Nonalcoholic Fatty Liver Disease in Patients with Inherited and Sporadic Motor Neuron Degeneration.
Nonalcoholic Fatty Liver Disease in Patients with Inherited and Sporadic Motor Neuron Degeneration.
复制标题
作者：
我们描述了与对照组相比，具有遗传和散发病因的运动神经元变性形式的患者中脂肪肝疾病的证据。一组13名运动神经元病患者接受了肝脏成像和实验室分析。该队列包括5名遗传性痉挛性截瘫患者，4名散发性肌萎缩侧索硬化症（ALS）患者，3名家族性ALS患者和1名原发性侧索硬化症患者。13名运动神经元病（MND）患者中有9名报告了基因突变。13例MND患者中有10例（77%）通过磁共振波谱检测到脂肪肝，平均圆顶肝内三酰甘油含量为17%（范围2- 63%，参考≤5.5%）。肝脏超声显示13例患者中有6例（46%）有脂肪肝的证据，血清肝功能检测显示MND患者的丙氨酸氨基转移酶水平与年龄匹配的对照组相比显著升高。脂肪肝疾病可能代表各种形式的MND的非神经元临床成分。
We describe evidence of fatty liver disease in patients with forms of motor neuron degeneration with both genetic and sporadic etiology compared to controls. A group of 13 patients with motor neuron disease underwent liver imaging and laboratory analysis. The cohort included five patients with hereditary spastic paraplegia, four with sporadic amyotrophic lateral sclerosis (ALS), three with familial ALS, and one with primary lateral sclerosis. A genetic mutation was reported in nine of the thirteen motor neuron disease (MND) patients. Fatty liver disease was detected in 10 of 13 (77%) MND patients via magnetic resonance spectroscopy, with an average dome intrahepatic triacylglycerol content of 17% (range 2–63%, reference ≤5.5%). Liver ultrasound demonstrated evidence of fatty liver disease in 6 of the 13 (46%) patients, and serum liver function testing revealed significantly elevated alanine aminotransferase levels in MND patients compared to age-matched controls. Fatty liver disease may represent a non-neuronal clinical component of various forms of MND.
登录
查看更多内容
影响因子：
4.5
作者：
Joo JH;Kim HJ;Park EC;Jang SI
通讯作者：
Jang SI
影响因子：
4.6
作者：
Yoo, Dallah;Kim, Ryul;Lee, Jee-Young
通讯作者：
Lee, Jee-Young
影响因子：
29.4
作者：
Cotter, Thomas G.;Rinella, Mary
通讯作者：
Rinella, Mary
影响因子：
5.3
作者：
Deguise, Marc-Olivier;Baranello, Giovanni;Kothary, Rashmi
通讯作者：
Kothary, Rashmi
影响因子：
2.6
作者：
Hallsworth K;Thoma C;Moore S;Ploetz T;Anstee QM;Taylor R;Day CP;Trenell MI
通讯作者：
Trenell MI