生酮饮食治疗线粒体脑肌病(MELAS)的临床疗效观察(2)
總结，从我们的经验和国外的文献报道，生酮饮食可以作为治疗线粒体病的辅助治疗的方法，是安全的，可以改善MELAS患儿的临床症状，尤其是降低血乳酸水平及减少临床的抽搐发作，但影像学的研究还需要进一步随访观察。这里必须强调的是我们目前只有两例患者，虽然在治疗中未发现明显的副作用，但病例数太少，观察时间不足为其缺点。
参考文献：
[1]Avula S，Parikh S，Demarest S，et al.Treatment of Mitochondrial Disorders[J].Current Treatment Options in Neurology，2014，16(6)：1-20.
[2]El-Hattab A W，Adesina A M，Jones J，et al.MELAS syndrome：Clinical manifestations，pathogenesis，and treatment options[J].Molecular Genetics &Metabolism，2015，116(1-2)：4-12.
[3]Steriade C，Andrade D M，Faghfoury H，et al.Mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) may respond to adjunctive ketogenic diet[J].Pediatric Neurology，2014，50(5)：498-502.
[4]Lee E，Kang H C，Kim H D.ERRATUM：Ketogenic Diet for Children with Epilepsy：A Practical Meal Plan in a Hospital[J].Clin Nutr Res， 2016，5(2)：141.
[5]Eun S H，Kang H C，Kim D W，et al.Ketogenic diet for treatment of infantile spasms[J].Brain&Development，2006，28(9)：566-571.
[6]Wexler I D，Hemalatha S G，Mcconnell J，et al.Outcome of pyruvate dehydrogenase deficiency treated with ketogenic diets.Studies in patients with identical mutations[J].Neurology，1997，49(6)：1655-1661.
[7]Kang H C，Lee Y M，Kim H D，et al.Safe and effective use of the ketogenic diet in children with epilepsy and mitochondrial respiratory chain complex defects[J].Epilepsia，2007，48(1)：82-8., http://www.100md.com(王四美 张元凤 陆燕芬 奚佳铭 王春梅 王曼 陈育才)
参考文献：
[1]Avula S，Parikh S，Demarest S，et al.Treatment of Mitochondrial Disorders[J].Current Treatment Options in Neurology，2014，16(6)：1-20.
[2]El-Hattab A W，Adesina A M，Jones J，et al.MELAS syndrome：Clinical manifestations，pathogenesis，and treatment options[J].Molecular Genetics &Metabolism，2015，116(1-2)：4-12.
[3]Steriade C，Andrade D M，Faghfoury H，et al.Mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) may respond to adjunctive ketogenic diet[J].Pediatric Neurology，2014，50(5)：498-502.
[4]Lee E，Kang H C，Kim H D.ERRATUM：Ketogenic Diet for Children with Epilepsy：A Practical Meal Plan in a Hospital[J].Clin Nutr Res， 2016，5(2)：141.
[5]Eun S H，Kang H C，Kim D W，et al.Ketogenic diet for treatment of infantile spasms[J].Brain&Development，2006，28(9)：566-571.
[6]Wexler I D，Hemalatha S G，Mcconnell J，et al.Outcome of pyruvate dehydrogenase deficiency treated with ketogenic diets.Studies in patients with identical mutations[J].Neurology，1997，49(6)：1655-1661.
[7]Kang H C，Lee Y M，Kim H D，et al.Safe and effective use of the ketogenic diet in children with epilepsy and mitochondrial respiratory chain complex defects[J].Epilepsia，2007，48(1)：82-8., http://www.100md.com(王四美 张元凤 陆燕芬 奚佳铭 王春梅 王曼 陈育才)