Effect Of Carnitine Supplementation Research Articles

Effect of carnitine supplementation on energy utilization in patients with type 2 diabetes and long-term tube feeding: A CARE-compliant case report

Introduction: Carnitine is essential for the energy utilization of long-chain fatty acids. However, the effects of carnitine supplementation on patients undergoing tube feeding remain unclear, and some enteral formula products do not contain carnitine. In order to gain insights into the role of carnitine supplementation on energy utilization in patients with long-term tube feeding, we observed the changes in respiratory quotient (RQ) of patients before and after the addition of carnitine due to the renewal of an enteral formula product. Case presentation: We observed 6 patients who continued tube feeding with the same enteral formula product scheduled for renewal. All 6 participants had diabetes, and indirect calorimetry data were available for 4 patients. Participants were observed for 3 months after switching to the new formulation. After the switching, a carnitine supplementation of 120 to 180 mg/day was provided, depending on the intake amount. One month after switching, blood free carnitine and total carnitine levels in all 6 patients increased from below the lower limit to within the range of the Japanese reference standard. In 3 of the 4 patients for whom indirect calorimetry was possible, the fasting RQ at baseline was >0.90, suggesting impaired lipid utilization. The mean fasting RQ significantly decreased 2 months after the switch. The 1- and 2-hour postprandial RQ also showed a significant decrease after switching. Conclusion: Carnitine supplementation of enteral formulas may be important for normal energy utilization of lipids in patients receiving long-term tube feeding. An evaluation using randomized controlled trials with a larger number of patients is required.

Effect of Carnitine Supplementation in Pediatric Patients with Left Ventricular Dysfunction.

Carnitine is an essential amino acid involved in transporting fatty acids across the mitochondrial membrane. Fatty acids are a primary source of energy for the myocardium. Studies in adults demonstrated decreased carnitine levels in the ischemic myocardium, but subsequent exogenous carnitine supplementation showed improvement of myocardial metabolism and left ventricular function. However, only limited data regarding carnitine are available in pediatrics. A single-center retrospective, paired data study was conducted. Patients < 18years, left ventricular ejection fraction (LVEF) < 55% by echocardiography, and had received at least 7days of oral or intravenous carnitine supplementation between January 2018 and March 2021 are included in the study. Several endpoints and covariates were collected for each patient: before, one week after, one month after, and 6months after carnitine supplementation. Univariate analysis consisted of an analysis of variance (ANOVA), followed by an analysis of covariance (ANCOVA) to model LVEF while adjusting for other variables. 44 patients included in the final analyses. LVEF significantly improved from 50.5 to 56.6% (p < 0.01). When LVEF was adjusted for other interventions (mechanical ventilation, afterload reduction, diuretic therapy, spironolactone), the estimated means demonstrated a significant increase from 45.7 to 58.0% (p < 0.01). Free carnitine level increased significantly (p = 0.03), and N-terminal-pro-brain natriuretic peptide (p = 0.03), creatinine (p < 0.01), and lactate (p < 0.01) all significantly decreased over the study period. Carnitine supplementation in pediatric patients with left ventricular systolic dysfunction may be associated with an increase in LVEF and improvement in laboratory markers of myocardial stress and cardiac output.

The Effect of Carnitine Supplementation on Left Ventricular Function: Lessons from Current Evidence and Insights for Future Studies

Introduction: In children, data on the effects on carnitine supplementation and myocardial function are limited. A few studies have investigated the relationship between serum carnitine levels in the setting of depressed cardiac function and have demonstrated possible benefits. As such, this systematic review and meta-analyses aimed to assess the effects carnitine supplementation on left ventricular function. Materials and Methods: A systematic review of the literature was performed to identify full text manuscripts in English. PubMed, EMBASE, and the Cochrane databases were queried. Studies were included with data from pediatric patients, that used carnitine supplementation and included preand post-carnitine data for at least one of the outcomes of interest. Results: A total of six studies including 144 patients were included. Carnitine dosage ranged from 50 to 100 mg/kg/day. The average duration of carnitine therapy was 9.8 months. Left ventricular ejection fraction was higher after carnitine supplementation with a mean difference between groups of 3.56 [95% confidence interval 0.06–7.06, p-value 0.04]. Left ventricular shortening fraction was higher after carnitine supplementation with a mean difference between groups of 3.68 [95% confidence interval 1.22–6.15, p-value 0.01]. Left ventricular end diastolic diameter was higher after carnitine supplementation, but the difference did not reach significance. Conclusion: Carnitine supplementation may augment left ventricular ejection fraction and shortening fraction. Those with lower baseline ejection fraction and shortening fraction appear to benefit the most from carnitine supplementation. Additional studies of the effects of carnitine on cardiac function are warranted.

