肝豆状核变性合并肝性脊髓病1例报道
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摘要
肝豆状核变性作为一种罕见的神经系统疾病,其肝脏损害最为显著,肝功能衰竭所致的代谢紊乱又常导致其他相关并发症。本文报道1例肝豆状核变性合并肝性脊髓病患者在原发病基础上出现脊髓病变的相关症状。本例患者于2013年确诊为肝豆状核变性,后多次入住安徽中医药大学第一附属医院行排铜保肝治疗,2018年4月患者开始出现四肢远端麻木、力弱,进行性加重,半年内发展至不能行走。目前诊断为肝豆状核变性合并肝性脊髓病,治疗上予以营养神经、保肝排铜等综合治疗。肝豆状核变性和肝性脊髓病都与肝脏损害关系密切,合并报道尚不多见,为探究兼病时的诊断要点及治疗的相关性,特此报道。
As a rare neurological disease, wilson disease has the most significant liver damage, and the metabolic disorder caused by liver failure often leads to other related complications. This paper reports a case of hepatolenticular degeneration with hepatic myelopathy. This patient was diagnosed with hepatolenticular degeneration in 2013, and was admitted to the First Affiliated Hospital of Anhui University of Traditional Chinese Medicine for several times for the treatment of liver protection with copper discharge. In April 2018, the patient began to suffer from distal limb numbness and weakness, with progressive aggravation, and developed to be unable to walk within half a year. At present, hepatolenticular degeneration combined with hepatic myelopathy was diagnosed, and comprehensive treatment including nerve nutrition, liver protection and copper drainage was given. Both hepatolenticular degeneration and hepatic myelopathy are closely related to liver damage, and combined reports are rare. To explore the diagnosis and treatment of concurrent diseases, this report is hereby reported.
As a rare neurological disease, wilson disease has the most significant liver damage, and the metabolic disorder caused by liver failure often leads to other related complications. This paper reports a case of hepatolenticular degeneration with hepatic myelopathy. This patient was diagnosed with hepatolenticular degeneration in 2013, and was admitted to the First Affiliated Hospital of Anhui University of Traditional Chinese Medicine for several times for the treatment of liver protection with copper discharge. In April 2018, the patient began to suffer from distal limb numbness and weakness, with progressive aggravation, and developed to be unable to walk within half a year. At present, hepatolenticular degeneration combined with hepatic myelopathy was diagnosed, and comprehensive treatment including nerve nutrition, liver protection and copper drainage was given. Both hepatolenticular degeneration and hepatic myelopathy are closely related to liver damage, and combined reports are rare. To explore the diagnosis and treatment of concurrent diseases, this report is hereby reported.
引文
[1]李慎杰,张苓,钟萍,等.以急性肝衰竭和溶血为特征的肝豆状核变性1例[J].重庆医学,2016,45(10):1439-1440.
[2]李春岩,王桂荣,刘瑞春,等.感觉性共济失调型格林-巴利综合征3例临床与电生理[J].脑与神经疾病杂志,2001,9(1):11-12.
[3]格林-巴利综合征及亚急性联合变性文献重新分析—兼与李春岩院士等商榷[J].世界最新医学信息文摘,2018,18(98):118-120.
[4]Van den Bergh PY,Hadden RD,Bouche P,et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy:report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society-first revision[J].Eur J Neurol,2010,17(3):356-363.
[5]李鹏鹏,赵斌,赵晓峰.脊髓亚急性联合变性临床研究进展[J].中华临床医师杂志,2016,10(1):121-123.
[6]尹变利,任向阳,马聪敏.肝性脊髓病临床诊治分析[J].中国实用神经疾病杂志,2013,16(21):38-39.
[7]李祥,杨文明.肝豆状核变性基因诊断与治疗进展[J].中国实用神经疾病杂志,2012,15(3):90-93.
[8]张晓莉,徐丹,贾天明,等.无肝功能损害的肝豆状核变性1例[J].中国儿童保健杂志,2016,24(1):111-112.
[9]李翔,蔡洁,许钰雯,等.肝豆状核变性合并肝细胞癌1例报告[J].临床肝胆病杂志,2018,34(9):1967-1971.
[10]尤志菲,王晓平.肝豆状核变性睡眠障碍初步研究[J].中国现代神经疾病杂志,2016,16(5):280-283.
[11]蔡勇,莫樱,蒋小云,等.表现为肾病综合征的儿童肝豆状核变性[J].临床误诊误治,2016,29(9):46-48.
[12]靳培娜,禚志红,陈晓昕,等.以双下肢水肿肌无力为首发症状肝豆状核变性1例[J].中国实用儿科杂志,2014,29(10):794-795.
[13]王共强,王伟,薛本春,等.Wilson病中医证候文献分析[J].安徽中医学院学报,2011,30(4):5-8.
[14]杨文明,韩辉,鲍远程,等.中医对肝豆状核变性病因病机及辨证论治的探索[J].北京中医药大学学报,2012,19(4):6-8.
