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Carnitine Levels (carnitine + level)
Selected AbstractsPlasma and liver carnitine status of children with chronic liver disease and cirrhosisPEDIATRICS INTERNATIONAL, Issue 4 2001Mukadder A Selimo AbstractBackground: Carnitine is an essential cofactor in the transfer of long-chain fatty acids across the inner mitochondrial membrane for oxidation. As its synthesis is performed in the liver, alterations in carnitine metabolism is expected in liver diseases, especially in cirrhosis. Methods: In this study, we investigated plasma and liver carnitine concentrations of 68 children with chronic liver disease, 36 of whom had cirrhosis as well. Carnitine level was determined by enzymatic method. Results: Plasma and liver carnitine concentrations were not correlated. Mean plasma carnitine level of cirrhotic children was significantly lower than that of the control group (P<0. 0001). While there was no difference between liver carnitine concentrations of children with chronic liver disease and cirrhosis (P>0.05), mean plasma level of cirrhotics were lower (P<0.05). Plasma carnitine was correlated with albumin, triglyceride and gamma glutamyl transpeptidase (GGT) in patients with chronic liver disease (P<0.05). Liver carnitine was correlated with GGT in cirrhotic patients (P<0.005). Children with malnutrition had higher plasma and liver carnitine levels (P<0.05). The highest plasma and liver carnitine levels were detected in children with biliary atresia and criptogenic cirrhosis, respectively. Both the lowest plasma and liver carnitine levels were detected in Wilson's disease. Conclusion: Children with cirrhosis have low plasma carnitine concentrations. This finding is prominent in children with Wilson's disease. As carnitine is an essential factor in lipid metabolism, the carnitine supplementation for patients with cirrhosis in childhood, especially with Wilson's disease, seems to be mandatory. [source] Plasma carnitine levels in children with down syndrome,AMERICAN JOURNAL OF HUMAN BIOLOGY, Issue 6 2001Mehmet Seven Carnitine is responsible for several chemical processes, including lipid metabolism, nerve cell conduction, reduction in muscle hypotonia, and limitation in oxidative damage to cells. In patients with Down syndrome (DS), the process of growth is behind that of normal children and neuromuscular control is attained somewhat later. The purpose of this study was to assess variation in levels of carnitine in normal and DS children and the relationship between the amount of carnitine and age. The study involved 30 (15 girls, 15 boys) normal children and 40 (20 girls, 20 boys) DS patients of Turkish ancestry, 6 months to 13 years of age. Carnitine level was determined using Deufel's enzymatic method. Carnitine level was significantly lower in DS patients compared with normal children between 6 months to 5 years of age. Between 5 and 13 years of age, the level of carnitine was about the same in both the normal and DS groups. The results suggest that carnitine level shows a different pattern of age related increase in DS compared to normal children. Am. J. Hum. Biol. 13:721,725, 2001. Published 2001 Wiley-Liss, Inc. [source] Carnitine Palmityltransferase II (CPT2) Deficiency and Migraine Headache: Two Case ReportsHEADACHE, Issue 5 2003Marielle A. Kabbouche MD Background.,Migraine headache is common and has multiple etiologies. A number of mitochondrial anomalies have been described for migraine, and mitochondrial dysfunction has been implicated as one potential pathophysiological mechanism. Carnitine is used by mitochondria for fatty acid transportation; its deficiency, however, has not been implicated in migraine pathophysiology. Methods and Results.,Two adolescent girls presented to the Headache Center at Cincinnati Children's Hospital Medical Center with frequent headaches and were diagnosed with migraine by the International Headache Society (IHS) criteria. Both girls had a history of recurrent fatigue, muscle cramps, and multiple side effects from their prophylactic treatment. Carnitine levels were measured and found to be low. Carnitine supplementation was initiated. Both patients had a reduction in headache frequency, as well as an improvement in their associated symptoms and other complaints. A skin and muscle biopsy obtained from one patient revealed a partial carnitine palmityltransferase II deficiency in the muscle only. Conclusion.,Carnitine palmityltransferase II deficiency may represent another etiology for migraine headache, and may be useful in further defining the pathophysiology of migraine. When properly recognized, supplementation with