Effect of Carnitine Supplementation and Resistance Training on Skeletal Muscle Function

Effect of Carnitine Supplementation and Resistance Training on Skeletal Muscle Function

Effect of carnitine supplementation on growth performance and economics in pigs fed high fat diet

Thirty two weaned piglets were randomly divided into three groups allotted to the three dietary treatments, T1- control ration, T2- control ration plus 5 per cent fat and T3- T2 plus 150 mg/kg carnitine. The total dry matter intake, fortnightly body weight of each individual animal and economics were recorded. There was a significant improvement (P&gt;0.01; and 0.05) observed for pigs under supplemented group (T2 and T3) than that of control group (T1) with regards to all growth parameters studied during growing phase than finisher phase and overall period. The cost of feed per kg weight gain during the overall period was found to be comparable among treatments (T1, T2, and T3). It may be concluded that primary response of pigs to dietary supplements were more during growing stage than finisher and inclusion of 5 % fat would be beneficial for improving growth in weaned LWY piglets.

EFFECT OF CARNITINE SUPPLEMENTATION ON CARDIAC FUNCTION AND METABOLISM ASSESSED USING CINE MRI AND HYPERPOLARIZED MRS

Carnitine is responsible for the movement of fatty acids across the mitochondrial membrane to allow for subsequent β-oxidation. Carnitine has been shown to act as a buffer for excess acetyl units within mitochondria through the generation of acetylcarnitine. It is known that carnitine levels are decreased in multiple diseases, such as diabetes and heart failure, and that carnitine supplementation can have a cardioprotective effect. The purpose of this study was to investigate the effect of L-Carnitine supplementation of cardiac function and metabolism in the healthy rat heart. 8 healthy rats treated for 2 weeks with daily IP injections of either L-Carnitine (3 g/kg/day) or saline (control group) were subjected to CINE-MRI, hyperpolarized MRS and echocardiography. Carnitine treated animals gained less weight compared to controls. Cardiac index was higher for the carnitine treated animals compared to controls and diastolic function analysis by echocardiography showed that the E/E’ ratio was lower in the carnitine treated group. Hyperpolarized MRS demonstrated elevated acetylcarnitine levels in the myocardium of L-Carnitine treated animals and blood plasma analysis showed increased levels of carnitine in the treated group after 2 weeks of daily treatment with L-Carnitine. For the first time it has been shown that daily injections of L-Carnitine can alter cardiac metabolism and function in vivo in the healthy rat heart, can increase acetylcarnitine levels in the myocardium and improve diastolic function. On-going work is exploring the effect of carnitine supplementation on the diabetic heart to investigate the mechanisms behind the previously demonstrated cardioprotective effects of carnitine supplementation.

全自動二次元電気泳動装置（Auto2D）を用いたカルニチンの抗酸化作用の解析

全自動二次元電気泳動装置（Auto2D）を用いたカルニチンの抗酸化作用の解析

Antioxidant effects of carnitine supplementation on 14-3-3 protein isoforms in the aged rat hippocampus detected using fully automated two-dimensional chip gel electrophoresis