[15]汪美霞,汪长春.杨文明从痰瘀论治肝豆状核变性[J].中医药临床杂志,2017,29(7):983-985.
[16]Su AP,Cao SS,Le Tian B,et al. Effect of transjugular intrahepatic portosystemic shunt on glycometabolism in cirrhosis patients[J]. Clin Res Hepatol Gastroenterol,2012,36(1):53-59.
[17]郭丰昌,付兴,郑瑞琦,等.乙型肝炎后肝硬化并发肝性脊髓病1例报告[J].临床肝胆病杂志,2017,33(9):1178-1780.
[18]潘宜鹏,范宁,李明.肝性脊髓病研究现状[J].中华灾害救援医学,2014,2(12):710-712.
[19]杨阳,杨龙.肝性脊髓病研究进展[J].临床肝胆病杂志,2015,31(12):2097-2099.
[20]李惠珍,周小平,杨生义,等.肝移植治疗肝性脊髓病1例远期疗效观察[J].实用肝脏病杂志,2012,15(6):584.
[21]毛家玺,邹游,郭闻渊.肝移植治疗肝豆状核变性的效果观察[J].临床肝胆病杂志,2017,33(10):155-158.
[22]KhanAA,Shaik MV,Parveen N,et al. Human fetal liverderived stem cell transplantation as supportive modality in the management of end-stage decompensated liver cirrhosis[J]. Celll Transplant,2010,47(1):1.
[2]李春岩,王桂荣,刘瑞春,等.感觉性共济失调型格林-巴利综合征3例临床与电生理[J].脑与神经疾病杂志,2001,9(1):11-12.
[3]格林-巴利综合征及亚急性联合变性文献重新分析—兼与李春岩院士等商榷[J].世界最新医学信息文摘,2018,18(98):118-120.
[4]Van den Bergh PY,Hadden RD,Bouche P,et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy:report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society-first revision[J].Eur J Neurol,2010,17(3):356-363.
[5]李鹏鹏,赵斌,赵晓峰.脊髓亚急性联合变性临床研究进展[J].中华临床医师杂志,2016,10(1):121-123.
[6]尹变利,任向阳,马聪敏.肝性脊髓病临床诊治分析[J].中国实用神经疾病杂志,2013,16(21):38-39.
[7]李祥,杨文明.肝豆状核变性基因诊断与治疗进展[J].中国实用神经疾病杂志,2012,15(3):90-93.
[8]张晓莉,徐丹,贾天明,等.无肝功能损害的肝豆状核变性1例[J].中国儿童保健杂志,2016,24(1):111-112.
[9]李翔,蔡洁,许钰雯,等.肝豆状核变性合并肝细胞癌1例报告[J].临床肝胆病杂志,2018,34(9):1967-1971.
[10]尤志菲,王晓平.肝豆状核变性睡眠障碍初步研究[J].中国现代神经疾病杂志,2016,16(5):280-283.
[11]蔡勇,莫樱,蒋小云,等.表现为肾病综合征的儿童肝豆状核变性[J].临床误诊误治,2016,29(9):46-48.
[12]靳培娜,禚志红,陈晓昕,等.以双下肢水肿肌无力为首发症状肝豆状核变性1例[J].中国实用儿科杂志,2014,29(10):794-795.
[13]王共强,王伟,薛本春,等.Wilson病中医证候文献分析[J].安徽中医学院学报,2011,30(4):5-8.
[14]杨文明,韩辉,鲍远程,等.中医对肝豆状核变性病因病机及辨证论治的探索[J].北京中医药大学学报,2012,19(4):6-8.
[15]汪美霞,汪长春.杨文明从痰瘀论治肝豆状核变性[J].中医药临床杂志,2017,29(7):983-985.
[16]Su AP,Cao SS,Le Tian B,et al. Effect of transjugular intrahepatic portosystemic shunt on glycometabolism in cirrhosis patients[J]. Clin Res Hepatol Gastroenterol,2012,36(1):53-59.
[17]郭丰昌,付兴,郑瑞琦,等.乙型肝炎后肝硬化并发肝性脊髓病1例报告[J].临床肝胆病杂志,2017,33(9):1178-1780.
[18]潘宜鹏,范宁,李明.肝性脊髓病研究现状[J].中华灾害救援医学,2014,2(12):710-712.
[19]杨阳,杨龙.肝性脊髓病研究进展[J].临床肝胆病杂志,2015,31(12):2097-2099.
[20]李惠珍,周小平,杨生义,等.肝移植治疗肝性脊髓病1例远期疗效观察[J].实用肝脏病杂志,2012,15(6):584.
[21]毛家玺,邹游,郭闻渊.肝移植治疗肝豆状核变性的效果观察[J].临床肝胆病杂志,2017,33(10):155-158.
[22]KhanAA,Shaik MV,Parveen N,et al. Human fetal liverderived stem cell transplantation as supportive modality in the management of end-stage decompensated liver cirrhosis[J]. Celll Transplant,2010,47(1):1.