carnitine and Hyperammonemia studies

Hyperammonemia: Elevated level of AMMONIA in the blood. It is a sign of defective CATABOLISM of AMINO ACIDS or ammonia to UREA.

Research Excerpts

Excerpt	Relevance	Reference
"Longitudinal assessments of carnitine and fatigue in patients with hepatocellular carcinoma suggest that lenvatinib affects the carnitine system in patients undergoing lenvatinib therapy and that carnitine insufficiency increases fatigue."	9.34	Carnitine insufficiency is associated with fatigue during lenvatinib treatment in patients with hepatocellular carcinoma. ( Ando, H; Ikejima, K; Ishizuka, K; Kitagawa, R; Kokubu, S; Miyazaki, A; Nagahara, A; Okubo, H; Okubo, S; Saito, H; Shiina, S, 2020)
"Valproate (VPA) administration may be associated with adverse metabolic effects, among is hyperammonemia, which could suggest metabolic abnormalities as carnitine deficiency."	9.14	The risk of asymptomatic hyperammonemia in children with idiopathic epilepsy treated with valproate: relationship to blood carnitine status. ( Abdella, MM; Hamed, SA, 2009)
"Here, I present the effects of levocarnitine on renal anemia in hemodialysis patients with carnitine deficiency, focusing on the effect on dose reduction in erythropoiesis-stimulating agents and the influence on erythropoiesis resistance index."	8.98	Effects of Levocarnitine on Cardiac Function and Renal Anemia in Hemodialysis Patients. ( Higuchi, T, 2018)
"Resolution of hyperammonemia caused by Valproic acid therapy may be enhanced with the administration of L-carnitine."	8.95	Sudden valproate-induced hyperammonemia managed with L-carnitine in a medically healthy bipolar patient: Essential review of the literature and case report. ( Ballabio, M; Cattaneo, CI; D'Innella, P; Fornaro, M; Ressico, F; Valsesia, R, 2017)
"Could 10-20% of autism be prevented? We hypothesize that nonsyndromic or "essential" autism involves extreme male bias in infants who are genetically normal, but they develop deficiency of carnitine and perhaps other nutrients in the brain causing autism that may be amenable to early reversal and prevention."	8.95	Brain carnitine deficiency causes nonsyndromic autism with an extreme male bias: A hypothesis. ( Beaudet, AL, 2017)
"Hyperammonemia is one of the major symptoms of primary carnitine deficiency."	8.80	Antagonizing effect of AP-1 on glucocorticoid induction of urea cycle enzymes: a study of hyperammonemia in carnitine-deficient, juvenile visceral steatosis mice. ( Kobayashi, K; Li, MX; Saheki, T, 2000)
" Both levocarnitine and lactulose, either alone or in combination, have been used for the treatment of hyperammonemia associated with valproic acid, however they have not been directly compared in the literature."	8.02	Evaluation of levocarnitine, lactulose, and combination therapy for the treatment of valproic acid-induced hyperammonemia in critically ill patients. ( Cunningham, B; Fowler, M; Mullen, C; Nicholson, C, 2021)
"The aim of the study was to examine the frequency of hyperammonemia secondary to valproic acid treatment in status epilepticus and to describe the characteristics of the patients."	7.91	Intravenous use of valproic acid in status epilepticus is associated with high risk of hyperammonemia. ( Lind, J; Nordlund, P, 2019)
" L-Carnitine is a vitamin-like substance and several reports have described the usefulness of L-carnitine supplementation in cases of cirrhosis, with confirmed effectiveness against refractory hepatic encephalopathy."	7.81	Usefulness of Levocarnitine and/or Branched-Chain Amino Acids during Invasive Treatment for Hepatocellular Carcinoma. ( Hasegawa, H; Ishihara, T; Iwasa, M; Kobayashi, Y; Mifuji-Moroka, R; Sekoguchi-Fujikawa, N; Sugimoto, R; Takei, Y; Tanaka, H; Yoshikawa, K, 2015)
"Disorders of carnitine biosynthesis have recently been associated with neurodevelopmental syndromes such as autism spectrum disorder (ASD)."	7.81	Improvement of regressive autism symptoms in a child with TMLHE deficiency following carnitine supplementation. ( Beaudet, AL; Comeaux, MS; Elsea, SH; Scaglia, F; Schaaf, CP; Sun, Q; Yang, Y; Ziats, MN, 2015)