We here described the antioxidant effects of carnitine supplementation on 14-3-3 protein isoforms in the aged rat hippocampus detected using the fully automated two-dimensional chip gel electrophoresis system (Auto2D). This system was easy and convenient to use, and the resolution obtained was more sensitive and higher than that of conventional two-dimensional polyacrylamide gel electrophoresis (2-D PAGE). We separated and identified five isoforms of the 14-3-3 protein (beta/alpha, gamma, epsilon, zeta/delta, and eta) using the Auto2D system. We then examined the antioxidant effects of carnitine supplementation on the protein profiles of the cytosolic fraction in the aged rat hippocampus, demonstrating that carnitine supplementation suppressed the oxidation of methionine residues in these isoforms. Since methionine residues are easily oxidized to methionine sulfoxide, the convenient and high-resolution 2-D PAGE system can be available to analyze methionine oxidation avoiding artifactual oxidation. We showed here that the Auto2D system was a very useful tool for studying antioxidant effects through proteomic analysis of protein oxidation.

Carnitine modulates crucial myocardial adenosine triphosphatases and acetylcholinesterase enzyme activities in choline-deprived rats

Choline is an essential nutrient, and choline deficiency has been associated with cardiovascular morbidity. Choline is also the precursor of acetylcholine (cholinergic component of the heart's autonomic nervous system), whose levels are regulated by acetylcholinesterase (AChE). Cardiac contraction-relaxation cycles depend on ion gradients established by pumps like the adenosine triphosphatases (ATPases) Na(+)/K(+)-ATPase and Mg(2+)-ATPase. This study aimed to investigate the impact of dietary choline deprivation on the activity of rat myocardial AChE (cholinergic marker), Na(+)/K(+)-ATPase, and Mg(2+)-ATPase, and the possible effects of carnitine supplementation (carnitine, structurally relevant to choline, is used as an adjunct in treating cardiac diseases). Adult male albino Wistar rats were distributed among 4 groups, and were fed a standard or choline-deficient diet for one month with or without carnitine in their drinking water (0.15% w/v). The enzyme activities were determined spectrophotometrically in the myocardium homogenate. Choline deficiency seems to affect the activity of the aforementioned parameters, but only the combination of choline deprivation and carnitine supplementation increased myocardial Na(+)/K(+)-ATPase activity along with a concomitant decrease in the activities of Mg(2+)-ATPase and AChE. The results suggest that carnitine, in the setting of choline deficiency, modulates cholinergic myocardial neurotransmission and the ATPase activity in favour of cardiac work efficiency.

Effects of carnitine supplementation on myocardial function and energy provision in experimental uraemia

Cardiovascular complications are the leading cause of death in patients with chronic kidney disease. The uraemic heart undergoes remodelling and changes in metabolic function. Experimental uraemia produces a reduction in the myocardial energy reserve phosphocreatine in parallel with left ventricular hypertrophy and depletion of serum carnitine. This study investigated the effects of chronic L-carnitine supplementation on myocardial substrate metabolism and function in the experimental uraemia. Experimental uraemia was induced surgically in male Sprague-Dawley rats via a subtotal nephrectomy. Carnitine was administered continuously via subcutaneous mini-osmotic pumps. Cardiac function and substrate oxidation were assessed in vitro by means of isovolumic perfusion using 13C NMR, at 3 and 6 weeks. Uraemic animals exhibited anaemia, kidney dysfunction and systemic carnitine deficiency but no myocardial tissue carnitine deficiency. Myocardial hypertrophy was abolished following carnitine supplementation. This was associated with a reduction in glucose utilisation. In summary carnitine supplementation prevents cardiac hypertrophy, and this effect is amplified with the duration of treatment. This is associated with a reduction in myocardial glucose utilisation but no significant modulation of myocardial function.

