Effectiveness of monitoring free carnitine levels for L ‐carnitine supplementation in hemodialysis patients to maintain carnitine sufficiency and nutritional factors

l-carnitine is an important factor in fatty acid metabolism, and carnitine deficiency is common in dialysis patients. This study evaluated whether l-carnitine supplementation improved muscle spasm, cardiac function, and renal anemia in dialysis patients. Eighty Japanese outpatients (62 hemodialysis (HD) patients and 18 peritoneal dialysis (PD) patients) received oral l-carnitine (600 mg/day) for 12 months; the HD patients further received intravenous l-carnitine injections (1000 mg three times/week) for 12 months, amounting to 24 months of treatment. Muscle spasm incidence was assessed using a questionnaire, and cardiac function was assessed using echocardiography. Baseline free carnitine concentrations were relatively low in patients who underwent dialysis for >4 years. Total carnitine serum concentration, free carnitine, and acylcarnitine significantly increased after oral l-carnitine treatment for 12 months, and after intravenous l-carnitine injection. There was no significant improvement in muscle spasms, although decreased muscle cramping after l-carnitine treatment was reported by 31% of patients who had undergone HD for >4 years. Hemoglobin concentrations increased significantly at 12 and 24 months in the HD group. Therefore, l-carnitine may be effective for reducing muscle cramping and improving hemoglobin levels in dialysis patients, especially those who have been undergoing dialysis for >4 years.

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P37 A single paediatric centre experience of l-carnitine supplementation in medium-chain acyl-coa dehydrogenase deficiency (mcadd)

Archives of Disease in Childhood ◽

10.1136/archdischild-2017-314585.46 ◽

2018 ◽

Vol 103 (2) ◽

pp. e2.41-e2

Author(s):

William Batten ◽

Efstathia Chronopoulou ◽

Germaine Pierre

Keyword(s):

Fatty Acids ◽

Dehydrogenase Deficiency ◽

Free Carnitine ◽

List Type ◽

Carnitine Supplementation ◽

Medium Chain ◽

Number Of Patients ◽

Chain Acyl ◽

Body Odour ◽

Low Levels

University Hospitals BristolAimThe aim of the project was to establish whether patients with MCADD treated at Bristol Royal Hospital for Children (BRHC) are supplemented with l-carnitine and to establish further details for supplementation.BackgroundMCADD is the most common type of fatty acid oxidation disorder in the UK.1l-carnitine is a co-factor in the oxidation of fatty acids in mitochondria and eliminates excess medium chain fatty acids. l-carnitine supplementation in MCADD is controversial with no consistent findings in publications.2MethodsThe paediatric metabolic database at BRHC was interrogated to find the number of patients with MCADD. Patients who were either deceased or discharged from the metabolic service were excluded. Medical notes and pharmacy records were reviewed for each patient to determine patient demographics, dosing information and reasons for implementation of l-carnitine using a data collection tool which was piloted on the first 5 patients. The data was analysed using Microsoft Excel.ResultsOf 35 patients treated for MCADD, 13 patients (37%) received l-carnitine. The most common age group treated was 6–10 years (n=6). The mean dose of L-carnitine=77.7 mg/kg/day; range 45–100 mg/kg/day; most common dose=100 mg/kg/day (n=6). 92% of patients received the 300 mg/mL liquid. Of the 13 patients, 1 had low free carnitine but was asymptomatic; 8 were symptomatic with low levels and 3 were symptomatic with borderline low levels and 1 had no reason documented. All symptomatic patients improved with therapy. Those who stopped supplementation on correction (n=3), all restarted due to return of symptoms with falling levels. 2 of these patients have a vegetarian diet, a reason for low free carnitine. 2 patients had their doses reduced from 100 to 50 mg/kg/day due to side effects, particularly fishy body odour exacerbated by hot weather. 1 patient complained of the same effect, but the dose remained the same.ConclusionThe majority of patients in this centre are not treated with l-carnitine. The reason for treatment is mainly low free carnitine associated with symptoms. Clinical and biochemical improvement was observed with treatment. The most common dose prescribed was 100 mg/kg/day; with the observed side effect of a fishy body odour also being the most common reason for dose reduction. The most commonly prescribed preparation is a 300 mg/mL licensed liquid. Problems with availability and palatability reduce the usage of licensed tablet preparations. We hope to expand the project nationally with other UK metabolic centres.ReferencesSaudebray. Inborn metabolic diseases: Diagnosis and treatment (5th ed.) 2011. Springer.Matern D, Rinaldo P. Medium-chain acyl-coenzyme a dehydrogenase deficiency 2015. GeneReviews®. 2000, updated 2015. http://www.ncbi.nlm.nih.gov/books/NBK1424/

