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目的 探讨小儿重症肌无力(MG)的手术适应证,围手术期处理及影响疗效的有关因素。 方法 按Os-serman临床分型分为型(单纯眼肌型)11例,A型(轻度全身型)6例,B型(中度全身型)2例。胸腺病理检查:增生12例,正常组织学表现7例,无合并胸腺瘤者。12例胸腺标本进行了免疫组织化学观察。 结果 全组无手术死亡。术后发生肌无力危象1例,总有效率89.5%。全身型术后缓解率75%,较单纯眼肌型36.3%高。常规病理分型与疗效无关,根据免疫组织化学观察,12例胸腺分为上皮细胞密集型6例和上皮细胞松散型6例,两者术后疗效相差显著。 结论 小儿全身型MG应行手术治疗,而对单纯眼肌型MG的手术适应证应从严掌握,对学龄前儿童尽可能采用药物治疗。使用人工呼吸器是治疗术后肌无力危象的有效措施。胸腺免疫组织化学分型能较好地评估手术后的疗效。
Objective To investigate the surgical indications of pediatric myasthenia gravis (MG), the perioperative management and the related factors that affect the curative effect. Methods According to Os-serman clinical classification, 11 cases were type (simple ocular muscle type), 6 cases were type A (mild systemic type) and 2 cases were type B (moderate systemic type). Thymus pathological examination: hyperplasia in 12 cases, normal histological findings in 7 cases, without thymoma. Thymus specimens of 12 cases were observed by immunohistochemistry. Results All patients died without surgery. One case of myasthenia gravis crisis occurred after operation, the total effective rate was 89.5%. Systemic postoperative remission rate of 75%, 36.3% higher than the simple ocular muscle type. According to immunohistochemical observation, 12 cases of thymus were divided into epithelial cell-dense type and loose epithelial type in 6 cases, the difference between the two groups was significant after the operation. Conclusion Pediatric whole-body MG should be treated surgically, while indications for simple ocular MG should be strictly controlled and medication should be used for preschool children as much as possible. The use of artificial respirator is an effective measure for postoperative myasthenia gravis crisis. Thymus immunohistochemical typing can better assess the efficacy of surgery.