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青少年重症肌无力与胸腺切除术:10例报告

Juvenile myasthenia gravis and thymectomy: report of ten cases.

作者信息

de Assis J L, Marchiori P E, Zambon A A, Scaff M

机构信息

Department of Neurology, University of São Paulo Medical School.

出版信息

Rev Hosp Clin Fac Med Sao Paulo. 1992 Sep-Oct;47(5):234-6.

PMID:1340608
Abstract

Ten patients from 7 to 12 years of age presented generalized acquired myasthenia gravis: six had a severe form and four a moderate one. All patients showed progressive worsening and poor response to the medical treatment; all of them were submitted to thymectomy by median sternotomy. Long-term results were beneficial for 60% of the patients who experimented a marked improvement or even complete remission. One patient improved without medication being followed for two years. Another patient has been in remission taking only a minimal dose of anticholinesterase drug for seven years. In most cases prednisone has been used as long-term maintenance in the postoperative phase. Short-term results were not favorable: one death occurred and 30% of the patients worsened.

摘要

10名7至12岁的患者出现了全身性获得性重症肌无力:6例为重度,4例为中度。所有患者均表现出病情逐渐恶化且对药物治疗反应不佳;他们均通过正中胸骨切开术接受了胸腺切除术。60%的患者取得了有益的长期结果,这些患者有明显改善甚至完全缓解。1例患者未用药病情改善,且随访了两年。另1例患者仅服用最小剂量的抗胆碱酯酶药物维持缓解状态达7年。在大多数情况下,泼尼松被用作术后阶段的长期维持治疗。短期结果并不理想:1例患者死亡,30%的患者病情恶化。

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