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The Effects of Carnitine Supplementation in Hemodialysis Patients

The Ewha Medical Journal ◽

10.12771/emj.2012.35.2.89 ◽

2012 ◽

Vol 35 (2) ◽

pp. 89

Author(s):

Seung-Jung Kim

Keyword(s):

Hemodialysis Patients ◽

Carnitine Supplementation

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Effect of L-Carnitine Supplementation in Hemodialysis Patients

Nephron ◽

10.1159/000045993 ◽

2001 ◽

Vol 88 (3) ◽

pp. 218-223 ◽

Cited By ~ 39

Author(s):

E. Veselá ◽

J. Racek ◽

L. Trefil ◽

V. Jankovy’ch ◽

M. Pojer

Keyword(s):

Hemodialysis Patients ◽

Carnitine Supplementation

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Effects of L-carnitine supplementation on nutritional, immunological, and cardiac parameters in hemodialysis patients: a pilot study

Renal Replacement Therapy ◽

10.1186/s41100-015-0004-0 ◽

2015 ◽

Vol 1 (1) ◽

Cited By ~ 1

Author(s):

Haruki Sada ◽

Akihiko Kato ◽

Ryo Sumimoto ◽

Hiromitsu Ohmori ◽

Hideki Ohdan

Keyword(s):

Pilot Study ◽

Hemodialysis Patients ◽

Carnitine Supplementation

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Oral carnitine supplementation improves dyslipidemia in hemodialysis patients

Journal of Renal Nutrition ◽

10.1053/j.jrn.2008.03.167 ◽

2008 ◽

Vol 18 (3) ◽

pp. S42

Keyword(s):

Hemodialysis Patients ◽

Carnitine Supplementation

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P1588CAN L-CARNITINE SUPPLEMENTATION IMPROVE CARDIOPULMONARY FUNCTION? A RANDOMIZED CONTROLLED TRIAL IN HEMODIALYSIS PATIENTS

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p1588 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Fatemeh Yassari ◽

Alireza Eslaminejad ◽

Mehran Marashian ◽

Farzaneh Dastan ◽

Farzaneh Maleki Kazeri

Keyword(s):

Clinical Trial ◽

Randomized Clinical Trial ◽

Controlled Trial ◽

Hemodialysis Patients ◽

Chronic Hemodialysis ◽

Carnitine Supplementation ◽

Carnitine Level ◽

Cardiopulmonary Function ◽

Significant Difference ◽

Spss Software

Abstract Background and Aims It is well known that L-Carnitine is a cardioprotective agent, which balances cardiac energy metabolism, by promoting mitochondrial β-oxidation and facilitating transport of long chain fatty acids into the mitochondrial matrix. It has been shown that L-Carnitine level in plasma and tissue is lower in hemodialysis patients and they may lose the benefits of this substance. As far as our knowledge, the effect of L-Carnitine on cardiopulmonary function has not been evaluated by ergospirometry up to now. The aim of this randomized clinical trial was to assess the effects of L-carnitine supplementation on cardiopulmonary Function in hemodialysis patients through ergospirometry. Method This randomized clinical trial was conducted on 46 chronic hemodialysis patients. The patients were divided into two groups. In both groups ergospirometry parameters (VE Max, VO2-Max, VCO2 Max, AT, VE/VCO2 Slope) were recorded for a 3-month period. During this period, one group received L-Carnitine at doses of 2 gr/day orally and the other group received only placebo. After three months, all of the mentioned parameters were rechecked and statistical analysis performed by SPSS software. Results Only CRP was different between the two groups increasing in placebo group significantly after 3 months (P = 0.018). No significant difference was detected in Cardiopulmonary factors. In terms of ergospirometry factors, PET-CO2 was the only parameter significantly increased in the treatment group (P = 0.026). Conclusion The present results indicate that L-Carnitine can improve cardiopulmonary function among hemodialysis patients.

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