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以肝病为首发表现的成人肝豆状核变性1例报告

程然 李怡婧 方舒 徐小元 万华

引用本文:
Citation:

以肝病为首发表现的成人肝豆状核变性1例报告

DOI: 10.3969/j.issn.1001-5256.2018.03.035
详细信息
  • 中图分类号: R742.4

A case of Wilson′s disease in adults with liver disease as the initial manifestation

  • 摘要:

    <正>肝豆状核变性又名Wilson病,简称WD,是一种常染色体隐性遗传的铜代谢障碍疾病[1]。本病好发于青少年,中老年发病相对少见。肝病与神经精神症状是WD最常见的临床表现,一般18岁以下者的首发症状以肝损伤居多,而成年人则以锥体外系症状多见,以肝病为首发症状的WD较为少见[2]。本院近期收治了1例50岁以肝病起病的WD患者,以此完善有关该病的病例报告并进行相关文献复习。

     

  • [1]Parkinson's Disease and Movement Disorders Study Group.Neurology Branch of Chinese Medical Association.Guidelines for the diagnosis and treatment of hepatolenticular degeneration[J].Chin J Neurol, 2008, 41 (8) :566-569. (in Chinese) 中华医学会神经病学分会帕金森病及运动障碍学组.肝豆状核变性的诊断与治疗指南[J].中华神经科杂志, 2008, 41 (8) :566-569.
    [2]SHAH D.Wilson's disease:hepatic manifestations[J].Dis Mon, 2014, 60 (9) :465-474.
    [3]SAMAR H, HARRIS VKN, SAMEER S.Wilson's disease[J].Lancet, 2007, 369 (9565) :902-903.
    [4]ROBERTS EA, SCHILSKY ML.A practice guideline on Wilson disease (vol 37, pg 1475, 2003) [J].Hepatology, 2003, 38 (2) :536.
    [5]European Association for Study of Liver.EASL Clinical Practice Guidelines:Wilson's disease[J].J Hepatol, 2012, 56 (3) :671-685.
    [6]ROBERTS EA, SCHILSKY ML.Diagnosis and treatment of Wilson disease:an update[J].Hepatology, 2008, 47 (6) :2089-2111.
    [7]MEDICI V, TREVISAN CP, D'INCAR, et al.Diagnosis and management of Wilson's disease:results of a single center experience[J].J Clin Gastroenterol, 2006, 40 (10) :936-941.
    [8]DUNN LL, ANNABLE WL, KLIEGMAN RM.Pigmented corneal rings in neonates with liver disease[J].J Pediatr, 1987, 110 (5) :771-776.
    [9]LIANG N, WANG FF, MO DF.Analysis of ultrasound diagnosis in hepatolenticular degeneration[J].Chin Ultrasound Med, 2009, 25 (11) :1057-1059. (in Chinese) 梁娜, 王付发, 莫定芳.肝豆状核变性的超声诊断价值探讨[J].中国超声医学杂志, 2009, 25 (11) :11057-1059.
    [10]YUAN XY, XIONG H, GAN HT.Analysis of clinical misdiagnosis for HLD combined liver damage[J].J Chengdu Med Coll, 2015, 10 (4) :452-454. (in Chinese) 袁兴娅, 熊海, 甘华田.肝豆状核变性合并肝脏损害的临床误诊分析[J], 成都医学院学报, 2015, 10 (4) :452-454.
    [11]ZHANG DF, WANG YS, TENG JF.Clinical research about liver function of hepatolenticular degeneration combined liver fibrosis by transplantation of bone marrow stem cells[J].Chin J Immunol, 2016, 32 (2) :193-196, 200. (in Chinese) 张东锋, 王煜姝, 滕军放.骨髓间充质干细胞对肝豆状核变性合并肝纤维化肝功能的临床研究[J].中国免疫学杂志, 2016, 32 (2) :193-196, 200.
    [12]JIANG B, WANG QY, XIE YC, et al.The progress of treatment of hepatolenticular degeneration[J].Shandong Med J, 2010, 50 (4) :112-114. (in Chinese) 姜彬, 王倩怡, 谢琰臣, 等.肝豆状核变性的治疗进展[J].山东医药, 2010, 50 (4) :112-114.
    [13]BREWER GJ, JOHNSON V, DICK RD, et al.Treatment of Wilson disease with ammonium tetrathiomolybdate.II.Initial therapy in 33neurologically affected patients and follow-up with zinc therapy[J].Arch Neurol, 1996, 53 (10) :1017-1025.
    [14]SINHA S, TALY AB.Withdrawal of penicillamine from zinc sulphate-penicillamine maintenance therapy in Wilson's disease:promising, safe and cheap[J].J Neurol Sci, 2008, 264 (1) :129-132.
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  • 出版日期:  2018-03-20
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