No abstract available.
Anticonvulsants* ; Carnitine* ; Liver*

No abstract available.
Carnitine* ; Renal Dialysis*

No abstract available.
Animals ; Carnitine* ; Heart* ; Myocardium* ; Rats*

No abstract available.
Adult ; Carnitine ; Hepatitis ; Humans ; Transferases

PURPOSE: The purpose of this study is to review the evidence comparing the efficacy and safety between L-carnitine and extracorporeal elimination therapy in the management of acute valproic acid L-carnitine vs Extracorporeal Elimination for Acute Valproic acid Intoxication METHODS: PubMed, Embase, Cochrane library, Web of Science, KoreaMed, KMbase, and KISS were searched, using the terms carnitine and valproic acid. All studies, regardless of design, reporting efficacy or safety endpoints were included. Reference citations from identified publications were reviewed. Both English and Korean languages were included. Two authors extracted primary data elements including poisoning severity, presenting features, clinical management, and outcomes. RESULTS: Thirty two articles including 33 cases were identified. Poisoning severity was classified as 3 mild, 11 moderate, and 19 severe cases. Nine cases were treated with L-carnitine while 24 cases received extracorporeal therapy without L-carnitine. All patients except one expired patient treated with hemodialysis recovered clinically and no adverse effects were noted. A case report comparing two patients who ingested the same amount of valproic acid showed increased ICU stay (3 vs 11 days) in case of delayed extracorporeal therapy. CONCLUSION: Published evidence comparing L-carnitine with extracorporeal therapy is limited. Based on the available evidence, it is reasonable to consider L-carnitine for patients with acute valproic acid overdose. In case of severe poisoning, extracorporeal therapy would also be considered in the early phase of treatment.
Carnitine* ; Humans ; Poisoning ; Renal Dialysis ; Valproic Acid*

PURPOSE: Epileptic patients under prolonged treatment with anticonvulsants such as valproate tend to have low serum carnitine level. The object of this study is to observe the interrelationship between anticonvulsants and serum carnitine level and its related symptoms. METHODS: We measured the serum carnitine levels in 31 epileptic patients (18 males, 13 females) receiving diverse antiepileptic drugs and 32 healthy children (20 males, 12 females) using enzymatic cycling method. RESULTS: 1) Among the control group, total, free, and acyl-carnitine in serum were 50.04+/-11.44, 37.42+/-11.13, and 13.98+/-7.49micro mol/L (mean+/-S.D.), respectively, and no significant differences were observed between age and sex. 2) Among 31 patients with treatment, total serum carnitine level and free carnitine level below 2 standard deviation from the mean control value was not observed. 3) Among 31 patients with treatment, total serum carnitine level below 2 standard deviation from the age matched mean control value was not observed. 4) No significant relationship was observed between the serum carnitine level and the dosage, duration and the serum concentration of the antiepileptic drugs. 5) There were no abnormalities in serum AST, ALT levels among the patients treated. CONCLUSION: Although our study didn't reveal that serum carnitine level can be lowered in patients treated with anticonvulsants such as valproate, many others reported it. So periodic measurement of serum carnitine level before and after treatment with anticonvulsants should be considered.
Anticonvulsants* ; Carnitine* ; Child* ; Humans ; Male ; Valproic Acid

No abstract available.
Cardiomyopathy, Dilated* ; Carnitine* ; Estrogens, Conjugated (USP)*

Carnitine is necessary for transport of long-chain fatty acids into mitochondria, to enter the beta-oxidation cycle. Four carnitine cycle defects have been described. The carnitine transporter mediates carnitine transport across the plasma membrane. Symptoms include hypoketotic hypoglycaemia and cardiomyopathy. Some affected subjects are asymptomatic. Newborn screening detects very low levels of free carnitine in some but not all. Carnitine palmitoyltransferase type IA (CPTI) transports long-chain fatty acyl-CoAs across the outer mitochondrial membrane. Affected infants have hypoketotic hypoglycaemia with catabolic stress, but otherwise remain well. Newborn screening tests reveal elevated free carnitine, (elevated C0/C16+C18). Sensitivity is unclear and confirmation needs leukocyte or fibroblast assays. Carnitine-acylcarnitine translocase transfers fatty acylcarnitines across the inner mitochondrial membrane. The most common presentation is sudden death in the first days. Carnitine palmitoyltransferase type II (CPTII) converts long-chain acylcarnitines to long-chain acylCoAs for beta-oxidation. Severe deficiency is lethal. Newborn screening for both disorders reveals elevated palmitoylcarnitine and enzymology or mutation analysis is needed for diagnosis. Late-onset CPTII is the most common disorder, presenting as muscle pain and rhabdomyolysis on severe exercise. All 4 disorders can be detected by newborn screening, with variable sensitivity. Late-onset CPTII probably cannot be detected. Carnitine transporter, CPTI and late-onset CPTII have proven treatment strategies.
Carnitine ; metabolism ; Carnitine O-Palmitoyltransferase ; deficiency ; Humans ; Infant, Newborn ; Metabolism, Inborn Errors ; diagnosis ; enzymology ; Neonatal Screening