The Effects of Carnitine Supplementation in Hemodialysis Patients

Objectives: Patients receiving hemodialysis have been shown to be carnitine deficient due to many causes. Tissues, especially the skeletal muscle and myocardium, require carnitine for the production of energy. This study was performed to find out the effects of L-carnitine supplementation on muscular symptoms and cardiac functions in dialysis patients. Methods: Among 72 hemodialysis patients, 40 patients who showed decreased free carnitine levels were selected to receive L-carnitine intravenously after each hemodialysis session for 6 months. Before and after supplementation, echocardiography, various neurologic examinations and questionnaires were obtained. Results: After carnitine treatment for 6 months (1∼1.5 g per every hemodialysis session), the blood level of carnitine was increased more than 10 times (19.04±7.12 μmol/L vs. 267.24±69.94 μmol/L, P＜0.001). The left ventricular ejection fraction was improved in the patients who have less than 60% of ejection fraction (56.45±2.53% vs. 60.44±6.29%, P=0.03) after carnitine treatment. The neurological symptom score and isometric muscle power (pinch power) were improved, but the total neuropathy score, activities of daily living scale and grip power were not changed after carnitine supplementation on dialysis patients. Conclusion: Regular L-carnitine supplementation on hemodialysis patients can improve their left ventricular ejection fraction and some parts of functionality. (Ewha Med J 2012;35(2):89-94)

92 UTILISATION OF ENDOGENOUS FATTY ACID STORES FOR ENERGY PRODUCTION IN BOVINE PRE-IMPLANTATION EMBRYOS

Current embryo culture media are based on the carbohydrate metabolism of embryos. However, little is known about the metabolism of endogenous lipids. This is surprising given the high intracellular lipid densities of embryos of some species and the potential for ATP production via β-oxidation. L-carnitine is a β-oxidation co-factor that is absent in most culture media. The aim of this study is to investigate the influence of carnitine supplementation ± carbohydrates on bovine embryo development. Abattoir-derived cattle cumulus–oocyte complexes were cultured and fertilised (Sutton-McDowall et al. 2006 Biol. Reprod. 74, 881–888). Post-fertilisation (24 h), presumptive zygotes were transferred into an amino acid-free cleavage media ± carbohydrates (glucose, lactate and pyruvate) ±5 mM carnitine and cultured for 4 days. The absence of carbohydrates during culture resulted in embryos arresting at the 2- and 4-cell stages. Remarkably, +carnitine significantly increased development to the morula stage compared with +carbohydrates alone (20.4 ± 3% vs 4.7 ± 2.5% morula development; P &lt; 0.001). The combination of carbohydrates and carnitine supplementation further improved embryo development, with 14-fold more embryos reaching the morula stage after culture in the +carbohydrates +carnitine group compared with the +carbohydrates group (+carbohydrates = 3.1 ± 1.9 vs +carbohydrates +carnitine = 43.8 ± 9.1% morula development; P &lt; 0.05). The beneficial effects of carnitine supplementation on embryo development were reversed when embryos were cultured in presence of etomoxir, a non-reversible inhibitor of the rate-limiting enzyme of β-oxidation (development to 8-cell stage; +carnitine = 33.9 ± 8% vs +carnitine +etomoxir = 19.2 ± 4.9%; P &lt; 0.05). Intracellular lipid content of embryos +carnitine was determined by culturing presumptive zygotes in media -carbohydrates ± carnitine for 24 h. Lipid content of embryos was determined by measuring BODIPY 493/503 dye fluorescence. Carnitine supplementation reduced fluorescence intensity 1.8-fold (P &lt; 0.001). Adenosine triphosphate and ATP:ADP levels were measured in embryos after 24 h of culture ± carbohydrates ± carnitine. While there was a trend for +carnitine to increase ATP levels (P = 0.09), ADP levels were higher and ATP:ADP ratio were 1.9-fold lower (main effect, P &lt; 0.05) compared with embryos cultured in –carnitine. This indicates +carnitine embryos were more metabolically active, with higher rates of ATP-ADP conversion. We have shown carnitine supplementation supports pre-compaction embryo development and there is an additive effect of +carnitine +carbohydrate on early embryo development. This is most likely through increased β-oxidation levels within embryos. Current disparities between in vivo and in vitro embryo production, in particular increased lipid content (Romek et al. 2010 Theriogenology 74, 265–276) and decreased developmental potential of in vitro-produced embryos, may be an artefact resulting from limited lipid oxidation in vitro.