"Carnitine deficiency is relatively common in epilepsy; risk factors reportedly include combination antiepileptic drug (AED) therapy with valproic acid (VPA), young age, intellectual disability, diet and enteral or parenteral feeding."	7.81	Carnitine deficiency: Risk factors and incidence in children with epilepsy. ( Fukuda, M; Ishii, E; Iwano, S; Kawabe, M; Kikuchi, C; Kuwabara, K; Morimoto, T; Suzuki, Y; Takehara, M; Wakamoto, H, 2015)
"Valproic acid, a branched short-chain fatty acid, has numerous action mechanisms which turn it into a broad spectrum anticonvulsant drug and make its use possible in some other pathologies such as bipolar disorder."	7.80	Hyperammonemia associated with valproic acid concentrations. ( Alvariza, S; Fagiolino, P; Guevara, N; Ibarra, M; Magallanes, L; Maldonado, C; Olano, I; Olmos, I; Vázquez, M, 2014)
"A random retrospective chart review was conducted to document serum carnitine levels on 100 children with autism."	7.72	Relative carnitine deficiency in autism. ( Cummings, C; Filipek, PA; Gargus, JJ; Juranek, J; Nguyen, MT, 2004)
"Juvenile visceral steatosis (JVS) mouse is an animal model of human primary carnitine deficiency caused by a mutation of the gene encoding carnitine transporter, and suffers from various symptoms, such as fatty liver, growth retardation, hyperammonemia, hypoglycemia, and cardiac hypertrophy."	7.71	Hyperammonemia in carnitine-deficient adult JVS mice used by starvation. ( Horiuchi, M; Jalil, A; Kobayashi, K; Li, XX; Saheki, T; Yoshida, G, 2002)
"Understanding the consequences of cancer for energy metabolism is required in order to define strategies that both prevent and treat malnutrition."	6.78	Lower carnitine plasma values from malnutrition cancer patients. ( Demenice, R; dos Santos, JS; Jordão, AA; Leme, IA; Marchini, JS; Portari, GV; Rabito, EI, 2013)
"Carnitine is a vitamin-like substance that regulates lipid metabolism and energy production."	6.66	Usefulness of Carnitine Supplementation for the Complications of Liver Cirrhosis. ( Hanai, T; Imai, K; Shimizu, M; Shiraki, M; Suetugu, A; Takai, K, 2020)
"L-carnitine has also been shown to improve insulin sensitivity and elevate pyruvate dehydrogenase (PDH) flux."	6.66	The Importance of the Fatty Acid Transporter L-Carnitine in Non-Alcoholic Fatty Liver Disease (NAFLD). ( Hodson, L; Neubauer, S; Pavlides, M; Savic, D, 2020)
"Valproic acid (VPA) is a short-chain fatty acid widely prescribed in the treatment of seizure disorders and epilepsy syndromes, although its therapeutic value may be undermined by its toxicity."	5.91	Quantitative systems pharmacology Model to characterize valproic acid-induced hyperammonemia and the effect of L-carnitine supplementation. ( Fagiolino, P; Ibarra, M; Maldonado, C; Schiavo, A; Trocóniz, IF; Vázquez, M, 2023)
"Carnitine plays a crucial role in the metabolism of fatty acids as well as energy production."	5.72	Evaluation of the relationship between serum carnitine levels and intradialytic complications in children with kidney failure. ( Abbasi, A; Askarian, F; Bakhtiari Koohsorkhi, M; Bazargani, B; Fahimi, D; Moghtaderi, M; Mojtahedi, SY; Samimi, M, 2022)
"Non-ischemic dilated cardiomyopathy is the most common subgroup of heart failure in young adults."	5.72	Primary Carnitine Deficiency as a Treatable Cause of Heart Failure in Young Patients. ( Bayraktaroğlu, S; Canda, E; Kalkan Uçar, S; Kayıkçıoğlu, M; Özbay, B; Şimşek, E; Yağmur, B, 2022)
"No further seizures were noted by day 5."	5.51	A newborn with seizures born to a mother diagnosed with primary carnitine deficiency. ( Chen, S; Hu, Y; Huang, Y; Lin, J; Lin, Z; Nan, Y; Zhou, X, 2019)
"Rhabdomyolysis is an emergency requiring rapid diagnosis and suitable aetiological treatment."	5.48	Primary carnitine deficiency in a 57-year-old patient with recurrent exertional rhabdomyolysis. ( Biancalana, V; Chanson, JB; Echaniz-Laguna, A; Gaignard, P, 2018)
