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562例肝性脊髓病的临床特征分析
DOI: 10.3969/j.issn.1001-5256.2021.01.023
利益冲突声明：本研究不存在研究者、伦理委员会成员、受试者监护人以及与公开研究成果有关的利益冲突，特此声明。
作者贡献声明：杨明芝负责课题设计，数据采集，资料分析，撰写论文; 吴丹负责数据收集，分析解释数据; 唐亮参与数据收集，实施研究，分析解释数据; 文良志解释数据，统计分析，对文章的知识性内容作批评性审阅; 陈东风酝酿和设计实验分析，分析和解释数据，对文章的知识性内容作批评性审阅; 崔红利负责拟定写作思路，指导撰写文章并最后定稿。
Clinical features of hepatic myelopathy: An analysis of 562 cases
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摘要:
目的 总结我国肝性脊髓病(HM)患者的临床特征。 方法 收集2009年1月—2018年12月国内公开发表的HM相关文献，分析HM患者的临床特点、实验室检查结果、诊断治疗及预后情况。 结果 纳入文献94篇，共562例患者，其中男489例，女73例，年龄17~81岁，平均(46.3±17.5)岁。病因以乙型肝炎肝硬化多见(64.4%)，其次为酒精性肝硬化(10.3%)和丙型肝炎肝硬化(9.2%)。HM的临床表现主要有肌力下降(89.50%)、腱反射亢进(76.87%)、行走不稳及活动障碍(76.51%)等。实验室检查中血氨升高92.31%、白蛋白减少88.96%。影像学诊断主要依靠肌电图(64.92%)、脊髓MRI检查(22.82%)，单独的腹部彩超、CT及MRI诊断价值有限。经内科综合治疗后，治愈0例、好转106例(18.86%)、无效211例(37.54%)、加重31例(5.52%)、死亡129例(22.95%)、未说明53例(9.43%)。39例患者行肝移植手术，好转率56.41%。 结论 HM是终末期肝病的少见并发症，好发于中年男性，主要表现为慢性、进行性双下肢痉挛性截瘫。目前尚无有效治疗方法，部分患者可行肝移植术，总体治疗效果较差，预后不良。 Abstract:Objective To investigate the clinical features of patients with hepatic myelopathy (HM) in China. Methods The articles on HM, published in China from January 2009 to December 2018, were collected to analyze the clinical features, laboratory examination results, diagnosis, treatment, and prognosis of HM patients. Results A total of 94 articles were included, with 562 patients in total, among whom there were 489 male patients and 73 female patients. Their age ranged from 17-81 years, with a mean age of 46.3±17.5 years. Hepatitis B cirrhosis was the most common etiology (64.4%), followed by alcoholic cirrhosis (10.3%) and hepatitis C cirrhosis (9.2%). The clinical manifestations of HM mainly included decline of muscle strength (89.50%), tendon hyperreflexia (76.87%), ataxia, and movement disorder (76.51%). Laboratory examination showed an increase in blood ammonia by 92.31% and a reduction in albumin by 88.96%. Imaging diagnosis mainly depended on electromyography (64.92%) and spinal cord MRI (22.82%), and abdominal color Doppler ultrasound, CT, or MRI alone has limited clinical value. After comprehensive medical treatment, no patient (0%) was cured, 106 patients (18.86%) were improved, 211 patients (37.54%) had no response, 31 patients (5.52%) worsened, 129 patients (22.95%) died, and 53 patients (9.43%) were not explained. A total of 39 patients underwent liver transplantation, with an improvement rate of 56.41%. Conclusion HM is a rare complication of end-stage liver disease and is mainly observed in middle-aged men. It has the main manifestation of chronic and progressive spastic paraplegia of both lower limbs. Currently, there is no effective treatment method, and liver transplantation is feasible for some patients, with poor treatment response and poor prognosis. -
Key words:
- Hepatic Myelopathy /
- Liver Cirrhosis /
- Disease Attributes
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表 1 562例HM患者常见脊髓功能受损临床表现
临床表现 例数 百分比(%) 肌力下降 491 87.37 肌力0级 11 1.96 肌力1级 37 6.58 肌力2级 87 15.48 肌力3级 126 22.42 肌力4级 28 5.00 肌力5级 6 1.07 未分级 196 34.88 腱反射亢进 432 76.87 行走不稳、活动障碍 430 76.51 双下肢乏力 416 74.02 肌张力增强 411 73.13 病理征阳性 371 66.01 踝阵挛阳性 236 42.00 痉挛性截瘫 139 24.73 肌肉僵硬 76 13.52 感觉障碍 22 3.91 肌肉萎缩 7 1.25 主观麻木 6 1.07 括约肌功能障碍 2 0.36 
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表 2 562例HM患者异常实验室检查结果
指标 异常例数 受检例数 百分比(%) ALT升高 314 430 73.02 AST升高 292 430 67.91 血氨升高 276 299 92.31 白蛋白减少 266 299 88.96 总胆红素升高 214 264 81.06 直接胆红素升高 46 264 17.42 间接胆红素升高 27 264 10.23 未说明 141 264 53.41 肝炎标志物异常 184 237 77.64 乙型肝炎标志物阳性 166 237 70.04 丙型肝炎标志物阳性 18 237 7.59 PT延长 96 182 52.75 血常规异常 274 347 78.96 血小板减少 256 347 73.78 白细胞减少 215 347 61.96 红细胞减少 207 347 59.65 血红蛋白减少 202 347 58.21 维生素B12降低 4 42 9.52 铜蓝蛋白降低 3 76 3.95
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