儿童发病性硬化肌瘤国家注册表I:来自341名青少年局部性硬化肌瘤患者的见解
Samantha A Branton1, Leigh A Stubbs2, Haley J Havrilla3
1University of Pittsburgh, Pittsburgh, PA, USA.
Journal of scleroderma and related disorders
|November 15, 2024
概括
早期识别青少年发病局部性硬化症至关重要. 这种情况往往呈现出皮肤外表现,需要全身治疗和全面查,以获得更好的患者结果.
科学领域:
- 儿科风湿病学 儿科风湿病学
- 皮肤病学 皮肤病学
- 自免疫性疾病 自免疫性疾病
背景情况:
- 青少年发病局部性硬化皮肤病 (JOLS) 经常被低诊断.
- 在JOLS中,皮肤外表现 (ECM) 可能导致系统治疗延迟.
- 缺乏对JOLS人口统计和呈现的全面了解.
研究的目的:
- 描述JOLS.的人口统计,表现和疾病过程.
- 在JOLS.中识别常见的皮肤外表现和相关的实验室发现.
- 为了评估JOLS.系统治疗的有效性.
主要方法:
- 分析了341名来自国家儿童发作硬化肌病登记册的患者.
- 对人口统计数据,临床表现和实验室值的审查.
- 使用经过验证的工具来评估治疗结果,例如局部性硬化皮肤皮肤评估工具.
主要成果:
- 大多数JOLS患者是女性 (71%),高加索人 (94%),具有线性亚型 (56%) 并在7.5年开始.
- 观察到皮肤外表现的高患病率 (70%),主要是肌肉骨 (57%) 和神经 (46%).
- 系统治疗导致皮肤和损伤指数显著改善,分别p<0.001和p=0.001.
结论:
- 诊断JOLS存在显著的延迟,强调了早期识别的必要性.
- 在JOLS中频繁出现的皮肤外表现导致了大量的疾病负担.
- 系统治疗和彻底查皮肤外表现对JOLS患者有益.
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