肌酸激酶与肌萎缩侧索硬化症的进展速度。
Creatine Kinase and Progression Rate in Amyotrophic Lateral Sclerosis.
机构信息
Rare Neuromuscular Diseases Centre, Department of Human Neurosciences, Sapienza University of Rome, 00185 Rome, Italy.
Centre for Neuromuscular and Neurological Rare Diseases, San Camillo Forlanini Hospital, 00152 Rome, Italy.
出版信息
Cells. 2020 May 8;9(5):1174. doi: 10.3390/cells9051174.
Amyotrophic lateral sclerosis ALS) is a neurodegenerative disease with no recognized clinical prognostic factor. Creatinine kinase (CK) increase in these patients is already described with conflicting results on prognosis and survival. In 126 ALS patients who were fast or slow disease progressors, CK levels were assayed for 16 months every 4 months in an observational case-control cohort study with prospective data collection conducted in Italy. CK was also measured at baseline in 88 CIDP patients with secondary axonal damage and in two mouse strains (129SvHSD and C57-BL) carrying the same SOD1G93A transgene expression but showing a fast (129Sv-SOD1G93A) and slow (C57-SOD1G93A) ALS progression rate. Higher CK was found in ALS slow progressors compared to fast progressors in T1, T2, T3, and T4, with a correlation with Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) scores. Higher CK was found in spinal compared to bulbar-onset patients. Transgenic and non-transgenic C57BL mice showed higher CK levels compared to 129SvHSD strain. At baseline mean CK was higher in ALS compared to CIDP. CK can predict the disease progression, with slow progressors associated with higher levels and fast progressors to lower levels, in both ALS patients and mice. CK is higher in ALS patients compared to patients with CIDP with secondary axonal damage; the higher levels of CK in slow progressors patients, but also in C57BL transgenic and non-transgenic mice designs CK as a predisposing factor for disease rate progression.
肌萎缩侧索硬化症（ALS）是一种神经退行性疾病，目前尚无公认的临床预后因素。这些患者的肌酸激酶（CK）升高已有报道，但对预后和生存的影响结果存在争议。在意大利进行的一项观察性病例对照队列研究中，对 126 名快速或缓慢进展的 ALS 患者进行了为期 16 个月、每 4 个月 1 次的 CK 水平检测，前瞻性地收集了数据。在 88 名伴有继发性轴索损伤的 CIDP 患者和 2 种携带相同 SOD1G93A 转基因表达但表现出快速（129Sv-SOD1G93A）和缓慢（C57-SOD1G93A）ALS 进展率的小鼠品系（129SvHSD 和 C57-BL）中，基线时也测量了 CK。在 T1、T2、T3 和 T4 时，缓慢进展的 ALS 患者的 CK 高于快速进展的 ALS 患者，且与修订版肌萎缩侧索硬化功能评定量表（ALSFRS-R）评分呈正相关。与延髓起病患者相比，脊髓起病患者的 CK 更高。与 129SvHSD 品系相比，转基因和非转基因 C57BL 小鼠的 CK 水平更高。与 CIDP 相比，ALS 患者的 CK 水平更高。在 ALS 患者和小鼠中，CK 可预测疾病进展，进展缓慢的患者 CK 水平较高，进展较快的患者 CK 水平较低。与伴有继发性轴索损伤的 CIDP 患者相比，ALS 患者的 CK 水平更高；在缓慢进展的 ALS 患者和 C57BL 转基因和非转基因小鼠中，CK 水平较高，提示 CK 是疾病进展率的一个易感因素。
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