PDF(Pubmed)
Abstract：
MELAS syndrome, characterized by mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes, represents a devastating mitochondrial disease, with the stroke-like episodes being its primary manifestation. Arginine supplementation has been used and recommended as a treatment for these acute attacks; however, insufficient evidence exists to support this treatment for MELAS. The mechanisms underlying the effect of arginine on MELAS pathophysiology remain unclear, although it is hypothesized that arginine could increase nitric oxide availability and, consequently, enhance blood supply to the brain. A more comprehensive understanding of these mechanisms is necessary to improve treatment strategies, such as dose and regimen adjustments; identify which patients could benefit the most; and establish potential markers for follow-up. This review aims to analyze the existing evidence concerning the mechanisms through which arginine supplementation impacts MELAS pathophysiology and provide the current scenario and perspectives for future investigations.
摘要：
MELAS综合征,以线粒体肌病为特征，脑病,乳酸性酸中毒和中风样发作，代表了一种毁灭性的线粒体疾病，中风样发作是其主要表现。精氨酸补充剂已被使用，并被推荐作为这些急性发作的治疗方法；然而，没有足够的证据支持MELAS的这种治疗。精氨酸对MELAS病理生理学影响的潜在机制尚不清楚。尽管假设精氨酸可以增加一氧化氮的利用率，因此,增强大脑的血液供应。有必要更全面地了解这些机制，以改进治疗策略，如剂量和方案调整;确定哪些患者受益最大;并建立潜在的随访标志物。这篇综述旨在分析有关补充精氨酸影响MELAS病理生理学的机制的现有证据，并为未来的研究提供当前的情景和观点。