"Carnitine is an essential cofactor for the transportation of long-chain fatty acids into the mitochondria."	5.48	SLC22A5 Mutations in a Patient With Systemic Primary Carnitine Deficiency and Cleft Palate-Successful Perioperative Management. ( Hu, CH; Hu, CW; Lo, LJ; Wu-Chou, YH, 2018)
"Carnitine is a commonly used antidote for acute intoxication of valproic acid, but is not a therapeutic option for management of chronic adults with adverse effects related to valproic acid."	5.46	L-Carnitine supplementation to reverse hyperammonemia in a patient undergoing chronic valproic acid treatment: A case report. ( Fagiolino, P; Guevara, N; Maldonado, C; Silveira, A; Vázquez, M, 2017)
"Acylcarnitine analysis was performed by tandem mass spectrometry on dried-blood spots collected on day 3 of life."	5.46	Similarities between acylcarnitine profiles in large for gestational age newborns and obesity. ( Couce, ML; de Castro, MJ; López, M; Rite, S; Roca, I; Sánchez-Pintos, P, 2017)
"Longitudinal assessments of carnitine and fatigue in patients with hepatocellular carcinoma suggest that lenvatinib affects the carnitine system in patients undergoing lenvatinib therapy and that carnitine insufficiency increases fatigue."	5.34	Carnitine insufficiency is associated with fatigue during lenvatinib treatment in patients with hepatocellular carcinoma. ( Ando, H; Ikejima, K; Ishizuka, K; Kitagawa, R; Kokubu, S; Miyazaki, A; Nagahara, A; Okubo, H; Okubo, S; Saito, H; Shiina, S, 2020)
"We describe the case of a 56-year-old man who presented to the emergency department after an intentional VPA overdose, was found to have hyperammonemia, and was treated with L-carnitine exclusively."	5.22	Valproic Acid Overdose: Case Report and Literature Review. ( Berezowski, I; Frasure, SE; Mazer-Amirshahi, M; Patel, J; Pourmand, A; Tran, QK, 2022)
"Valproate (VPA) administration may be associated with adverse metabolic effects, among is hyperammonemia, which could suggest metabolic abnormalities as carnitine deficiency."	5.14	The risk of asymptomatic hyperammonemia in children with idiopathic epilepsy treated with valproate: relationship to blood carnitine status. ( Abdella, MM; Hamed, SA, 2009)
"Here, I present the effects of levocarnitine on renal anemia in hemodialysis patients with carnitine deficiency, focusing on the effect on dose reduction in erythropoiesis-stimulating agents and the influence on erythropoiesis resistance index."	4.98	Effects of Levocarnitine on Cardiac Function and Renal Anemia in Hemodialysis Patients. ( Higuchi, T, 2018)
"Could 10-20% of autism be prevented? We hypothesize that nonsyndromic or "essential" autism involves extreme male bias in infants who are genetically normal, but they develop deficiency of carnitine and perhaps other nutrients in the brain causing autism that may be amenable to early reversal and prevention."	4.95	Brain carnitine deficiency causes nonsyndromic autism with an extreme male bias: A hypothesis. ( Beaudet, AL, 2017)
"Resolution of hyperammonemia caused by Valproic acid therapy may be enhanced with the administration of L-carnitine."	4.95	Sudden valproate-induced hyperammonemia managed with L-carnitine in a medically healthy bipolar patient: Essential review of the literature and case report. ( Ballabio, M; Cattaneo, CI; D'Innella, P; Fornaro, M; Ressico, F; Valsesia, R, 2017)
"Some chemotherapeutic agents cause carnitine deficiency, which causes general fatigue."	4.12	Relationship between Carnitine Deficiency and Tyrosine Kinase Inhibitor Use in Patients with Chronic Myeloid Leukemia. ( Hamada, T; Hatta, Y; Iizuka, K; Iriyama, N; Koike, T; Kurihara, K; Miura, K; Nakagawa, M; Nakamura, H; Nakayama, T; Takahashi, H; Takei, M; Uchino, Y, 2022)
" Both levocarnitine and lactulose, either alone or in combination, have been used for the treatment of hyperammonemia associated with valproic acid, however they have not been directly compared in the literature."	4.02	Evaluation of levocarnitine, lactulose, and combination therapy for the treatment of valproic acid-induced hyperammonemia in critically ill patients. ( Cunningham, B; Fowler, M; Mullen, C; Nicholson, C, 2021)
