关键词: Glucocorticoid Ocular myasthenia gravis Outcome Ptosis Treatment

Mesh : Humans Child Myasthenia Gravis / diagnosis drug therapy Blepharoptosis / drug therapy etiology Pyridostigmine Bromide / therapeutic use Glucocorticoids / therapeutic use Retrospective Studies

来  源:   DOI:10.1016/j.pediatrneurol.2024.01.014

Abstract:
BACKGROUND: Currently, there is no universally accepted standard treatment for ocular myasthenia gravis (OMG) in children. We aimed to investigate the possible proper regimens and timing of treatment for pediatric OMG cases based on the clinical manifestations: OMG with ptosis only and OMG with other features.
METHODS: One hundred and forty two OMG cases attended at the Department of Pediatrics, Xiangya Hospital, Central South University, from 2010 to 2019 were included, and information from medical records was reviewed and recorded. Comparisons of clinical characteristics between patients with OMG with ptosis only and patients with OMG with other features as well as between patients treated with glucocorticoid (GC) within or after six months from disease onset were performed.
RESULTS: OMG with other features constituted about 54.9% of the cases, and 66.2% of the patients achieved optimal outcome. Patients with OMG with ptosis only responded to pyridostigmine alone more than patients with OMG with other features who required several therapies (P < 0.001). Patients with OMG with ptosis only had a larger proportion of optimal outcome than the patients with OMG with other features (P = 0.002), and the difference remained significant even when the individual outcome groups were compared (P < 0.001). Patients who received GC within six months had a greater proportion of optimal outcome than those who received it after six months (P < 0.001).
CONCLUSIONS: Although OMG with other features is a more common subtype of OMG, it is also more severe than OMG with ptosis only. An earlier addition of GC leads to optimal outcome.
摘要:
背景:目前,儿童眼部重症肌无力(OMG)尚无普遍接受的标准治疗方法.我们旨在根据临床表现探讨小儿OMG的可能的适当治疗方案和时机:仅有上下垂的OMG和具有其他特征的OMG。
方法:儿科就诊的一百四十二例OMG病例,湘雅医院,中南大学,包括2010年至2019年,对病历中的信息进行了审查和记录.比较仅患有上睑下垂的OMG患者与具有其他特征的OMG患者之间以及在疾病发作后六个月内或之后接受糖皮质激素(GC)治疗的患者之间的临床特征。
结果:OMG与其他特征构成约54.9%的病例,66.2%的患者达到最佳结果。与需要多种治疗的具有其他特征的OMG患者相比,仅对上睑下垂的OMG患者仅对吡啶斯的明有反应(P<0.001)。与具有其他特征的OMG患者相比,OMG患者仅有更大比例的最佳结果(P=0.002),即使比较了各个结局组,差异仍具有统计学意义(P<0.001)。在6个月内接受GC的患者比在6个月后接受GC的患者具有更大比例的最佳结果(P<0.001)。
结论:尽管具有其他特征的OMG是OMG的更常见亚型,它也比OMG更为严重。较早添加GC导致最佳结果。
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