Evans O B
Department of Pediatrics, University of Mississippi Medical Center, Jackson 39216.
Pediatr Neurol. 1985 Nov-Dec;1(6):325-8. doi: 10.1016/0887-8994(85)90065-7.
Lactic acidosis accompanies many acquired and inherited metabolic diseases. The role of lactic acid in anaerobic glycolysis, gluconeogenesis, and acid-base balance is key to the understanding of these disorders. Because lactic acid can be formed only from pyruvic acid, disorders which increase pyruvate production, inhibit its catabolism, or shift the equilibrium toward lactic acid formation cause lactic acidosis. Lactic acidosis results from systemic diseases and toxins which produce tissue hypoxia or mitochondrial injury. Abnormalities of other metabolites such as glucose, pyruvate, amino acids, and organic acids may provide clues to inborn metabolic errors. Treatment must first be directed toward removing precipitating causes of the acquired disorders and then toward correcting the acidosis and other metabolic complications such as hypoglycemia. Some of the inborn errors respond to specific therapies.
乳酸性酸中毒伴随许多获得性和遗传性代谢疾病。乳酸在无氧糖酵解、糖异生和酸碱平衡中的作用是理解这些疾病的关键。由于乳酸仅能由丙酮酸形成，增加丙酮酸生成、抑制其分解代谢或使平衡向乳酸生成方向移动的疾病会导致乳酸性酸中毒。乳酸性酸中毒由导致组织缺氧或线粒体损伤的全身性疾病和毒素引起。其他代谢物如葡萄糖、丙酮酸、氨基酸和有机酸的异常可能为先天性代谢缺陷提供线索。治疗必须首先针对消除获得性疾病的诱发原因，然后纠正酸中毒和其他代谢并发症，如低血糖。一些先天性缺陷对特定疗法有反应。