"Primary carnitine deficiency (PCD) affects fatty acid oxidation and is associated with cardiomyopathy and cardiac arrhythmia, but the risk of sudden death in PCD is unknown."	3.96	Increased risk of sudden death in untreated primary carnitine deficiency. ( Dunø, M; Hansen, SH; Joensen, HD; Køber, L; Lund, AM; Nielsen, OW; Rasmussen, J; Steuerwald, U, 2020)
" Her hyperammonemia was corrected by hemodialysis, followed by sodium benzoate, L-arginine, levocarnitine and protein-free diet therapy."	3.96	Long-term survival of a patient with acute neonatal-onset metabolic encephalopathy with carbamoyl phosphate synthetase 1 deficiency. ( Ando, Y; Arisaka, O; Imataka, G; Ishii, J; Nitta, A; Takagi, Y; Yoshihara, S, 2020)
"The aim of the study was to examine the frequency of hyperammonemia secondary to valproic acid treatment in status epilepticus and to describe the characteristics of the patients."	3.91	Intravenous use of valproic acid in status epilepticus is associated with high risk of hyperammonemia. ( Lind, J; Nordlund, P, 2019)
" Investigations revealed non-ketotic hypoglycemia suggesting FAOD which was later confirmed as carnitine uptake defect with gas chromatography and mass spectrometry and mutation analysis."	3.85	Infant with hepatomegaly and hypoglycemia: A setting for fatty acid oxidation defects. ( Pai, G; Poddar, U; Ravindranath, A; Srivastava, A; Yachha, SK, 2017)
", drowsiness, coma) were selected for treatment with l-arginine."	3.85	L-Arginine in the treatment of valproate overdose - five clinical cases. ( Eyer, F; Felgenhauer, N; Fernando, M; Rabe, C; Schrettl, V, 2017)
"Carnitine deficiency is relatively common in epilepsy; risk factors reportedly include combination antiepileptic drug (AED) therapy with valproic acid (VPA), young age, intellectual disability, diet and enteral or parenteral feeding."	3.81	Carnitine deficiency: Risk factors and incidence in children with epilepsy. ( Fukuda, M; Ishii, E; Iwano, S; Kawabe, M; Kikuchi, C; Kuwabara, K; Morimoto, T; Suzuki, Y; Takehara, M; Wakamoto, H, 2015)
" L-Carnitine is a vitamin-like substance and several reports have described the usefulness of L-carnitine supplementation in cases of cirrhosis, with confirmed effectiveness against refractory hepatic encephalopathy."	3.81	Usefulness of Levocarnitine and/or Branched-Chain Amino Acids during Invasive Treatment for Hepatocellular Carcinoma. ( Hasegawa, H; Ishihara, T; Iwasa, M; Kobayashi, Y; Mifuji-Moroka, R; Sekoguchi-Fujikawa, N; Sugimoto, R; Takei, Y; Tanaka, H; Yoshikawa, K, 2015)
"Disorders of carnitine biosynthesis have recently been associated with neurodevelopmental syndromes such as autism spectrum disorder (ASD)."	3.81	Improvement of regressive autism symptoms in a child with TMLHE deficiency following carnitine supplementation. ( Beaudet, AL; Comeaux, MS; Elsea, SH; Scaglia, F; Schaaf, CP; Sun, Q; Yang, Y; Ziats, MN, 2015)
"We report the case of an aborted awake craniotomy for a left frontotemporoinsular glioma due to ammonia encephalopathy on a patient taking Levetiracetam, valproic acid and clobazam."	3.81	Ammonia encephalopathy and awake craniotomy for brain language mapping: cause of failed awake craniotomy. ( Arroyo Pérez, R; Fernández-Candil, JL; León Jorba, A; Pacreu Terradas, S; Villalba Martínez, G; Vivanco-Hidalgo, RM, 2015)
"Carnitine deficiency can cause cardiomyopathy and cardiac arrhythmia."	3.80	Primary Carnitine deficiency in the Faroe Islands: health and cardiac status in 76 adult patients diagnosed by screening. ( Køber, L; Lund, AM; Nielsen, OW; Rasmussen, J, 2014)
"Valproic acid, a branched short-chain fatty acid, has numerous action mechanisms which turn it into a broad spectrum anticonvulsant drug and make its use possible in some other pathologies such as bipolar disorder."	3.80	Hyperammonemia associated with valproic acid concentrations. ( Alvariza, S; Fagiolino, P; Guevara, N; Ibarra, M; Magallanes, L; Maldonado, C; Olano, I; Olmos, I; Vázquez, M, 2014)
