Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Carnitine metabolism in renal disease and dialysis

Alan Wasserstein, MD
Section Editor
Steve J Schwab, MD
Deputy Editor
Alice M Sheridan, MD


Carnitine deficiency may be a significant problem in patients with kidney disease, particularly in those undergoing maintenance dialysis. A discussion of carnitine metabolism, as well as the role of carnitine supplementation in this patient population, is presented in this topic review.


Carnitine is an important intermediary in fat metabolism. It is required to shuttle long-chain fatty acids, in the form of acylcarnitines, into mitochondria for beta-oxidation; carnitine is, therefore, crucial for energy production in tissues dependent upon fatty acid oxidation, such as cardiac and skeletal muscle.

Reactions of carnitine with activated fatty acids (acyl CoA) are esterifications of the general form:

acyl CoA + carnitine -> acylcarnitine + CoASH

and are catalyzed by a family of carnitine acyltransferases.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Aug 07, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Hoppel C. The physiological role of carnitine. In: L-Carnitine and Its Role in Medicine: From Function to Therapy, Ferrari R, DiMauro S, Sherwood G (Eds), Academic Press, London 1992. p.5.
  2. Wanner C, Hörl WH. Carnitine abnormalities in patients with renal insufficiency. Pathophysiological and therapeutical aspects. Nephron 1988; 50:89.
  3. Ahmad S. Carnitine, kidney and renal dialysis. In: L-Carnitine and Its Role in Medicine: From Function to Therapy, Ferrari R, DiMauro S, Sherwood G (Eds), Academic Press, London 1992. p.381.
  4. Schreiber BD. Debate forum: levocarnitine therapy is rational and justified in selected dialysis patients. Blood Purif 2006; 24:128.
  5. Fouque D, Holt S, Guebre-Egziabher F, et al. Relationship between serum carnitine, acylcarnitines, and renal function in patients with chronic renal disease. J Ren Nutr 2006; 16:125.
  6. Golper TA, Ahmad S. L-carnitine administration to hemodialysis patients: has its time come? Semin Dial 1992; 5:94.
  7. Hothi DK, Geary DF, Fisher L, Chan CT. Short-term effects of nocturnal haemodialysis on carnitine metabolism. Nephrol Dial Transplant 2006; 21:2637.
  8. Golper TA, Wolfson M, Ahmad S, et al. Multicenter trial of L-carnitine in maintenance hemodialysis patients. I. Carnitine concentrations and lipid effects. Kidney Int 1990; 38:904.
  9. Ahmad S, Robertson HT, Golper TA, et al. Multicenter trial of L-carnitine in maintenance hemodialysis patients. II. Clinical and biochemical effects. Kidney Int 1990; 38:912.
  10. Evans AM, Faull RJ, Nation RL, et al. Impact of hemodialysis on endogenous plasma and muscle carnitine levels in patients with end-stage renal disease. Kidney Int 2004; 66:1527.
  11. Fornasini G, Upton RN, Evans AM. A pharmacokinetic model for L-carnitine in patients receiving haemodialysis. Br J Clin Pharmacol 2007; 64:335.
  12. Savica V, Santoro D, Mazzaglia G, et al. L-carnitine infusions may suppress serum C-reactive protein and improve nutritional status in maintenance hemodialysis patients. J Ren Nutr 2005; 15:225.
  13. Duranay M, Akay H, Yilmaz FM, et al. Effects of L-carnitine infusions on inflammatory and nutritional markers in haemodialysis patients. Nephrol Dial Transplant 2006; 21:3211.
  14. Biolo G, Stulle M, Bianco F, et al. Insulin action on glucose and protein metabolism during L-carnitine supplementation in maintenance haemodialysis patients. Nephrol Dial Transplant 2008; 23:991.
  15. Tabibi H, Hakeshzadeh F, Hedayati M, Malakoutian T. Effects of l-carnitine supplement on serum amyloid A and vascular inflammation markers in hemodialysis patients: a randomized controlled trial. J Ren Nutr 2011; 21:485.
  16. Fukami K, Yamagishi S, Sakai K, et al. Potential inhibitory effects of L-carnitine supplementation on tissue advanced glycation end products in patients with hemodialysis. Rejuvenation Res 2013; 16:460.
  17. Hurot JM, Cucherat M, Haugh M, Fouque D. Effects of L-carnitine supplementation in maintenance hemodialysis patients: a systematic review. J Am Soc Nephrol 2002; 13:708.
  18. Wanner C, Wieland H, Wäckerle B, et al. Ketogenic and antiketogenic effects of L-carnitine in hemodialysis patients. Kidney Int Suppl 1989; 27:S264.
  19. Elisaf M, Bairaktari E, Katopodis K, et al. Effect of L-carnitine supplementation on lipid parameters in hemodialysis patients. Am J Nephrol 1998; 18:416.
  20. Fagher B, Cederblad G, Eriksson M, et al. L-carnitine and haemodialysis: double blind study on muscle function and metabolism and peripheral nerve function. Scand J Clin Lab Invest 1985; 45:169.
  21. Siami G, Clinton ME, Mrak R, et al. Evaluation of the effect of intravenous L-carnitine therapy on function, structure and fatty acid metabolism of skeletal muscle in patients receiving chronic hemodialysis. Nephron 1991; 57:306.
