Division of Nephrology, Hypertension and Endocrinology, Department of Internal Medicine, Nihon University School of Medicine, 30-1 Oyaguchi Kami-cho, Itabashi-ku, Tokyo 173-8610, Japan.
Nutrients. 2021 Apr 7;13(4):1219. doi: 10.3390/nu13041219.
Carnitine is a naturally occurring amino acid derivative that is involved in the transport of long-chain fatty acids to the mitochondrial matrix. There, these substrates undergo β-oxidation, producing energy. The major sources of carnitine are dietary intake, although carnitine is also endogenously synthesized in the liver and kidney. However, in patients on dialysis, serum carnitine levels progressively fall due to restricted dietary intake and deprivation of endogenous synthesis in the kidney. Furthermore, serum-free carnitine is removed by hemodialysis treatment because the molecular weight of carnitine is small (161 Da) and its protein binding rates are very low. Therefore, the dialysis procedure is a major cause of carnitine deficiency in patients undergoing hemodialysis. This deficiency may contribute to several clinical disorders in such patients. Symptoms of dialysis-related carnitine deficiency include erythropoiesis-stimulating agent-resistant anemia, myopathy, muscle weakness, and intradialytic muscle cramps and hypotension. However, levocarnitine administration might replenish the free carnitine and help to increase carnitine levels in muscle. This article reviews the previous research into levocarnitine therapy in patients on maintenance dialysis for the treatment of renal anemia, cardiac dysfunction, dyslipidemia, and muscle and dialytic symptoms, and it examines the efficacy of the therapeutic approach and related issues.
肉碱是一种天然存在的氨基酸衍生物,参与长链脂肪酸向线粒体基质的转运。在那里,这些底物经历β-氧化,产生能量。肉碱的主要来源是饮食摄入,尽管肉碱也在肝脏和肾脏中内源性合成。然而,在接受透析的患者中,由于饮食摄入受限和肾脏内源性合成减少,血清肉碱水平逐渐下降。此外,由于肉碱的分子量较小(161Da)且其蛋白结合率非常低,因此无蛋白结合的肉碱通过血液透析治疗被清除。因此,透析过程是接受血液透析的患者肉碱缺乏的主要原因。这种缺乏可能导致这些患者出现几种临床疾病。与透析相关的肉碱缺乏的症状包括红细胞生成刺激剂抵抗性贫血、肌病、肌肉无力以及透析期间肌肉痉挛和低血压。然而,左卡尼汀的给药可能会补充游离肉碱并有助于增加肌肉中的肉碱水平。本文回顾了之前关于左卡尼汀治疗维持性透析患者的研究,用于治疗肾性贫血、心功能障碍、血脂异常、肌肉和透析症状,并检查了该治疗方法的疗效和相关问题。