"Supplemental L-carnitine is needed in patients receiving an enteral diet free of carnitine, those with combination therapy of VPA and PB under oral feeding conditions, and those who develop hyperammonemia during VPA therapy."	3.79	[The association of hypocarnitinemia with enteral diets and antiepileptic drugs in children and adults with severe physical and mental disabilities]. ( Maruhashi, K; Nakamura, N; Niida, Y; Ohno, I; Seki, H; Tsuji, T; Wakisaka, A; Yamada, S, 2013)
"Exposure to antibiotics containing pivalic acid was associated with encephalopathy and progression to lethal cardiac arrhythmia in patients suffering from PCD."	3.79	Primary carnitine deficiency and pivalic acid exposure causing encephalopathy and fatal cardiac events. ( Djurhuus, H; Køber, L; Lund, AM; Nielsen, OW; Rasmussen, J, 2013)
"We evaluated a 22-year-old woman with primary carnitine deficiency and ventricular fibrillation, as well as her first-degree relatives."	3.77	Primary carnitine deficiency and sudden death: in vivo evidence of myocardial lipid peroxidation and sulfonylation of sarcoendoplasmic reticulum calcium ATPase 2. ( Bristow, M; Cohen, R; Colucci, W; Joseph, L; Klein, M; Mazzini, M; Monahan, K; Qin, F; Siwik, D; Tadros, T, 2011)
"N-carbamoyl-L-glutamate, combined with a protein-restricted diet and L-carnitine supplementation, was apparently effective as first-line treatment of hyperammonemia in this infant with PA."	3.76	Outpatient treatment of propionic acidemia-associated hyperammonemia with N-carbamoyl-L-glutamate in an infant. ( Aydin, A; Demirci, E; Soyucen, E, 2010)
"Two cases of acute valproic acid poisoning with central nervous system depression and raised ammonia level without hepatotoxicity were reported."	3.74	Two cases of valproic acid poisoning treated with L-carnitine. ( Chan, YC; Lau, FL; Tse, ML, 2007)
"A random retrospective chart review was conducted to document serum carnitine levels on 100 children with autism."	3.72	Relative carnitine deficiency in autism. ( Cummings, C; Filipek, PA; Gargus, JJ; Juranek, J; Nguyen, MT, 2004)
"Juvenile visceral steatosis (JVS) mouse is an animal model of human primary carnitine deficiency caused by a mutation of the gene encoding carnitine transporter, and suffers from various symptoms, such as fatty liver, growth retardation, hyperammonemia, hypoglycemia, and cardiac hypertrophy."	3.71	Hyperammonemia in carnitine-deficient adult JVS mice used by starvation. ( Horiuchi, M; Jalil, A; Kobayashi, K; Li, XX; Saheki, T; Yoshida, G, 2002)
"L-carnitine is an important factor in fatty acid metabolism, and carnitine deficiency is common in dialysis patients."	2.94	Effects of L-Carnitine Supplementation in Patients Receiving Hemodialysis or Peritoneal Dialysis. ( Ichikawa, S; Io, H; Kanda, R; Kuwasawa-Iwasaki, M; Muto, M; Nakata, J; Nohara, N; Suzuki, Y; Tomino, Y; Wakabayashi, K, 2020)
"l-Carnitine treatment in asymptomatic patients with PCD may not only prevent cardiac complications but also boost skeletal muscle fat metabolism during exercise."	2.87	L-Carnitine Improves Skeletal Muscle Fat Oxidation in Primary Carnitine Deficiency. ( Hedermann, G; Lund, AM; Madsen, KL; Olesen, JH; Preisler, N; Rasmussen, J; Vissing, J, 2018)
" The primary safety outcome was difference in serious adverse events (SAEs) per patient between groups."	2.79	Preliminary safety and efficacy of L-carnitine infusion for the treatment of vasopressor-dependent septic shock: a randomized control trial. ( Claremont, H; Jones, AE; Kline, JA; Krabill, V; Puskarich, MA, 2014)
"Understanding the consequences of cancer for energy metabolism is required in order to define strategies that both prevent and treat malnutrition."	2.78	Lower carnitine plasma values from malnutrition cancer patients. ( Demenice, R; dos Santos, JS; Jordão, AA; Leme, IA; Marchini, JS; Portari, GV; Rabito, EI, 2013)
"L-carnitine has also been shown to improve insulin sensitivity and elevate pyruvate dehydrogenase (PDH) flux."	2.66	The Importance of the Fatty Acid Transporter L-Carnitine in Non-Alcoholic Fatty Liver Disease (NAFLD). ( Hodson, L; Neubauer, S; Pavlides, M; Savic, D, 2020)
