儿科发病多发性硬化症 (POMS):从丹麦多发性硬化症注册表 (DMSR) 探索表型特征和青春期影响调节疾病活性
Silvy Pilotto1, Luigi Pontieri2, Helle Hvilsted Nielsen3
1Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.
概括
青春期显著影响儿科多发性硬化症 (MS) 的发展. 早期发病与更高的残疾相关,但复发率较低,活动在青春期达到顶峰.
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) 很少见 (约5%的MS病例),并带来独特的挑战.
- 青春期期间的荷尔蒙变化可能会影响POMS的表现,复发率和长期结果.
研究的目的:
- 检查青春期阶段如何影响POMS的临床特征,复发活动和残疾进展.
- 使用来自丹麦多发性硬化病登记处 (DMSR) 的数据.
主要方法:
- 185名POMS患者的全国队列根据青春期阶段进行分类:青春期前 (<11岁),青春期前 (11-14岁) 和青春期后 (>14岁).
- 对比人口统计,症状,MRI发现,复发率和扩展残疾状况量表 (EDSS) 成绩.
- 在54名过渡到青春期的患者中,纵向分析了复发率.
主要成果:
- 青春期前发作与严重的症状 (如小脑),较高的损伤负担和增加的10年残疾相关 (EDSS中位数为3.75).
- 青春期前发病率显示年复发率较低 (ARR=0.200),而青春期前发病率达到峰值 (ARR=0.302).
- 男性的性别与复发率的降低有关;女性与男性的比例从1:1转移到约2:1的青春期后.
结论:
- 青春期显著改变了POMS的疾病过程.
- 强调需要早期,性别特定的干预和主动监测.
- 对荷尔蒙影响POMS进展和治疗的进一步研究是有必要的.
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