Carnitine Deficiency Aggravates Cyclophosphamide-Induced Cardiotoxicity in Rats

Background: This study examined, for the first time, the involvement of carnitine deficiency in cardiotoxicity, particularly cyclophosphamide (CP)-induced cardiomyopathy, as well as effects of carnitine supplementation with propionyl-L-carnitine (PLC) on cardiotoxicity. Methods: An animal model of carnitine deficiency was developed in rats treated with D-carnitine (DC)-mildronate (MD). Adult male Wistar albino rats were assigned to one of six treatment groups: the first three groups were injected intraperitoneally with normal saline, PLC (250 mg/kg/day), and DC (250 mg/kg/day) combined with MD (200 mg/kg/day), respectively, for 10 successive days. In groups 4–6, the same doses of normal saline, PLC and DC-MD were injected, respectively, during the 5 successive days before and after a single dose of CP (200 mg/kg). On day 6 after CP treatment, 24-hour urine was collected, then animals were sacrificed, and serum as well as hearts were isolated. Results: CP caused a significant increase in serum creatine phosphokinase isoenzyme (CK-MB), lactate dehydrogenase (LDH), urinary carnitine excretion and clearance and intramitochondrial acetyl-CoA/CoA-SH, and a significant decrease in serum free carnitine, total carnitine and adenosine triphosphate (ATP) contents in cardiac tissue. In the carnitine-depleted rats, CP induced dramatic increases in CK-MB and LDH levels, carnitine clearance and intramitochondrial acetyl-CoA/CoA-SH, as well as progressive reduction in total carnitine and ATP in cardiac tissues. Interestingly, PLC supplementation completely reversed the biochemical and histopathological changes induced by CP to the control values. Conclusion: (1) Carnitine deficiency is a risk factor which is involved in CP-related cardiomyopathy; (2) serum and urinary carnitine levels should be monitored and viewed as indices of CP-induced multiple organ toxicity, and (3) carnitine supplementation, using PLC, prevents the development of CP-induced cardiotoxicity.

Effect of carnitine supplementation on cardiac function in hemodialyzed children

Thirteen carnitine-deficient children (mean age, 16.1 +/- 2.56 years) on a three-times-weekly hemodialysis program for at least 1 year, and 11 healthy age matched children were involved in the study. All the patients had stable blood pressure and hemoglobin (Hb) levels with a maintenance dose of erythropoetin and none were digitalized. The total carnitine (TC) and free carnitine (FC) plasma levels were sampled prior to hemodialysis (HD) before and after 3 months of carnitine supplementation. A free carnitine (FC) to acylcarnitine (AC) ratio less than 4 was defined as carnitine deficiency. Intravenous L-carnitine was injected at a dose of 20-40 mg/kg three times weekly at the end of each dialysis session for a 3-month period. Echocardiographic examination was performed the day following HD, before and after carnitine treatment. Systolic and diastolic functions of the left ventricle, including the ejection fraction, were measured. Almost all the parameters were significantly different in controls and hemodialyzed patients. In carnitine-deficient hemodialyzed patients, 3 months of L-carnitine supplementation resulted in a significant increase in blood carnitine levels and the FC/AC ratio, but this was not associated with any significant improvement of cardiac function. Furthermore no significant changes were observed in plasma triglycerides, total cholesterol or other lipoprotein parameters before or after carnitine supplementation. Although there was a moderate increase in mean hematocrit (Hct) and Hb levels, these also did not reach statistically significant levels. These results suggest that the 3 months of carnitine supplementation is not sufficient to ameliorate cardiac function or increase Hb levels in children.