carnitine may improve the outcome of the migraine as well as the carnitine-associated symptoms. [source] Chemical form of dietary l -Carnitine affects plasma but not tissue Carnitine concentrations in male Sprague,Dawley ratsJOURNAL OF ANIMAL PHYSIOLOGY AND NUTRITION, Issue 2 2009B. D. Lambert Summary In Experiment 1, rats (n = 54) were randomly assigned to control or one of the four sources of l -Carnitine supplemented at either 100 or 200 ,mol/kg/day and were allowed to acclimate for 14 days. Following a 12-h fast, plasma samples were obtained at 0, 5, 10, 15, 30, 60, 120, 240, 480 and 720 min after l -Carnitine feeding and assayed for free l -Carnitine concentration. Plasma-free l -Carnitine levels were affected by time after treatment intake (p < 0.0001) and l -Carnitine source (p < 0.0001). The time × source interaction was not statistically significant (p = 0.99). In Experiment 2, rats (n = 54) were randomly assigned to control or one of the four sources of l -Carnitine at either 100 or 200 ,mol/kg/day and were acclimated as in experiment 1. Rats were sacrificed 120 min after feeding. Samples of liver and skeletal muscle were obtained and assayed for free l -Carnitine concentration. Neither skeletal muscle (p = 0.44) or liver (p = 0.59) tissue concentrations of l -Carnitine were affected by any l -Carnitine source as compared with the control. We conclude that some differences exist in plasma concentrations of free l -Carnitine following ingestion of different chemical forms of l -Carnitine. It is unclear if these differences in the circulating concentration of free l -Carnitine translate into any physiological differences for the animal. In this study, chemical form of l -Carnitine had no effect on skeletal muscle or liver tissue concentrations of l -Carnitine in young male Wistar rats. [source] Stevia rebaudiana Bertoni extract supplementation improves lipid and carnitine profiles in C57BL/6J mice fed a high-fat dietJOURNAL OF THE SCIENCE OF FOOD AND AGRICULTURE, Issue 7 2010Jeong-Eun Park Abstract BACKGROUND: Stevia (Stevia rebaudiana Bertoni) is a non-caloric natural-source alternative to artificially produced sugar substitutes. This study investigated the effect of stevia extract on lipid profiles in C57BL/6J mice. Forty mice were divided into four groups: N-C (normal diet and distilled water), H-C (high-fat diet and distilled water), H-SC (high fat diet and sucrose, 1 mL kg,1 per day), and H-SV (high-fat diet and stevia extract, 1 mL kg,1 per day). RESULTS: Body weight gain was significantly higher in the H-SC group than in the H-SV group. Triglyceride concentrations in serum and liver were lower in the H-SV group than in the H-SC group. Serum total cholesterol concentrations were lower in the H-SV and H-C groups compared to the H-SC group. The concentrations of acid-insoluble acylcarnitine (AIAC) in serum were higher in the H-SV group than in the H-C and H-SC groups and the acyl/free carnitine level in liver was significantly higher in the H-SV group than in the N-C group. These results were supported by mRNA expression of enzymes related to lipid metabolism (ACO, PPAR,, ACS, CPT-I, ACC) assessed by real-time polymerase chain reaction. CONCLUSION: These results suggest that the supplementation of stevia extract might have an anti-obesity effect on high-fat diet induced obese mice. Copyright © 2010 Society of Chemical Industry [source] Plasma and liver carnitine status of children with chronic liver disease and cirrhosisPEDIATRICS INTERNATIONAL, Issue 4 2001Mukadder A Selimo AbstractBackground: Carnitine is an essential cofactor in the transfer of long-chain fatty acids across the inner mitochondrial membrane for oxidation. As its synthesis is performed in the liver, alterations in carnitine metabolism is expected in liver diseases, especially in cirrhosis. Methods: In this study, we investigated plasma and liver carnitine concentrations of 68 children with chronic liver disease, 36 of whom had cirrhosis as well. Carnitine level was determined by enzymatic method. Results: Plasma and liver carnitine concentrations were not correlated. Mean plasma carnitine level of cirrhotic children was significantly lower than that of the control group (P<0. 