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艾地苯醌治疗Friedreich共济失调和苯醌缺乏症 被引量:3

Idebenone Treatment into Patients with Friedreich Ataxia and Quinone Deficience
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摘要 艾地苯醌的苯并苯醌环路能进行可逆性的还原/氧化反应,影响线粒体中的电子链传导。早期临床研究证明,艾地苯醌能预防和改善Friedreich共济失调患者的心肌病。近年的研究证实大剂量和长期应用艾地苯醌治疗Friedreich共济失调不但能改善患者心肌病,而且可改善或稳定神经功能的紊乱。尤其在儿童患者有长时间改善神经功能的作用。艾地苯醌可治疗线粒体脑肌病。 The benzoquinone ring of idebenone can undergo reversible redox reactions, which influence the electron balance in mitochondria. Early clinical studies of idebenone at a dose of 5 mg·kg-1·d-1 mainly demonstrated the efficacy of idebenone on Friedreich ataxia(FRDA)-associated cardiomyopathy. More recent trials have shown that higher doses of idebenone and longer time of idebenone treatment not only could improve cardiomyopathy, but improve neurological function in the patients with FRDA. Especially the stabilizing function of idebenone was obvious for a long time in paediatric patients. Meanwhile, idebenone could improve cerebral mitochondrial metabolism in the patients with mitochondrial encephalopathy.
出处 《中国临床神经科学》 2010年第6期631-636,652,共7页 Chinese Journal of Clinical Neurosciences
关键词 艾地苯醌 FRIEDREICH共济失调 线粒体脑病 苯醌缺乏症 idebenone Friedreich ataxia mitochondrial encephalopathy quinone deficience
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参考文献19

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