126例肝性脊髓病患者临床特征分析
DOI: 10.12449/JCH260821
-
摘要:
目的 分析126例肝性脊髓病患者的临床资料,总结其临床表现及预后特征,为临床诊疗提供参考依据。 方法 收集2016年1月1日—2025年6月30日就诊于兰州大学第一医院的126例肝性脊髓病患者的临床资料,根据入院时肌力检测将患者分为肌力<3级组、肌力3级组和肌力4级组,分析比较3组患者的临床资料,包括病因、症状、临床体征、既往史、个人史、实验室检验、影像学检查、治疗及转归情况。符合正态分布的计量资料多组间比较采用方差分析,进一步两两比较采用LSD-t检验;不符合正态分布的计量资料多组间比较采用Kruskal-Wallis H检验,进一步两两比较采用Bonferroni校正法。计数资料多组间比较采用χ2检验。 结果 126例肝性脊髓病患者均为肝硬化背景,男女比例约为2.7∶1,年龄23~73岁,平均(53.9±9.3)岁,中位年龄54岁。肝硬化病因前三位分别为乙型肝炎78例(61.9%)、丙型肝炎9例(7.1%)、酒精性肝炎8例(6.3%)。患者病程中均有双下肢乏力表现,其中9例(7.1%)伴随下肢疼痛及麻木症状。126例患者中肌力0级3例(2.4%)、1级4例(3.2%)、2级9例(7.1%)、3级39例(31.0%)、4级71例(56.3%)。3组不同肌力患者组间比较结果显示,在合并症方面,显性肝性脑病(OHE)(χ2=6.254,P=0.044)、腹水(χ2=6.248,P=0.044)和肝衰竭(χ2=7.704,P=0.029)构成比差异均有统计学意义;实验室指标方面,红细胞(RBC)(F=5.334,P=0.006)、血红蛋白(HGB)(F=4.627,P=0.012)、天冬氨酸氨基转移酶(AST)(H=6.866,P=0.032)、丙氨酸氨基转移酶(H=6.444,P=0.040)、白蛋白(Alb)(F=6.331,P=0.002)、胆碱酯酶(ChE)(H=11.064,P=0.004)、总胆固醇(TC)(F=4.843,P=0.009)、凝血酶原活动度(PTA)(F=3.937,P=0.022)及国际标准化比值(INR)(H=6.448,P=0.040)比较差异亦均有统计学意义。其中,肌力<3级组患者RBC、HGB和ChE水平显著低于肌力4级组(P值均<0.05),AST水平显著高于肌力4级组(P<0.05);与肌力3级组比较,肌力<3级组患者Alb、TC和PTA明显降低(P值均<0.05),INR明显升高(P<0.05)。出院时,好转者27例(21.4%),无效者99例(78.6%),其中死亡2例。 结论 肝性脊髓病以双下肢对称性、痉挛性截瘫为主要临床表现,好发于男性,病因主要为乙型肝炎、丙型肝炎及酒精性肝炎,该病的肌力强弱与合并OHE、腹水和肝衰竭有关,肌力较弱患者的RBC、HGB、Alb、ChE、TC及PTA水平较低,而AST和INR水平较高,提示上述指标变化与患者肌力减退程度可能存在相关性。肝性脊髓病预后差,目前尚无有效治疗手段。 Abstract:Objective To analyze the clinical data of 126 patients with hepatic myelopathy (HM), to summarize their clinical manifestations and prognostic features, and to provide a reference for clinical diagnosis and treatment. Methods Related clinical data were collected from 126 HM patients who attended The First Hospital of Lanzhou University from January 1, 2016 to June 30, 2025, and according to their muscle strength on admission, they were divided into grade<3 group, grade 3 group, and grade 4 group. The three groups were compared in terms of the clinical data including etiology, symptoms, clinical signs, past history, personal history, laboratory test results, imaging findings, treatment, and outcome. An analysis of variance was used for comparison of normally distributed continuous data between multiple groups, and the least significant difference t-test was used for further comparison between two groups; the Kruskal-Wallis H test was used for comparison of non-normally distributed continuous data between multiple groups, and the Bonferroni correction method was used for further comparison between two groups; the chi-square test was used for comparison of categorical data between multiple groups. Results All 126 patients with HM were comorbid with liver cirrhosis, with a male/female ratio of 