0001). While there was no difference between liver carnitine concentrations of children with chronic liver disease and cirrhosis (P>0.05), mean plasma level of cirrhotics were lower (P<0.05). Plasma carnitine was correlated with albumin, triglyceride and gamma glutamyl transpeptidase (GGT) in patients with chronic liver disease (P<0.05). Liver carnitine was correlated with GGT in cirrhotic patients (P<0.005). Children with malnutrition had higher plasma and liver carnitine levels (P<0.05). The highest plasma and liver carnitine levels were detected in children with biliary atresia and criptogenic cirrhosis, respectively. Both the lowest plasma and liver carnitine levels were detected in Wilson's disease. Conclusion: Children with cirrhosis have low plasma carnitine concentrations. This finding is prominent in children with Wilson's disease. As carnitine is an essential factor in lipid metabolism, the carnitine supplementation for patients with cirrhosis in childhood, especially with Wilson's disease, seems to be mandatory. [source] Changes in amino acid composition in the tissues of African catfish (Clarias gariepinus) as a consequence of dietary L-carnitine supplementsJOURNAL OF APPLIED ICHTHYOLOGY, Issue 3 2002R. O. A. Ozório A study was undertaken to examine the effect of different amounts of dietary lysine (13 and 21 g kg,1 diet), lipid (80 and 160 g kg,1 diet) and L -carnitine (0.2 and 1.0 g kg,1 diet) on growth performance, proximate composition and amino acid metabolism of the African catfish (Clarias gariepinus). Juvenile African catfish (23 ± 1.5 g/fish) were stocked into 70-L aquaria (16 aquaria, 28 fish/aquarium) connected to a recirculation system during a maximum period of 74 days. All groups were fed at a level of 24 g kg,0.8 day,1 in an experiment run at pair feeding. Animals receiving 1.0 g carnitine accumulated up to six times more carnitine in their tissues than animals receiving 0.2 g (P < 0.05). Acyl-carnitine and free L -carnitine levels increased in the whole body and in tissues. Dietary L -carnitine supplements increased protein-to-fat ratios in the body, but did not affect growth rate. Protein-to-fat ratios were only affected when the biosynthesis capacity of L -carnitine was restricted due to low lysine levels and when there was a shortage of dietary fat. When lysine was offered at 21 g kg,1 feed, dietary L -carnitine supplements did not affect the amino acid concentrations of body tissues. Dietary L -carnitine supplements raised the concentration of glutamic acid,>,aspartic acid,>,glycine > alanine > arginine > serine > threonine in skeletal muscle tissue (P < 0.05). Total amino acid concentration in muscle and liver tissues (dry-matter basis) increased from 506 to 564 and from 138 to 166 mg g,1, respectively, when diets were offered with high L -carnitine, low lysine and low fat levels. These data suggest that dietary L -carnitine supplementation may increase fatty acid oxidation and possibly decrease amino acid combustion for energy. [source] Effect of hemodialysis on carnitine levels in children with chronic renal failurePEDIATRICS INTERNATIONAL, Issue 1 2002Sevgi Mir Abstract Background:,Impaired structural and metabolic integrity of the kidney in chronic renal failure (CRF) effects carnitine metabolism by means of many factors. Depletion due to hemodialysis (HD) is one of the major concerns. The aim of the study was to investigate the effects of chronic renal failure and HD on plasma free carnitine (FC) concentrations in children. Methods:,Plasma FC concentrations were measured in age-matched 14 undialyzed patients, 20 dialyzed patients and 12 healthy children. In the HD group, measurements were done pre- and postdialysis and an hour after ceasing HD. None of the children have been receiving exogenous l -carnitine replacement. Results:,Plasma FC concentrations on either HD or conservative treatment were found to be decreased as compared to the healthy subjects (P < 0.001 and P = 0.001, respectively). The patients on HD had lower levels of plasma FC at the predialysis period than those on conservative treatment (P = 0.01). The FC levels significantly dropped at the postdialysis period as compared to those at the predialysis period (P < 0.001), but recovered at 1 h after ceasing HD. The mean duration of HD did not correlate with plasma FC levels at predialysis period. Conclusions:,Children with CRF, either dialyzed or undialyzed, have decreased plasma FC levels. Hemodialysis treatment significantly depletes plasma FC concentrations during the procedure, but predialysis levels are reached 1 hr after ceasing HD. [source] Plasma and liver carnitine status of children with chronic liver disease and cirrhosisPEDIATRICS INTERNATIONAL, Issue 4 2001Mukadder A Selimo AbstractBackground: Carnitine is an essential cofactor in the transfer of long-chain fatty acids across the inner mitochondrial membrane for oxidation. As its synthesis is performed in the liver, alterations in carnitine metabolism is expected in liver diseases, especially in cirrhosis. Methods: In this study, we investigated plasma and liver carnitine concentrations of 68 children with chronic liver disease, 36 of whom had cirrhosis as well. Carnitine level was determined by enzymatic method. Results: Plasma and liver carnitine concentrations were not correlated. Mean plasma carnitine level of cirrhotic children was significantly lower than that of the control group (P<0. 