PURPOSE: This study aimed to evaluate the effect of anticonvulsants on serum carnitine levels as well as normal serum carnitine levels. METHODS: We measured the serum carnitine levels in 53 healthy children(34 males, 19 females) and 115 epileptic children(55 males, 60 females) receiving a various antiepileptic drugs. We assessed the effects of antiepileptic drugs on serum carnitine level together with a correlation between serum carnitine level and duration of treatment, and blood level of anticonvulsant. RESULTS: 1.Carnitine levels in healthy children 1)There was a positive correlation between total and free carnitine serum levels and the age of the children. 2)The serum total carnitine levels were increased in age group over 5 years of age and serum free carnitine levels were increased in age group over 1 year of age as compared with those of between 1 month and 12 months age group. 2.Carnitine levels in epileptic children receiving anticonvulsants 1)The serum levels of total and free carnitine were significantly reduced in the patients with valproate monotherapy group, valproate polytherapy group, and polytherapy group without valproate as compared with the control group. 2)The reduction was more significant in the patients of valproate polytherapy group than in those of valproate monotherapy group. 3.There was a significant inverse correlation between the serum carnitine levels and the duration of the valproate treatment but not between serum carnitine levels and the blood level of valproate. 4.Carnitine deficiency was corrected in all cases after oral administration of L-carnitine(50mg/kg/day). CONCLUSIONS: Carnitine deficiency may be suspected in patients taking valproate therapy and regular measurement of carnitine levels appears warranted in these patients. If carnitine deficiency is documented, the patient can be treated by oral carnitine supplementation.
Administration, Oral ; Anticonvulsants* ; Carnitine* ; Child ; Humans ; Male ; Valproic Acid

PURPOSE: To evaluate the effects of oral Tamoxifen only versus L-Carnitine plus Tamoxifen in patients with Peyronie's disease. MATERIALS AND METHODS: All 45 patients with Peyronie's disease, diagnosed using accepted definitions, were randomized into two groups and treated for 3 months with Tamoxifen only(40 mg/day)(n=17) or a combination ofL-Carnitine(2 g/day) and Tamoxifen(40 mg/day)(n=28). A medical history was obtained, and a physical examination was performed. Plaque size, pain, erectile function(IIEF score), and penile curvature were assessed. Both before and after therapy, the differences between the 2 groups were compared using independent-sample t-test with p<0.05 considered significant. RESULTS: The mean age of the 45 patients was 52.1 years, and no severe adverse events occurred in either group. In the Tamoxifen only group, the mean decrease of plaque-length was 0.46+/-0.88 mm, and mean reduction in the pain rating scale was 0.44+/-0.53. In the L-Carnitine and Tamoxifen group, mean decrease of plaque-length was 1.57+/-0.92 mm, and mean reduction in the pain rating scale was 1.27+/-0.96. Based on IIEF scores, the improvement of erectile function was 0.88+/-0.64 in the Tamoxifen only group and 1.56+/-0.75 in the L-Carnitine and Tamoxifen group. The degree of penile curvature was also measured, and the reduction of curvature angle was 9.17+/-4.92 degrees in the Tamoxifen only group and 9.55+/-6.50 degrees in the L-Carnitine and Tamoxifen group. CONCLUSIONS: This study showed significantly greater improvements in plaque size, pain, erectile function, and curvature in patients with Peyronie's disease who were treated with L-Carnitine and Tamoxifen compared with those treated with Tamoxifen only.
Carnitine* ; Humans ; Male ; Penile Induration* ; Physical Examination ; Tamoxifen*