2.7∶1, an age of 23 — 73 years, a mean age of 53.9±9.3 years, and a median age of 54 years. The top three etiologies of liver cirrhosis were hepatitis B in 78 patients (61.9%), hepatitis C in 9 patients (7.1%), and alcoholic hepatitis in 8 patients (6.3%). During the course of the disease, all 126 patients with HM showed weakness in both lower limbs, among whom 9 patients (7.1%) also had the symptoms of lower limb pain and numbness. Among the 126 patients, 3 (2.4%) had grade 0 muscle strength, 4 (3.2%) had grade 1 muscle strength, 9 (7.1%) had grade 2 muscle strength, 39 (31.0%) had grade 3 muscle strength, and 71 (56.3%) had grade 4 muscle strength. Comparison of related data between the three groups of patients with varying grades of muscle strength showed that as for comorbidities, there were significant differences between the three groups in the constituent ratios of overt hepatic encephalopathy (OHE) (χ2=6.254, P=0.044), ascites (χ2=6.248, P=0.044), and liver failure (χ2=7.704, P=0.029); as for laboratory markers, there were significant differences between the three groups in red blood cell count (RBC) (F=5.334, P=0.006), hemoglobin (HGB) (F=4.627, P=0.012), aspartate aminotransferase (AST) (H=6.866, P=0.032), alanine aminotransferase (H=6.444, P=0.040), albumin (Alb) (F=6.331, P=0.002), cholinesterase (ChE) (H=11.064, P=0.004), total cholesterol (TC) (F=4.843, P=0.009), prothrombin activity (PTA) (F=3.937, P=0.022) and international normalized ratio (INR) (H=6.448, P=0.040). Compared with the grade 4 group, the grade<3 group had significantly lower levels of RBC, HGB, and ChE (all P<0.05) and a significantly higher level of AST (P<0.05); compared with the grade 3 group, the grade<3 group had significant reductions in Alb, TC, and PTA (all P<0.05) and a significant increase in INR (P<0.05). At discharge, 27 patients (21.4%) achieved improvement, and 99 patients (78.6%) showed no response, among whom 2 patients died. Conclusion HM has the main clinical manifestation of symmetrical spastic paraplegia of both lower limbs, and it is more common in men. The main causes of HM include hepatitis B, hepatitis C, and alcoholic hepatitis. Muscle strength in HM patients is associated with the comorbidity of OHE, ascites, or liver failure, and patients with weak muscle strength tend to have low levels of RBC, HGB, Alb, ChE, TC, and PTA and high levels of AST and INR, suggesting that the changes in these indicators may be correlated with the degree of muscle strength impairment. HM often has a poor prognosis, and there are currently no effective treatment methods. -
Key words:
- Hepatic Myelopathy /
- Liver Cirrhosis /
- Signs and Symptoms
-
表 1 徒手肌力检测量表
Table 1. Manual muscle testing
肌力级别 标准 0级 肌肉无任何收缩 1级 肌肉有轻微收缩 2级 去除重力可做主动运动 3级 不施加阻力时,可抗重力做主动运动 4级 抗重力及部分阻力做主动运动 5级 正常力量 表 2 肝性脊髓病患者一般临床资料
Table 2. General clinical data of hepatic myelopathy patients
项目 肌力<3级组(n=16) 肌力3级组(n=39) 肌力4级组(n=71) χ2值 P值 男[例(%)] 11(68.8) 33(84.6) 48(67.6) 3.866 0.145 肝硬化病因[例(%)] 26.652 0.170 乙型肝炎 10(62.5) 28(71.8) 40(56.3) 丙型肝炎 1(6.3) 0(0.0) 8(11.3) 酒精性肝炎 1(6.3) 4(10.3) 3(4.2) 既往史[例(%)] 糖尿病 0(0.0) 8(20.5) 10(14.1) 3.904 0.142 高血压 0(0.0) 1(2.6) 9(12.7) 5.103 0.078 吸烟史 1(6.3) 8(20.5) 9(12.7) 2.229 0.328 饮酒史 3(18.8) 9(23.1) 11(15.5) 0.973 0.615 脾肾分流[例(%)] 0(0.0) 3(7.7) 11(15.5) 3.842 0.146 附脐静脉开放[例(%)] 2(12.5) 6(15.4) 14(19.7) 0.641 0.726 TIPS[例(%)] 9(56.3) 17(43.6) 36(50.7) 0.874 0.646 脾切除术[例(%)] 4(25.0) 7(17.9) 14(19.7) 0.356 0.837 合并症[例(%)] OHE 10(62.5) 29(74.4) 62(87.3) 6.254 0.044 腹水 11(68.8) 18(46.2) 25(35.2) 6.248 0.044 门静脉血栓 2(12.5) 13(33.3) 21(29.6) 2.494 0.287 消化道出血 11(68.8) 16(41.0) 34(47.9) 3.510 0.173 感染 6(37.5) 11(28.2) 11(15.5) 4.829 0.089 肝癌 2(12.5) 3(7.7) 13(18.3) 2.365 0.306 电解质紊乱 8(50.0) 13(33.3) 31(43.7) 1.684 0.431 肝衰竭 10(62.5) 10(25.6) 23(32.4) 7.704 0.029 注:TIPS,经颈静脉肝内门体分流术;OHE,显性肝性脑病。
表 3 肝性脊髓病患者实验室检验结果
Table 3. Laboratory test data of hepatic myelopathy patients
项目 肌力<3级组(n=16) 肌力3级组(n=39) 肌力4级组(n=71) 统计值 P值 年龄(岁) 53.6±1.9 52.7±1.8 54.5±1.0 F=0.579 0.749 血氨(μmol/L) 45.9(31.8~67.6) 47.8(29.0~83.4) 36.0(25.8~63.2) H=2.540 0.281 RBC(×109/L) 3.1±0.1 3.5±0.1 3.7±0.11) F=5.334 0.006 HGB(g/L) 100.2±3.4 108.7±4.6 118.6±2.81) F=4.627 0.012 WBC(×1012/L) 5.3(2.0~7.7) 2.6(2.0~4.1) 3.2(1.9~4.2) H=4.387 0.112 PLT(×109/L) 61.0(31.5~88.3) 50.0(43.0~100.0) 63.0(41.0~104.0) H=0.536 0.765 AST(U/L) 69.1(37.8~99.6) 44.0(30.0~70.0) 44.0(33.0~57.0)1) H=6.866 0.032 ALT(U/L) 44.7(24.5~74.9) 27.0(18.0~41.0) 26.0(21.0~40.0) H=6.444 0.040 TBil(μmol/L) 46.2(34.2~288.2) 41.0(30.2~61.2) 51.1(34.5~82.7) H=3.259 0.196 DBil(μmol/L) 15.7(9.5~130.7) 12.4(8.5~19.3) 14.1(8.0~22.4) H=2.061 0.357 IBil(μmol/L) 30.2(24.9~87.9) 28.8(20.9~44.0) 36.2(25.9~60.1) H=3.084 0.214 Alb(g/L) 27.1±1.8 31.4±1.11) 32.9±0.6 F=6.331 0.002 ALP(U/L) 147.8(90.4~198.9) 122.4(80.1~159.1) 127.4(85.3~156.0) H=2.133 0.344 ChE(kU/L) 2.2(1.3~3.0) 2.9(2.1~3.6) 3.3(2.5~4.2)1) H=11.064 0.004 Urea(mmol/L) 6.0(4.2~8.1) 5.8(4.1~7.9) 5.4(4.1~6.9) H=1.963 0.375 Cr(μmol/L) 68.4(60.7~77.9) 65.0(57.0~73.7) 65.3(56.0~72.1) H=1.080 0.583 Na+(mmol/L) 137.2(132.4~142.8) 139.0(135.3~141.0) 138.4(136.0~141.0) H=1.306 0.521 K+(mmol/L) 4.0(3.4~4.4) 3.9(3.7~4.2) 4.0(3.7~4.5) H=0.871 0.647 Ca2+(mmol/L) 1.98±0.05 2.03±0.03 2.08±0.02 