"Carnitine is a vitamin-like substance that regulates lipid metabolism and energy production."	2.66	Usefulness of Carnitine Supplementation for the Complications of Liver Cirrhosis. ( Hanai, T; Imai, K; Shimizu, M; Shiraki, M; Suetugu, A; Takai, K, 2020)
"Carnitine deficiency is a common condition in hemodialysis patients."	2.61	Carnitine Profile by Tandem Mass Spectrometry and Dialysis Patients. ( Hanafusa, N; Kamei, D; Kamei, Y; Mineshima, M; Miwa, N; Nitta, K; Tanaka, N; Tsuchiya, K; Tsukada, M, 2019)
"VPA is widely used for the treatment of generalized epilepsy."	2.61	[Valproic acid-induced hyperammonemic encephalopathy in a patient receiving valproic acid monotherapy]. ( Araki, M; Mukai, T; Naka, H; Shishido, T; Tokinobu, H; Yamada, H, 2019)
" For example, high dosage l-carnitine is an effective intervention for patients with Primary Carnitine Deficiency (PCD)."	2.58	Lipid storage myopathies: Current treatments and future directions. ( Little, DG; Schindeler, A; Summers, MA; Vasiljevski, ER, 2018)
"l-Carnitine is a wáter-soluble compound that humans may obtain both by food ingestion and endogenous synthesis from trimethyl-lysine."	2.55	Significance of l-carnitine for human health. ( Adeva-Andany, MM; Calvo-Castro, I; Donapetry-García, C; Fernández-Fernández, C; Pedre-Piñeiro, AM, 2017)
"Carnitine was approved by the US Food and Drug Administration in 1985 for treatment of 'primary carnitine deficiency', and later in 1992 for treatment of 'secondary carnitine deficiency', a definition that included the majority of relevant metabolic disorders associated with low or abnormal plasma carnitine levels."	2.53	Historical Perspective on Clinical Trials of Carnitine in Children and Adults. ( Buist, NR, 2016)
"Carnitine deficiency has been reported to cause erythropoietin refractory anemia in chronic hemodialysis patients and thrombocytopenia or leukopenia of cirrhosis, and carnitine supplementation can improve hematopoiesis in patients with hepatic or renal failure."	2.53	[Correlation between serum L-carnitine concentration and neutrophil engraftment in patients treated with cord blood transplantation]. ( Hayashi, K; Hyo, R; Kondo, T; Koresawa, R; Matsuhashi, Y; Sano, F; Sugihara, T; Susuki, S; Tasaka, T; Wada, H, 2016)
"Carnitine is a hydrophilic quaternary amine that plays a number of essential roles in metabolism with the main function being the transport of long-chain fatty acids from the cytosol to the mitochondrial matrix for β-oxidation."	2.52	Disorders of carnitine biosynthesis and transport. ( El-Hattab, AW; Scaglia, F, 2015)
"Carnitine is an essential cofactor in the proper metabolism of valproic acid and ammonia elimination."	2.48	Levocarnitine for valproic-acid-induced hyperammonemic encephalopathy. ( Mock, CM; Schwetschenau, KH, 2012)
"Not surprisingly, a group of muscle disorders due to defects in this system usually leads to the development of acute rhabdomyolysis in conditions such as infection, fasting and prolonged exercise."	2.46	State of the art in muscle lipid diseases. ( Liang, WC; Nishino, I, 2010)
"Carnitine is an amino acid derivative that is an essential cofactor in the beta-oxidation of fatty acids."	2.45	Carnitine in the treatment of valproic acid-induced toxicity. ( Hantson, P; Lheureux, PE, 2009)
"Valproic acid (VPA) is a broad-spectrum antiepileptic drug and is usually well tolerated, but rare serious complications may occur in some patients receiving VPA chronically, including haemorrhagic pancreatitis, bone marrow suppression, VPA-induced hepatotoxicity (VHT) and VPA-induced hyperammonaemic encephalopathy (VHE)."	2.43	Science review: carnitine in the treatment of valproic acid-induced toxicity - what is the evidence? ( Gris, M; Lheureux, PE; Penaloza, A; Zahir, S, 2005)
"Valproic acid (VPA) is a short-chain fatty acid widely prescribed in the treatment of seizure disorders and epilepsy syndromes, although its therapeutic value may be undermined by its toxicity."	1.91	Quantitative systems pharmacology Model to characterize valproic acid-induced hyperammonemia and the effect of L-carnitine supplementation. ( Fagiolino, P; Ibarra, M; Maldonado, C; Schiavo, A; Trocóniz, IF; Vázquez, M, 2023)
