NEuroMuscular Omnicentre (NEMO), ASST Grande Ospedale Metropolitano Niguarda, Fondazione Serena Onlus, Piazza Ospedale Maggiore, 3, 20162, Milan, Italy.
Neurology Unit, San Gerardo Hospital, ALS Clinic, University of Milano-Bicocca, Monza, Italy.
J Neurol. 2018 Dec;265(12):3001-3008. doi: 10.1007/s00415-018-9093-3. Epub 2018 Oct 22.
The progression of amyotrophic lateral sclerosis (ALS) leads to a decline of the nutritional status that represents an independent prognostic factor for survival. Recent studies recognize the muscle tissue as an endocrine organ able to release several molecules, called myokines. Among them, irisin seems to be involved in the regulation of metabolism, body weight and development and function of the nervous system.
(1) To evaluate irisin serum levels in patients with ALS, with comparison to healthy subjects; (2) to assess the possible association of circulating irisin levels of ALS patients with the metabolic status, clinical and biochemical features.
We performed an observational, cross-sectional study in 50 ALS patients and 32 age- and sex-comparable healthy controls. Patients underwent to a complete set of neurological, pulmonary and nutritional evaluations. Serum irisin concentration was measured by enzyme immunoassay. According to indirect calorimetry, ALS patients were divided into a normo-metabolic patient group (n = 24) and a hyper-metabolic patient group (n = 26).
ALS patients showed significantly higher serum irisin levels compared to healthy subjects (51.0 ± 37.8 vs 13.1 ± 2.2 ng/mL, p < 0.0001). Hyper-metabolic ALS patients displayed higher serum irisin levels compared to normo-metabolic ALS patients and healthy controls (p < 0.0009 and p < 0.0001, respectively). Serum irisin levels showed significant association with the ALSFRS-R (β=-1.18, p = 0.042), Forced Vital Capacity (β = - 0.64, p = 0.013), Fat Mass (β=-1.44, p = 0.034), pCO arterial blood levels (β = 2.67, p = 0.003), HCO arterial blood levels (β = 5.44, p = 0.001) and Free Fat Mass (β = 1.07, p = 0.025) adjusted for sex, age and metabolic status.
ALS patients with impaired metabolic status showed higher serum irisin levels compared to normo-metabolic ALS patients and healthy subjects. Irisin levels were also negatively correlated with the extent of functional and respiratory impairment, due to as yet unknown causes, being more elevated in patients with greater disability.
肌萎缩侧索硬化症（ALS）的进展导致营养状况下降，这是生存的独立预后因素。最近的研究将肌肉组织识别为一种能够释放多种分子的内分泌器官，这些分子被称为肌因子。其中，鸢尾素似乎参与了代谢、体重以及神经系统的发育和功能的调节。
（1）评估 ALS 患者的血清鸢尾素水平，并与健康对照者进行比较；（2）评估 ALS 患者循环鸢尾素水平与代谢状态、临床和生化特征的可能相关性。
我们对 50 名 ALS 患者和 32 名年龄和性别相匹配的健康对照者进行了一项观察性、横断面研究。患者接受了全面的神经学、肺功能和营养评估。通过酶联免疫吸附法测定血清鸢尾素浓度。根据间接量热法，将 ALS 患者分为代谢正常组（n=24）和代谢亢进组（n=26）。
与健康对照组相比，ALS 患者的血清鸢尾素水平显著升高（51.0±37.8 与 13.1±2.2ng/ml，p<0.0001）。与代谢正常的 ALS 患者和健康对照组相比，代谢亢进的 ALS 患者的血清鸢尾素水平更高（p<0.0009 和 p<0.0001）。血清鸢尾素水平与 ALSFRS-R 评分（β=-1.18，p=0.042）、用力肺活量（β=-0.64，p=0.013）、脂肪量（β=-1.44，p=0.034）、动脉血 pCO2 水平（β=2.67，p=0.003）、动脉血 HCO3-水平（β=5.44，p=0.001）和游离脂肪量（β=1.07，p=0.025）呈显著负相关，这些变量均经过性别、年龄和代谢状态调整。
代谢状态受损的 ALS 患者的血清鸢尾素水平高于代谢正常的 ALS 患者和健康对照组。由于未知原因，鸢尾素水平与功能和呼吸损伤的严重程度呈负相关，在残疾程度较高的患者中，鸢尾素水平更高。
