There were 23 females and seven males with a mean age of 13.2 years (range 4-16). The mean duration of the disease was 19.3 months (range 2-144). According to Osserman classification, there were 14 children in class 11; 12 in class 111; and four children in class IV. One child in class IV required postoperative ventilation and one was re-explored to drain a pericardial effusion secondary to central line leak. We found ectopic thymic tissue in 10 cases (33.3%). During a mean follow-up period of 53.5 months (range 9-180), complete remission was noted in 13 children (43.4%) and improvement in 14 (46.6%). The remaining three children (10%) did not improve following surgery. Univariate analysis (P < 0.05) showed that ectopic thymic tissue is a significant prognostic factor for outcome. Conclusion: Maximal thymectomy appears to provide a high rate of remission and improvement in children with MG. However, the presence of ectopic thymic tissue has poor prognostic value. (C) 2003 Elsevier Science B.V. All rights reserved.