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Wilson氏病 被引量:1

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摘要 Wilson氏病(WD)和非印第安儿童期肝硬化(特发性铜中毒,或简称ICT)的致病因素是铜在肝脏内的过量蓄积。第13号染色体的常染色体隐性基因的一对等位基因是引起Wilson氏病的铜蓄积的必要的和充分的条件,减少饮食铜摄入并不能阻止Wilson氏病的发展。
作者 张林 石蕾
机构地区 西安医科大学
出处 《国外医学(医学地理分册)》 CAS 1997年第3期118-120,共3页 Foreign Medical Sciences:Section of Medgeography
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  • 1吴志英,王柠.肝豆状核变性的分子生物学研究进展[J].国外医学(神经病学.神经外科学分册),1996,23(5):231-233. 被引量:3
  • 2史玉泉.实用神经病学(第2版)[M].上海:上海科学技术出版社,1994.736.
  • 3Bull PC,Thomas GR, Rommens JM,et al. The Wilson's disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene. Nat Genet, 1993, 5:327-337.
  • 4Martin WRW, Palmer MR, Patlak CS, et al. Nigrostriatal function in humans studied with positron emission tomography. Ann Neurol, 1989, 26(4): 535-542.
  • 5Oertet WH, Tatsch K, Schwarz J, et al. Decrease of D2,receptors indicated by ^123I-Iodobenzamide Single-Photon emission computed tomography relates to neurological deficit in treated Wilson's disease.Ann Neurol, 1992,32(6):743 -748.
  • 6Oder W,Brucke T,Kollegger H,et al.Dopamine D2 receptor binding is reduced in Wilson's disease:correlation of neurological deficits with striatal ^123I-iodobenzamide binding.J Neurol Transm,1996,103:1093-1103.
  • 7Jeon B, Kim JM, Jeong JM, et al. Dopamine transporter imaging with ^123I-β-CIT demonstrates presynaptic nigrostriatal dopaminergic damage in Wilson's disease. J Neurol Neurosurg Psychiatry, 1998,65:60-64.
  • 8Westermark K, Tedroff J, Thuomas KA, et al. Neurological Wilson's disease studied with magnetic resonance imaging and with positron emission tomography using dopaminergic markers. Mov Disord, 1995,10(5):596-603.
  • 9Schwarz J, Antonini A, Kraft E, et al. Treatment with D-peni-cillamine improves dopamine D2-receptor binding and T2-sig-nal intensity in de novo Wilson's disease. Neurol. 1994, 44(6):1079-1082.
  • 10Schlaug G, Hefler H, Nebeling B, et al. Dopamine D2 receptor binding and cerebral glucose metabolism recover after D- peni-cillamine-therapy in Wilson' s disease. J Neurol, 1994,241(10):577-584.

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