Effect of carnitine supplementation on lipid profile and anemia in children on chronic dialysis

We prospectively evaluated the effects of L-carnitine supplementation on plasma free carnitine (FC) levels, serum lipid profile, and erythropoietin (rhEPO) requirement in 24 children treated with peritoneal dialysis (PD; n=16) or hemodialysis (HD; n=8). The study was divided into a 3-month observation period, and a 3-month treatment period during which patients received 20 mg/kg per day of L-carnitine given orally. Clinical, biochemical, and hematological data were collected every 3 months. FC levels were measured in plasma and peritoneal dialysate by tandem mass spectrometry. There were no statistically significant changes in lipid levels, hemoglobin, or rhEPO requirements during the course of the study. Fifteen patients (13 PD, 2 HD) had plasma FC levels measured before and after treatment; FC levels increased from 32.1 +/- 14.1 micromol/l to 80.9 +/- 38.7 micromol/l (P<0.001). In PD patients, dialysate FC losses increased from 106 +/- 78 micromol/day at baseline to 178 +/- 119 micromol/day after supplementation. Positive correlations between FC plasma levels and dialysate levels (R=0.507) or daily excretion (R=0.603) were found after treatment. In our case series, an oral dose of 20 mg/kg per day of L-carnitine restored FC levels and produced a positive carnitine balance with no significant effects on hematological parameters or lipid profile over a 3-month period. Prolonged treatment duration may be required to obtain significant results.

Metabolic Effects of Abomasal l-Carnitine Infusion and Feed Restriction in Lactating Holstein Cows

l-Carnitine is required for mitochondrial fatty acid oxidation, but the effects of carnitine supplementation on nutrient metabolism during dry matter intake depression have not been determined in dairy cows. Studies in other species have revealed responses to l-carnitine that may be of specific benefit to dairy cows during the periparturient period. Eight lactating Holstein cows (132±36 d in milk) were used in a replicated 4×4 Latin square experiment with 14-d periods. Treatments were factorial combinations of abomasal infusion of either water or l-carnitine (20g/d; d 5 to 14) and either ad libitum or restricted intake (50% of previous 5-d dry matter intake; d 10 to 14) of a balanced lactation diet. Liver and muscle biopsies were obtained on d 14 of each period. Feed restriction induced negative balances of energy and metabolizable protein. In feed-restricted cows, carnitine infusion increased 3.5% fat-corrected milk yield compared with those infused with water. Total carnitine concentration in liver was increased in feed-restricted cows infused with carnitine but not in feed-restricted cows infused with water. Carnitine infusion stimulated in vitro oxidation of [1-14C] palmitate to acid-soluble products and decreased the proportion of [1-14C] palmitate that was converted to esterified products by liver slices. Feed-restricted cows infused with carnitine had lower liver total lipid concentration and tended to have decreased triglyceride accumulation compared with feed-restricted cows infused with water. Plasma nonesterified fatty acid concentration was not altered by carnitine infusion but was increased by feed restriction; serum β-hydroxybutyric acid was increased by carnitine infusion in feed-restricted cows. In cows fed for ad libitum intake, carnitine infusion affected β-hydroxybutyric acid, insulin, and urea N in serum, liver glycogen concentration, and in vitro alanine oxidation by liver slices, suggesting that hepatic and peripheral nutrient metabolism was influenced. l-Carnitine infusion effectively decreased liver lipid accumulation during feed restriction as a result of greater capacity for hepatic fatty acid oxidation. Further research examining dietary supplementation of l-carnitine during the periparturient period is warranted.

Is sudden death with vitamin C deficiency caused by lack of carnitine?

We investigated the effect of carnitine supplementation during vitamin C (ASC) deficiency by measuring the levels of ASC and carnitine in plasma and cardiac muscle cells (CMC), and histological analysis with electron microscopy. The levels of carnitine were significantly decreased in ASC-deficient rats in plasma and the heart than those in the control. In carnitine supplemented ASC-deficient rats, a significant increase of carnitine levels were observed in both plasma and heart. The number of lipid droplets significantly increased in the ASC-deficient rats compared to the control rats, but did not increase in carnitine supplemented rats. These results indicate that ASC deficiency causes a generalized mitochondrial abnormality and accumulation of lipid droplets in CMC as observed in carnitine deficiency, and supplementation of carnitine prevented these changes even in the presence of ASC deficiency.