"Non-ischemic dilated cardiomyopathy is the most common subgroup of heart failure in young adults."	1.72	Primary Carnitine Deficiency as a Treatable Cause of Heart Failure in Young Patients. ( Bayraktaroğlu, S; Canda, E; Kalkan Uçar, S; Kayıkçıoğlu, M; Özbay, B; Şimşek, E; Yağmur, B, 2022)
"Carnitine plays a crucial role in the metabolism of fatty acids as well as energy production."	1.72	Evaluation of the relationship between serum carnitine levels and intradialytic complications in children with kidney failure. ( Abbasi, A; Askarian, F; Bakhtiari Koohsorkhi, M; Bazargani, B; Fahimi, D; Moghtaderi, M; Mojtahedi, SY; Samimi, M, 2022)
"Carnitine is a potent endogenous antioxidant and cellular fatty acid transporter for antioxidative stress and energy production in the cardiovascular system."	1.62	Role of carnitine in regulation of blood pressure (MAP/SBP) and gene expression of cardiac hypertrophy markers (α/β-MHC) during insulin-induced hypoglycaemia: Role of oxidative stress. ( Al-Harbi, NO; Alanazi, MM; Alanazi, WA; Alasmari, AF; Alasmari, F; Alhoshani, A; Ali, N; Ansari, MA; Imam, F, 2021)
"Levocarnitine is the treatment of choice in valproic acid toxicity-related hyperammonaemia."	1.62	Managing valproic acid toxicity-related hyperammonaemia: an unpredicted course. ( Edquist, C; O'Rourke, N; Pagali, S, 2021)
"L-carnitine was taken by the patient until the morning of surgery."	1.62	General anesthesia with cisatracurium and sevoflurane for a patient with primary carnitine deficiency receiving L-carnitine therapy: A case report. ( Ai, L; Fang, Y, 2021)
" They were also offered an increased dosage of stiripentol if treatment with carnitine improved the encephalopathy."	1.56	Starting stiripentol in adults with Dravet syndrome? Watch for ammonia and carnitine. ( Andrade, DM; Marques, P; Sadoway, T; Selvarajah, A; Tabarestani, S; Zulfiqar Ali, Q, 2020)
"No further seizures were noted by day 5."	1.51	A newborn with seizures born to a mother diagnosed with primary carnitine deficiency. ( Chen, S; Hu, Y; Huang, Y; Lin, J; Lin, Z; Nan, Y; Zhou, X, 2019)
"Rhabdomyolysis is an emergency requiring rapid diagnosis and suitable aetiological treatment."	1.48	Primary carnitine deficiency in a 57-year-old patient with recurrent exertional rhabdomyolysis. ( Biancalana, V; Chanson, JB; Echaniz-Laguna, A; Gaignard, P, 2018)
"Carnitine is an essential cofactor for the transportation of long-chain fatty acids into the mitochondria."	1.48	SLC22A5 Mutations in a Patient With Systemic Primary Carnitine Deficiency and Cleft Palate-Successful Perioperative Management. ( Hu, CH; Hu, CW; Lo, LJ; Wu-Chou, YH, 2018)
"Acylcarnitine analysis was performed by tandem mass spectrometry on dried-blood spots collected on day 3 of life."	1.46	Similarities between acylcarnitine profiles in large for gestational age newborns and obesity. ( Couce, ML; de Castro, MJ; López, M; Rite, S; Roca, I; Sánchez-Pintos, P, 2017)
"Carnitine transport was reduced to 20% or less of normal in fibroblasts of 140 out of 358 subjects."	1.46	Functional and molecular studies in primary carnitine deficiency. ( Balakrishnan, B; Calderon, FRO; Frigeni, M; Longo, N; Mao, R; Pasquali, M; Yin, X, 2017)
"Carnitine is a commonly used antidote for acute intoxication of valproic acid, but is not a therapeutic option for management of chronic adults with adverse effects related to valproic acid."	1.46	L-Carnitine supplementation to reverse hyperammonemia in a patient undergoing chronic valproic acid treatment: A case report. ( Fagiolino, P; Guevara, N; Maldonado, C; Silveira, A; Vázquez, M, 2017)
"Hyperammonemia is a rare, often fatal complication after transplantation."	1.43	Hyperammonemia Syndrome After Lung Transplantation: A Single Center Experience. ( Bain, KB; Byers, DE; Chen, C; Hachem, RR; Iuppa, JA; Patterson, GA; Trulock, EP; Witt, CA; Yusen, RD, 2016)