0001). While there was no difference between liver carnitine concentrations of children with chronic liver disease and cirrhosis (P>0.05), mean plasma level of cirrhotics were lower (P<0.05). Plasma carnitine was correlated with albumin, triglyceride and gamma glutamyl transpeptidase (GGT) in patients with chronic liver disease (P<0.05). Liver carnitine was correlated with GGT in cirrhotic patients (P<0.005). Children with malnutrition had higher plasma and liver carnitine levels (P<0.05). The highest plasma and liver carnitine levels were detected in children with biliary atresia and criptogenic cirrhosis, respectively. Both the lowest plasma and liver carnitine levels were detected in Wilson's disease. Conclusion: Children with cirrhosis have low plasma carnitine concentrations. This finding is prominent in children with Wilson's disease. As carnitine is an essential factor in lipid metabolism, the carnitine supplementation for patients with cirrhosis in childhood, especially with Wilson's disease, seems to be mandatory. [source] Plasma carnitine levels in children with down syndrome,AMERICAN JOURNAL OF HUMAN BIOLOGY, Issue 6 2001Mehmet Seven Carnitine is responsible for several chemical processes, including lipid metabolism, nerve cell conduction, reduction in muscle hypotonia, and limitation in oxidative damage to cells. In patients with Down syndrome (DS), the process of growth is behind that of normal children and neuromuscular control is attained somewhat later. The purpose of this study was to assess variation in levels of carnitine in normal and DS children and the relationship between the amount of carnitine and age. The study involved 30 (15 girls, 15 boys) normal children and 40 (20 girls, 20 boys) DS patients of Turkish ancestry, 6 months to 13 years of age. Carnitine level was determined using Deufel's enzymatic method. Carnitine level was significantly lower in DS patients compared with normal children between 6 months to 5 years of age. Between 5 and 13 years of age, the level of carnitine was about the same in both the normal and DS groups. The results suggest that carnitine level shows a different pattern of age related increase in DS compared to normal children. Am. J. Hum. Biol. 13:721,725, 2001. Published 2001 Wiley-Liss, Inc. [source] Disorders of carnitine transport and the carnitine cycle,AMERICAN JOURNAL OF MEDICAL GENETICS, Issue 2 2006Nicola Longo Abstract Carnitine plays an essential role in the transfer of long-chain fatty acids across the inner mitochondrial membrane. This transfer requires enzymes and transporters that accumulate carnitine within the cell (OCTN2 carnitine transporter), conjugate it with long chain fatty acids (carnitine palmitoyl transferase 1, CPT1), transfer the acylcarnitine across the inner plasma membrane (carnitine-acylcarnitine translocase, CACT), and conjugate the fatty acid back to Coenzyme A for subsequent beta oxidation (carnitine palmitoyl transferase 2, CPT2). Deficiency of the OCTN2 carnitine transporter causes primary carnitine deficiency, characterized by increased losses of carnitine in the urine and decreased carnitine accumulation in tissues. Patients can present with hypoketotic hypoglycemia and hepatic encephalopathy, or with skeletal and cardiac myopathy. This disease responds to carnitine supplementation. Defects in the liver isoform of CPT1 present with recurrent attacks of fasting hypoketotic hypoglycemia. The heart and the muscle, which express a genetically distinct form of CPT1, are usually unaffected. These patients can have elevated levels of plasma carnitine. CACT deficiency presents in most cases in the neonatal period with hypoglycemia, hyperammonemia, and cardiomyopathy with arrhythmia leading to cardiac arrest. Plasma carnitine levels are extremely low. Deficiency of CPT2 present more frequently in adults with rhabdomyolysis triggered by prolonged exercise. More severe variants of CPT2 deficiency present in the neonatal period similarly to CACT deficiency associated or not with multiple congenital anomalies. Treatment for deficiency of CPT1, CPT2, and CACT consists in a low-fat diet supplemented with medium chain triglycerides that can be metabolized by mitochondria independently from carnitine, carnitine supplements, and avoidance of fasting and sustained exercise. © 2006 Wiley-Liss, Inc. [source] | |||||||||||||