驱铜药治疗后肝豆状核变性神经系统症状恶化机制探讨
杨文明,李祥
摘要(Abstract):
<正>肝豆状核变性(HLD)又称Wilson病,为一种遗传性铜代谢障碍所致的肝硬化和基底节为主的脑部变性疾病。研究证实,WD患者因ATP7B基因突变引起铜代谢障碍,使得游离的铜离子在肝、豆状核、角膜、肾脏等组织器官缓慢蓄
关键词(KeyWords): 肝豆状核变性;驱铜剂;神经症状加重;病理机制
基金项目(Foundation): 国家自然科学基金(30973748);; 国家中医药管理局科研基金(06-07LP23)
作者(Author): 杨文明,李祥
参考文献(References):
- [1]Ala A,Walker AP,Ashkan K,et al.Wilson’s disease[J].Lancet,2007,369:397-408.
- [2]贾建平主编.神经病学[M].北京:人民卫生出版社,2004:283.
- [3]Gollan JL,Sonay Hussein,Hoffbrand AV,et al.Red cellaplasia following prolonged D-penicillamine therapy[J].J ClinPathol,1976,29(2):135-139.
- [4]任明山,杨任民,张波,等.二巯丙磺钠与二巯丁二酸或青霉胺治疗肝豆状核变性的比较[J].中国新药与临床杂志,1998,17(1):23-25.
- [5]Brewer GJ,Terry CA,Aisen AM,et al.Worsening of neuro-logic syndrome in patients with Wilson's disease with initialpenicillamine therapy[J].Arch Neurol,1987,44(5):490-493.
- [6]Walshe JM,Yealland M.Chelation treatment of neurologicalWilson′s disease[J].Q J Med,1993,86(3):197-204.
- [7]Glass JD,Reich SG,DeLong MR.Wilson's disease,develop-ment of neurological disease after beginning penicillamine ther-apy[J].Arch Neurol,1990,47(5):595-596.
- [8]洪铭范,杨任民.肝豆状核变性患者肌肉铜、锌、铁、镁含量及二琉基丁二酸钠疗效探讨[J].临床神经病学杂志,1995,8(6):339-341.
- [9]梁秀龄.肝豆状核变性当前国外研究热点[J].国外医学.内科学分册,1993,20(3):110-113.
- [10]杨任民.肝豆状核变性的病因及治疗进展[J].中国临床神经学,2003,11(4):417-419.
- [11]Owen CA,JR.Randall RV,Goldstein NP.Effect of dietaryD-penicillamine on metabolism of copper in the rat[J].Am JPhysiol,1975,228(1):88-91.
- [12]Stuerenburg HJ.CSF copper concentrations,blood-brain bar-rier function,and coeruloplasmin synthesis during the treat-ment of Wilson's disease[J].J Neural Transm,2000,107(3):321-329.
- [13]李乃忠.青霉胺治疗肝豆状核变性的远期疗效及其不良反应[J].中国临床药学杂志,1996,5(2):45-47.
- [14]Brewer GJ,Hedera P,Kluin KJ,et al.Treatment of Wilsondisease with ammonium tetrathiomolybdateⅢ.Initial therapyin a total of 55 neurologically affected patients and follow-upwith Zinc therapy[J].Arch Neurol,2003,60(3):379-385.
- [15]杨永范.正常铜代谢与HLD的发病机制[J].中国神经精神疾病杂志,1984,10(1):64-66.