F=2.121 0.124 TC(mmol/L) 1.93±0.28 2.76±0.161) 2.66±0.10 F=4.843 0.009 TG(mmol/L) 0.56(0.39~0.68) 0.63(0.46~0.94) 0.50(0.40~0.64) H=4.428 0.109 Glu(mmol/L) 5.3(4.7~6.8) 5.2(4.6~7.0) 5.1(4.3~6.1) H=2.423 0.298 PT(s) 18.9(16.3~22.3) 16.5(14.4~18.2) 16.4(14.8~18.7) H=5.558 0.062 PTA(%) 47.9±3.8 61.4±3.11) 57.8±1.7 F=3.937 0.022 INR 1.76(1.43~2.06) 1.47(1.25~1.62)1) 1.48(1.34~1.64) H=6.448 0.040 MELD评分(分) 16.0(13.5~25.1) 15.0(13.0~16.0) 15.0(12.0~18.0) H=3.787 0.151 Child-Pugh评分(分) 9.6±0.6 8.6±0.3 8.6±0.2 F=2.210 0.114 注:与肌力<3级组比较,1)P<0.05。RBC,红细胞;HGB,血红蛋白;WBC,白细胞;PLT,血小板;AST,天冬氨酸氨基转移酶;ALT,丙氨酸氨基转移酶;TBil,总胆红素;DBil,直接胆红素;IBil,间接胆红素;Alb,白蛋白;ALP,碱性磷酸酶;ChE,胆碱酯酶;Urea,尿素;Cr,血肌酐;Na+,血清钠离子;K+,血清钾离子;Ca2+,血清钙离子;TC,总胆固醇;TG,甘油三酯;Glu,血清葡萄糖;PT,凝血酶原时间;PTA,凝血酶原活动度;INR,国际标准化比值;MELD,终末期肝病模型;Child-Pugh评分,蔡尔德-皮尤评分。
-
[1] Utku U, Asil T, Balci K, et al. Hepatic myelopathy with spastic paraparesis[J]. Clin Neurol Neurosurg, 2005, 107( 6): 514- 516. DOI: 10.1016/j.clineuro.2004.10.002. [2] Conn H O, Rössle M, Levy L, et al. Portosystemic myelopathy: Spastic paraparesis after portosystemic shunting[J]. Scand J Gastroenterol, 2006, 41( 5): 619- 625. DOI: 10.1080/00365520500318932. [3] Ma Fuquan, Huang Jin, Li Weizhi, et al. Effects of reduced portosystemic flow on hepatic myelopathy in patients with cirrhosis after TIPS[J]. Chin J Hepatol, 2022, 30( 10): 1063- 1068. DOI: 10.3760/cma.j.cn501113-20201026-00580.马富权, 黄金, 李伟之, 等. 减少门腔分流量对肝硬化患者经颈静脉肝内门体分流术后肝性脊髓病的影响[J]. 中华肝脏病杂志, 2022, 30( 10): 1063- 1068. DOI: 10.3760/cma.j.cn501113-20201026-00580. [4] Nardone R, Höller Y, Storti M, et al. Spinal cord involvement in patients with cirrhosis[J]. World J Gastroenterol, 2014, 20( 10): 2578- 2585. DOI: 10.3748/wjg.v20.i10.2578. [5] Kroupina K, Bémeur C, Rose C F. Amino acids, ammonia, and hepatic encephalopathy[J]. Anal Biochem, 2022, 649: 114696. DOI: 10.1016/j.ab.2022.114696. [6] Li M, Wang Z Q, Zhang L, et al. Burden of cirrhosis and other chronic liver diseases caused by specific etiologies in China, 1990-2016: Findings from the global burden of disease study 2016[J]. Biomed Environ Sci, 2020, 33( 1): 1- 10. DOI: 10.3967/bes2020.001. [7] Haj M, Rockey D C. Ammonia levels do not guide clinical management of patients with hepatic encephalopathy caused by cirrhosis[J]. Am J Gastroenterol, 2020, 115( 5): 723- 728. DOI: 10.14309/ajg.0000000000000343. [8] Chen Zhihui, Chen Dongfeng. Progress of hepatic myelopathy[J]. Chin J Gastroenterol Hepatol, 2016, 25( 7): 832- 834. DOI: 10.3969/j.issn.1006-5709.2016.07.028.陈志惠, 陈东风. 肝性脊髓病研究进展[J]. 