The effect of oral L‐carnitine supplementation on the lipid profiles of hyperlipidaemic children

To investigate the carnitine status and the effect of carnitine supplementation on serum lipid profiles in children with hyperlipoproteinaemia, a clinical open trial was conducted at Hacettepe University Ihsan Dogramaci Children's Hospital, Section of Nutrition and Metabolism. Patients were given carnitine at a dose of 100 mg/kg/d for 12 wk. Blood samples for the determination of lipid profile and carnitine levels and urine samples for carnitine levels were obtained on admission, at week 4 and week 12 of the study period. A total of 41 children were enrolled in the study: 20 patients had type II heterozygotes, eight patients had type II homozygotes, three patients had type III, six patients had type V and four patients had secondary hyperlipidaemias. Serum and urine carnitine levels were within normal limits on admission. No significant correlations were found between serum carnitine levels and serum lipid profiles. Serum HDL and apolipoprotein A-I decreased significantly during the 12 wk of intervention in type II heterozygotes. In type II homozygotes, total cholesterol and LDL levels at weeks 4 and 12 increased significantly compared to initial levels. No significant change was noted for lipid parameters in hyperlipoproteinaemia type V. The results of this trial demonstrated that carnitine supplementation was of no benefit for children with hyperlipidaemias, especially in primary hyperlipoproteinaemia type II heterozygotes, homozygotes and type V.

Effects of Carnitine Supplementation on Muscle Metabolism by the Use of Magnetic Resonance Spectroscopy and Near-Infrared Spectroscopy in End-Stage Renal Disease

Background/Aim: A defect in skeletal muscle mitochondrial metabolism develops in patients with chronic renal failure on haemodialysis. Treatment with carnitine, a compound essential for normal mitochondrial function, has been suggested to have significant benefits in such patients, so we carried out a study to see if carnitine acts by improving muscle bioenergetics and function. Methods: In a phase II randomised double-blind trial, patients with end-stage renal disease received placebo or intravenous L-carnitine (20 mg/kg dry body weight three times weekly after haemodialysis) for 16 weeks (n = 13 in each group). ³¹P magnetic resonance spectroscopy, ¹H magnetic resonance imaging and near-infrared spectroscopy were used to measure muscle bioenergetics and function at baseline and at 16 weeks. Results: There were no significant differences between groups at baseline. Mean plasma carnitine rose 10-fold in the carnitine group but was unchanged in the placebo group. L-Carnitine had no statistically significant effect on any of the parameters measured. The rate of proton efflux from muscle, as a measure of tissue perfusion, was low in both groups and was not affected by treatment. Conclusions: The study failed to show any significant effect of 16 weeks’ L-carnitine supplementation on these objective measures of muscle metabolism and function. Slow proton efflux from muscle provides evidence supporting low blood flow and, therefore, decreased oxygen availability, as an underlying mechanism for muscle mitochondrial dysfunction in this disorder.

Carnitine supplementation improves apolipoprotein B levels in pediatric peritoneal dialysis patients.

There have been conflicting reports concerning the effect of carnitine supplementation on lipid metabolism in patients on peritoneal dialysis (PD). We investigated several parameters of lipid metabolism in pediatric PD patients supplemented with carnitine. The study included 20 patients receiving PD (treatment group) aged 2-18 years and a matched healthy control group. In the treatment group, baseline triglyceride, total cholesterol, low-density lipoprotein cholesterol, very low-density lipoprotein cholesterol, and apolipoprotein B levels were higher than in the control group. High-density lipoprotein cholesterol, free fatty acid, phospholipids, and apolipoprotein A-I levels were not different from those in the control group. The baseline plasma free carnitine level was lower and acyl-carnitine level was higher in the treatment group. No difference was found between the groups with respect to plasma total carnitine levels. Oral l-carnitine supplementation (50 mg/kg per day for 30 days) led to a significant decrease (from a baseline value of 146.6+/-51.8 mg/dl to 63.6+/-22.2 mg/dl, P<0.001) in apolipoprotein B levels, and no significant change in the other lipid parameters of the treatment group. Oral l-carnitine supplementation does not ameliorate the lipid profile in pediatric PD patients, but it causes a significant decrease in apolipoprotein B levels. Hence, carnitine supplementation may be recommended for decreasing apolipoprotein B levels in this patient population.