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儿科发病多发性硬化症的长期预后特征:来自土耳其的大量队列
Canan Duman İlki1, Ahmed Serkan Emekli2, Tuncay Gündüz2
1Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey; Department of Neurology, Sakarya University Training and Research Hospital, Sakarya, Turkey.
Multiple sclerosis and related disorders
|January 20, 2026
概括
儿科发病多发性硬化症 (POMS) 患者在14岁以后发病,脑干受影响或初始恢复不良,面临更早的残疾里程碑. 早期特征是优化POMS治疗策略的关键.
科学领域:
- 神经学 神经学
- 儿科 儿科 儿科
- 免疫学 免疫学 免疫学
背景情况:
- 儿科发病多发性硬化症 (POMS) 是一种罕见的疾病,具有与成人发病多发性硬化症不同的特征.
- 了解POMS的人口统计,临床过程和长期结果对于有效管理至关重要.
研究的目的:
- 描述土耳其第三级多发性硬化中心POMS患者的人口特征,临床表现和长期结果.
- 确定POMS中残疾进展和转化为二次进展性MS (SPMS) 的预测因素.
主要方法:
- 对143名在16岁之前被诊断出POMS患者的回顾性分析,在1981年至2018年期间进行监测.
- 收集的数据包括发病时的年龄,最初的症状,复发史和扩展残疾状态量表 (EDSS) 评分.
- 卡普兰-梅尔分析和考克斯回归被用来检查残疾里程碑和SPMS转换.
主要成果:
- 该队列 (69.2%的女性) 的中位随访时间为11年,中位发病年龄为14.6岁.
- 22.4%的人达到EDSS 4,16.8%的人转换为SPMS.
- 14岁后开始,男性性别,更高的基线EDSS和不完全的初始恢复预测了残疾进展. 延迟开始治疗也会增加风险.
结论:
- 在这个土耳其POMS队列中,晚期发病,脑干参与和初始恢复不良与早期残疾里程碑相关.
- 早期疾病特征显著影响POMS的预后.
- 基于早期预后因素优化治疗策略对于改善儿科MS的长期结果至关重要.
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