胃肠病学和肝病学杂志, 2016, 25( 7): 832- 834. DOI: 10.3969/j.issn.1006-5709.2016.07.028. [9] Huang Jin, Xue Hui, Ma Fuquan. Research progress of hepatic myelopathy[J]. Mod Dig Interv, 2024, 29( 8): 1011- 1014. DOI: 10.3969/j.issn.1672-2159.2024.08.027.黄金, 薛挥, 马富权. 肝性脊髓病的研究进展[J]. 现代消化及介入诊疗, 2024, 29( 8): 1011- 1014. DOI: 10.3969/j.issn.1672-2159.2024.08.027. [10] Wang M Q, Dake M D, Cui Z P, et al. Portal-systemic myelopathy after transjugular intrahepatic portosystemic shunt creation: Report of four cases[J]. J Vasc Interv Radiol, 2001, 12( 7): 879- 881. DOI: 10.1016/s1051-0443(07)61514-0. [11] Sureka B, Bansal K, Patidar Y, et al. Neurologic manifestations of chronic liver disease and liver cirrhosis[J]. Curr Probl Diagn Radiol, 2015, 44( 5): 449- 461. DOI: 10.1067/j.cpradiol.2015.03.004. [12] Baltrusch S. The role of neurotropic B vitamins in nerve regeneration[J]. Biomed Res Int, 2021, 2021: 9968228. DOI: 10.1155/2021/9968228. [13] Chinese Society of Hepatology, Chinese Medical Association. Chinese guidelines on the management of liver cirrhosis[J]. J Clin Hepatol, 2019, 35( 11): 2408- 2425. DOI: 10.3969/j.issn.1001-5256.2019.11.006.中华医学会肝病学分会. 肝硬化诊治指南[J]. 临床肝胆病杂志, 2019, 35( 11): 2408- 2425. DOI: 10.3969/j.issn.1001-5256.2019.11.006. [14] Caldwell C, Werdiger N, Jakab S, et al. Use of model for end-stage liver disease exception points for early liver transplantation and successful reversal of hepatic myelopathy with a review of the literature[J]. Liver Transplant, 2010, 16( 7): 818- 826. DOI: 10.1002/lt.22077. [15] Koul R, Lal B B, Pamecha V, et al. Liver transplantation reverses hepatic myelopathy in 2 children with hepatitis A infection[J]. Child Neurol Open, 2021, 8: 2329048 X 20983763. DOI: 10.1177/2329048X20983763. [16] Li Huiwen, Xu Yubo, Xu Zehua, et al. Treating hepatic myelopathy with chronic and acute liver failure caused by stopping antiviral drugs by acupuncture[J]. Clin J Chin Med, 2022, 14( 35): 53- 56. DOI: 10.3969/j.issn.1674-7860.2022.35.015.李惠文, 许钰波, 许泽华, 等. 针刺治疗停抗病毒药致肝性脊髓病截瘫伴慢加急性肝衰竭案[J]. 中医临床研究, 2022, 14( 35): 53- 56. DOI: 10.3969/j.issn.1674-7860.2022.35.015. [17] Xu Yubo, Li Yuewei, Li Chengyu. One case of end-stage complications of hepatic myelopathy by Qi-acupuncture of the TCM plus medicine[J]. Clin J Chin Med, 2021, 13( 23): 56- 58. DOI: 10.3969/j.issn.1674-7860.2021.23.018.许钰波, 李月伟, 李成玉. 中医气针疗法配合药物治疗肝性脊髓病终末期多并发症1例[J]. 中医临床研究, 2021, 13( 23): 56- 58. DOI: 10.3969/j.issn.1674-7860.2021.23.018. [18] Smith M L, Wade J B, Wolstenholme J, et al. Gut microbiome-brain-cirrhosis axis[J]. Hepatology, 2024, 80( 2): 465- 485. DOI: 10.1097/HEP.0000000000000344. -
本文二维码
计量
- 文章访问数: 5
- HTML全文浏览量: 1
- PDF下载量: 0
- 被引次数: 0

PDF下载 ( 638 KB)
下载: 