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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 被引量:6

A case of Wilson's disease in adults with liver disease as the initial manifestation
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摘要 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。
作者 程然 李怡婧 方舒 徐小元 万华 CHENG Ran, LI Yijing, FANG Shu, et al.(Department of lnfectious Diseases, Peking University First Hospital, Beijing 100034, Chin)
出处 《临床肝胆病杂志》 CAS 北大核心 2018年第3期607-609,共3页 Journal of Clinical Hepatology
关键词 肝豆状核变性 肝疾病 成年人 病例报告 hepatolenticular degeneration liver diseases adult ease reports
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  • 1梁秀龄,陈曦,李洵桦,黄丽,石铸,汤其强.肝豆状核变性临床若干问题[J].中华神经科杂志,2005,38(1):57-59. 被引量:54
  • 2王金萍,王兴族,王宁新,许红强,黄猛,刘卫勇,陈晓艺.肝肾B超检查在肝豆状核变性诊断中的价值[J].安徽中医学院学报,2006,25(3):56-57. 被引量:11
  • 3Ala A, Walker AP, Ashkan K, et al. Wilson's disease. Lancet, 2007, 369:397-405.
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  • 6Wilson DC ,Phillips M J, Cox DW, et al. Severe hepatic Wilson's disease in preschool-aged children. J Pediatr, 2000, 137: 719- 722.
  • 7Ala A, Borjigin J, Rochwarger A, et al. Wilson disease in septuagenarian siblings : Raising the bar for diagnosis. Hepatology, 2005, 41:668-670.
  • 8Ferenci P, Caca K, Loudianos G, et al. Diagnosis and phenotypic classification of Wilson disease. Liver Int, 2003,23 : 139-142.
  • 9Sinha S ,Taly AB. Withdrawal of peniciUamine from zinc sulphatepenicillamine maintance therapy in Wilson's disease : promising, safe and cheap. J Neurol Sci, 2008,264: 129-132.
  • 10Brewer GJ, Hedera P, Khin KJ, et al. Treatment of Wilson disease with ammonium tetrathiomolybdate: Ⅲ. Initial therapy in a total of 55 neurologically affected patients and follow-up with zinc therapy. Arch Neurol, 2003,60: 379-385.

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