Abstract
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.
Highlights
Carnitine, with a molecular weight of 161 Da, is a water-soluble quaternary amine
Carnitine deficiency is associated with several clinical disorders, such as erythropoiesis-stimulating agent (ESA)-resistant anemia, muscle weakness, myopathy, and intradialytic muscle cramps and hypotension
Free carnitine levels are mainly decreased by its removal during hemodialysis, whereas the accumulation of acylcarnitine and an aberrantly elevated plasma acylcarnitine to free carnitine ratio are due to deficient renal clearance and β-oxidation failure [1,2]
Summary
With a molecular weight of 161 Da, is a water-soluble quaternary amine It is derived from lysine and methionine, which are two essential amino acids. Its primary role is in facilitating the transport of long-chain fatty acids to the mitochondrial matrix. These substrates are delivered for β-oxidation and the subsequent production of energy. Carnitine deficiency is associated with several clinical disorders, such as erythropoiesis-stimulating agent (ESA)-resistant anemia, muscle weakness, myopathy, and intradialytic muscle cramps and hypotension. We describe the profile of carnitine metabolism and the effects of carnitine treatment on the metabolism and function of dialysis patients. Current findings related to the carnitine treatment of patients undergoing dialysis therapy, its impact on cardiac function, ESA-resistant anemia, muscle symptoms, and malnutrition
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