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[肌萎缩侧索硬化症的病因性药物治疗]

[Etiological drug therapy for amyotrophic lateral sclerosis].

作者信息

Cintas P

机构信息

Centre SLA, Service de Neurologie et d'Explorations Fonctionnelles Neurologiques, Hôpital Rangueil, CHU Toulouse.

出版信息

Rev Neurol (Paris). 2006 Jun;162 Spec No 2:4S220-4S227.

Abstract

Progress in the last decades has greatly strengthened our understanding of the etiopathogenic mechanisms of amyotrophic lateral sclerosis. Such progress has lead to an evaluation of several compounds affecting excitotoxicity, oxidative stress, apoptosis, growth factors and inflammation. Riluzole has clearly proven efficiency on mortality with a 35 p.cent reduction of death or tracheotomy at 18 months. While the mechanism of action of this compound remains to be fully elucidated, there is evidence in favor of a neuroprotective effect. Other compounds have been tried in ALS. To date, results have been disappointing with worsening even being noted in some cases. These negative studies offer some insight into the pathophysiological mechanism regulating the disease, raising pertinent questions concerning the transposition of animals models to humans and about interractions between these different compounds and riluzole.

摘要

过去几十年的进展极大地增强了我们对肌萎缩侧索硬化症发病机制的理解。这样的进展促使人们对几种影响兴奋性毒性、氧化应激、细胞凋亡、生长因子和炎症的化合物进行了评估。利鲁唑已明确证明对死亡率有效,在18个月时可使死亡或气管切开率降低35%。虽然该化合物的作用机制仍有待充分阐明,但有证据支持其神经保护作用。其他化合物也已在肌萎缩侧索硬化症中进行了试验。迄今为止,结果令人失望,甚至在某些情况下还出现了病情恶化。这些阴性研究为调节该疾病的病理生理机制提供了一些见解,提出了关于动物模型向人类转化以及这些不同化合物与利鲁唑之间相互作用的相关问题。

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