肌肉病变的疾病谱与高的阿尔多酶和正常的肌酸激酶
Pannathat Soontrapa1,2, Shelly Shahar1,3,4, Lattawat Eauchai1,5
1Department of Neurology, Division of Neuromuscular Medicine, Mayo Clinic, Rochester, Minnesota, USA.
European journal of neurology
|November 3, 2023
概括
孤立的阿尔多酶升高可以表明各种可治疗的肌肉病变,最常见的是皮肤肌炎. 这种情况与肌肉肌肉炎 (dermatomyositis) 相比具有明显的特征,具有较高的肌酸激酶 (CK).
科学领域:
- 神经学 神经学
- 生物化学 生化学
- 免疫学 免疫学 免疫学
背景情况:
- 血清肌酸激酶 (CK) 升高是肌肉病的主要标志物.
- 在特定肌肉病症中观察到血清阿尔多酶与正常CK水平的孤立升高.
- 了解与孤立的阿尔多酶升高相关的肌肉病的谱系对于诊断和管理至关重要.
研究的目的:
- 为了研究由孤立的阿尔多酶升高的特征的神经病变的范围.
- 为了确定这些肌肉病的患病率和临床特征.
主要方法:
- 对18岁以上患者的病理确诊肌肉病变,阿尔多酶升高和正常CK水平的医疗记录的回顾性审查.
- 排除患有另类原因导致阿尔多酶升高的患者.
- 皮肤肌炎患者与孤立的阿尔多酶升高患者与CKCK高血症患者的比较.
主要成果:
- 确定了34名患有肌肉病变和孤立的阿尔多酶升高的患者;27人患有可治疗的疾病.
- 皮肤肌肉炎 (n=8),重叠肌肉炎 (n=4) 和非特异性肌肉病 (n=4) 是最常见的诊断.
- 在50%的病例中存在周围病理. 与CK高血症的皮肤肌炎相比,正常CK的皮肤肌炎表现出较少的皮肤干扰和纤维化潜力,但红细胞沉积率更高,外围状线粒体病理更频繁.
结论:
- 孤立的阿尔多酶升高与比以前更广泛的肌肉病变的频谱有关,大多数是可以治疗的.
- 皮肤肌肉炎是这个群体中最常见的肌肉病变,当CK水平正常时,它表现出独特的临床和病理特征.
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