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早发型重症肌无力:临床特征及治疗反应

Early-onset myasthenia gravis: clinical characteristics and response to therapy.

作者信息

Batocchi A P, Evoli A, Palmisani M T, Lo Monaco M, Bartoccioni M, Tonali P

机构信息

Institute of Neurology, Catholic University, Rome, Italy.

出版信息

Eur J Pediatr. 1990 Nov;150(1):66-8. doi: 10.1007/BF01959485.

Abstract

We studied 59 children with myasthenia gravis (MG). Disease onset was pre-pubertal in 26 patients and post-pubertal in 33. The male to female ratio was 0.62 in the early- and 0.17 in the late-onset groups. The frequency of ocular MG was higher in patients with prepubertal onset. Patients with generalized MG generally showed a good response to thymectomy and corticosteroid therapy proved effective with no major side-effects. In our experience early-onset MG has the same course as in adult life. We recommend thymectomy for generalized disease in childhood, except in very young children on account of possible long-term effects on immunological development. Immunosuppressive therapy should be considered in severely affected patients who do not respond adequately to other therapies.

摘要

我们研究了59例重症肌无力(MG)患儿。26例患者发病于青春期前,33例发病于青春期后。早发组男女比例为0.62,晚发组为0.17。青春期前发病的患者眼肌型MG的发生率更高。全身型MG患者通常对胸腺切除术反应良好,皮质类固醇治疗有效且无重大副作用。根据我们的经验,早发型MG的病程与成人相同。我们建议对儿童全身型疾病进行胸腺切除术,但非常年幼的儿童除外,因为可能对免疫发育有长期影响。对于对其他治疗反应不佳的重症患者,应考虑免疫抑制治疗。

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