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肝移植治疗肝豆状核变性七例 被引量:2

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摘要 肝豆状核变性又名Wilson病(WD),患者第13号染色体铜转运P型ATPase基因突变导致肝脏铜蓝蛋白合成障碍,体内过量的铜沉积于肝脏、脑、角膜和肾脏等部位,出现肝脏、神经系统、肾功能异常和角膜环等临床表现。如能早期行排铜治疗和低铜膳食,可获长期缓解,但对晚期患者,肝移植是目前最有效的治疗方法。
出处 《中华消化杂志》 CAS CSCD 北大核心 2007年第8期551-551,共1页 Chinese Journal of Digestion
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参考文献7

  • 1.肝豆状核变性[M].姚光弼.临床肝脏病学[C].上海:上海科学技术出版社,2004.590.
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共引文献2

同被引文献23

  • 1邵卓,杨广顺,杨宁,张海斌.肝移植治疗Wilson病5例分析[J].中国临床医学,2007,14(3):339-341. 被引量:2
  • 2Ala A,Walker AP,Ashkan K,et al.Wilson's disease[J].Lancet,2007,369(9559):397-408.
  • 3Hoogenraad TU.Paradigm shift in trentment of wilson's disease:Zinc therapy now treatment of choice[J].Brain Develop,2006,28(3):141-146.
  • 4BrewerGJ,AskariF,LorinczMT,et al.Treatme nt of Wilson disease with ammonium tetrathiomolybdate[J].Arch Neurol,2006,63(4):521-527.
  • 5阴瑞红,曲云东,林森.移植治疗肝豆状核变性的临床和研究进展[J].国际消化病杂志,2007,27(4):275-277. 被引量:4
  • 6Dhawan A,Ferenci P,Geubel A. Genes and metals:a deadly combination[J].{H}Acta Gastroenterologica Belgica,2005,(01):26-32.
  • 7Ala A,Walker AP,Ashkan K. Wilson's disease[J].{H}LANCET,2007,(9559):397-408.
  • 8Geissler I,Heinemann K,Rohm S. Liver transplantation for hepatic and neurological Wilson's disease[J].{H}TRANSPLANTATION PROCEEDINGS,2003,(04):1445-1446.
  • 9Sutclif e RP,Maguire DD,Muiesan P. Liver transplantation for Wilson's disease:long-term results and quality-of-life assessment[J].{H}TRANSPLANTATION,2003,(07):1003-1006.
  • 10Schilsky ML. Wilson disease:genetic basis of copper toxicity and natural history[J].{H}Seminars in Liver Disease,1996,(01):83-95.

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