  22. Thompson CH, Irish AB, Kemp GJ, et al. The effect of propionyl L-carnitine on skeletal muscle metabolism in renal failure. Clin Nephrol 1997; 47:372.
  23. Vaux EC, Taylor DJ, Altmann P, et al. Effects of carnitine supplementation on muscle metabolism by the use of magnetic resonance spectroscopy and near-infrared spectroscopy in end-stage renal disease. Nephron Clin Pract 2004; 97:c41.
  24. Lynch KE, Feldman HI, Berlin JA, et al. Effects of L-carnitine on dialysis-related hypotension and muscle cramps: a meta-analysis. Am J Kidney Dis 2008; 52:962.
  25. Mercadal L, Coudert M, Vassault A, et al. L-carnitine treatment in incident hemodialysis patients: the multicenter, randomized, double-blinded, placebo-controlled CARNIDIAL trial. Clin J Am Soc Nephrol 2012; 7:1836.
  26. Semeniuk J, Shalansky KF, Taylor N, et al. Evaluation of the effect of intravenous l-carnitine on quality of life in chronic hemodialysis patients. Clin Nephrol 2000; 54:470.
  27. Steiber AL, Davis AT, Spry L, et al. Carnitine treatment improved quality-of-life measure in a sample of Midwestern hemodialysis patients. JPEN J Parenter Enteral Nutr 2006; 30:10.
  28. Weinhandl ED, Rao M, Gilbertson DT, et al. Protective effect of intravenous levocarnitine on subsequent-month hospitalization among prevalent hemodialysis patients, 1998 to 2003. Am J Kidney Dis 2007; 50:803.
  29. Romagnoli GF, Naso A, Carraro G, Lidestri V. Beneficial effects of L-carnitine in dialysis patients with impaired left ventricular function: an observational study. Curr Med Res Opin 2002; 18:172.
  30. Signorelli SS, Fatuzzo P, Rapisarda F, et al. A randomised, controlled clinical trial evaluating changes in therapeutic efficacy and oxidative parameters after treatment with propionyl L-carnitine in patients with peripheral arterial disease requiring haemodialysis. Drugs Aging 2006; 23:263.
  31. Signorelli SS, Fatuzzo P, Rapisarda F, et al. Propionyl-L-carnitine therapy: effects on endothelin-1 and homocysteine levels in patients with peripheral arterial disease and end-stage renal disease. Kidney Blood Press Res 2006; 29:100.
  32. Iliceto S, Scrutinio D, Bruzzi P, et al. Effects of L-carnitine administration on left ventricular remodeling after acute anterior myocardial infarction: the L-Carnitine Ecocardiografia Digitalizzata Infarto Miocardico (CEDIM) Trial. J Am Coll Cardiol 1995; 26:380.
  33. Higuchi T, Abe M, Yamazaki T, et al. Levocarnitine Improves Cardiac Function in Hemodialysis Patients With Left Ventricular Hypertrophy: A Randomized Controlled Trial. Am J Kidney Dis 2016; 67:260.
  34. Wang Z, Klipfell E, Bennett BJ, et al. Gut flora metabolism of phosphatidylcholine promotes cardiovascular disease. Nature 2011; 472:57.
  35. Koeth RA, Wang Z, Levison BS, et al. Intestinal microbiota metabolism of L-carnitine, a nutrient in red meat, promotes atherosclerosis. Nat Med 2013; 19:576.
  36. Tang WH, Wang Z, Levison BS, et al. Intestinal microbial metabolism of phosphatidylcholine and cardiovascular risk. N Engl J Med 2013; 368:1575.
  37. Kim RB, Morse BL, Djurdjev O, et al. Advanced chronic kidney disease populations have elevated trimethylamine N-oxide levels associated with increased cardiovascular events. Kidney Int 2016; 89:1144.
  38. Consensus Group Statement. Role of L-carnitine in treating renal dialysis patients. Dial Transplant 1994; 23:177.
  39. Clinical practice guidelines for nutrition in chronic renal failure. K/DOQI, National Kidney Foundation. Am J Kidney Dis 2000; 35:S1.
  40. KDOQI, National Kidney Foundation. KDOQI Clinical Practice Guidelines and Clinical Practice Recommendations for Anemia in Chronic Kidney Disease. Am J Kidney Dis 2006; 47:S11.
  41. Eknoyan G, Latos DL, Lindberg J, National Kidney Foundation Carnitine Consensus Conference. Practice recommendations for the use of L-carnitine in dialysis-related carnitine disorder. National Kidney Foundation Carnitine Consensus Conference. Am J Kidney Dis 2003; 41:868.
  42. FDA summary of approval for carnitor injection in dialysis. NDA:20-182/s-006, Dec 1999
  43. Medicare Coverage Policy: Levocarnitine for end-stage renal disease decision memorandum. 2002.
  44. Steinman TI. L-carnitine supplementation in dialysis patients: does the evidence justify its use? Semin Dial 2005; 18:1.
  45. Bonomini M, Pandolfi A, Di Liberato L, et al. L-carnitine is an osmotic agent suitable for peritoneal dialysis. Kidney Int 2011; 80:645.
  46. Brass EP. Pharmacokinetic considerations for the therapeutic use of carnitine in hemodialysis patients. Clin Ther 1995; 17:176.
  47. Bain MA, Faull R, Fornasini G, et al. Accumulation of trimethylamine and trimethylamine-N-oxide in end-stage renal disease patients undergoing haemodialysis. Nephrol Dial Transplant 2006; 21:1300.
  48. Bain MA, Faull R, Milne RW, Evans AM. Oral L-carnitine: metabolite formation and hemodialysis. Curr Drug Metab 2006; 7:811.
  49. Schreiber B. Safety of oral carnitine in dialysis patients. Semin Dial 2002; 15:71.