"Carnitine deficiency was induced in Wistar rats by adding 20 mmol/L of sodium pivalate to drinking water (P)."	1.43	A Moderate Carnitine Deficiency Exacerbates Isoproterenol-Induced Myocardial Injury in Rats. ( Arduini, A; Bonomini, M; Giudice, PL, 2016)
"Sivelestat was given for the complication of ARDS."	1.42	Elevation of pivaloylcarnitine by sivelestat sodium in two children. ( Bo, R; Hasegawa, Y; Ishige, N; Kobayashi, H; Nakamura, M; Takahashi, T; Yamada, K; Yamaguchi, S, 2015)
"Carnitine levels were positively correlated to age in both males and females (p < 0."	1.40	Carnitine levels in 26,462 individuals from the nationwide screening program for primary carnitine deficiency in the Faroe Islands. ( Duno, M; Gislason, H; Janzen, N; Køber, L; Lund, AM; Nielsen, OW; Rasmussen, J; Steuerwald, U, 2014)
"Carnitine supplement was administrated in two patients, which resulted in resolution of symptoms and normalized ammonium levels."	1.40	Valproate-induced hyperammonemia in juvenile ceroid lipofuscinosis (Batten disease). ( Larsen, EP; Ostergaard, JR, 2014)
"Childhood cancer survivors are at high risk of developing congestive heart failure (CHF) compared with the general population, and there is a dose-dependent increase in CHF risk by anthracycline dose."	1.40	Carnitine and cardiac dysfunction in childhood cancer survivors treated with anthracyclines. ( Armenian, SH; Bhatia, S; Freyer, DR; Gelehrter, SK; Herrera, C; Landier, W; Mascarenhas, L; Menteer, JD; Reichman, L; Vase, T; Venkataraman, K; Venkatramani, R; Wilson, KD, 2014)
"N-carbamylglutamate is approved for the treatment of hyperammonemia in N-acetylglutamate synthetase deficiency and may have efficacy in other urea cycle disorders."	1.39	Successful early management of a female patient with a metabolic stroke due to ornithine transcarbamylase deficiency. ( Bellantuono, R; Bellino, V; De Palo, T; Favia, V; Morrone, A; Papadia, F; Ranieri, A; Tummolo, A, 2013)
"Carglumic acid was well tolerated with no side effects noted."	1.39	Use of carglumic acid in the treatment of hyperammonaemia during metabolic decompensation of patients with propionic acidaemia. ( Abacan, M; Boneh, A, 2013)
"Carnitine transport was significantly reduced in fibroblasts obtained from all patients with primary carnitine deficiency, but was significantly higher in the asymptomatic women's than in the symptomatic patients' fibroblasts (P < 0."	1.38	Genotype-phenotype correlation in primary carnitine deficiency. ( Ardon, O; di San Filippo, CA; Longo, N; Ndukwe Erlingsson, UC; Pasquali, M; Rose, EC, 2012)
" Information on therapeutic usage, administration, and dosage was also recorded."	1.36	Setting up an emergency stock for metabolic diseases. ( Fernandez-Llamazares, CM; Manrique-Rodríguez, S; Sanjurjo-Sáez, M; Serrano, ML, 2010)
"Because hyperammonemia is thought to contribute to the pathogenesis of hepatic encephalopathy, we examined the effects of ammonia on ATP levels, neuronal morphology, and synaptic function in rat hippocampal slices."	1.33	Ammonia-mediated LTP inhibition: effects of NMDA receptor antagonists and L-carnitine. ( Funatsu, M; Izumi, M; Izumi, Y; Matsukawa, M; Zorumski, CF, 2005)

Timeframe	Studies, this research(%)	All Research%
pre-1990	1 (0.56)	18.7374
1990's	0 (0.00)	18.2507
2000's	15 (8.47)	29.6817
2010's	115 (64.97)	24.3611
2020's	46 (25.99)	2.80

Trial	Phase	Enrollment	Study Type	Start Date	Status
Impact of Hemodialysis on Plasma Carnitine Levels in Patients With End Stage Renal Disease[NCT05817799]	Phase 2/Phase 3	83 participants (Anticipated)	Interventional	2023-01-01	Recruiting
PSG in Valproate-induced Nocturnal Enuresis in Children[NCT04191863]		260 participants (Actual)	Observational	2018-09-01	Completed
Fat and Carbohydrate Metabolism During Exercise, With and Without L-carnitine Supplementation in Patients With Carnitine Transporter Deficiency[NCT02226419]		14 participants (Actual)	Interventional	2013-08-31	Completed
Use of Acetyl L-Carnitine in Patients With Covid-19 Pneumonia[NCT04623619]		100 participants (Anticipated)	Interventional	2020-12-15	Not yet recruiting
Pilot Study: Effect of Carnitine Supplementation on Acylcarnitine Profile and Myocardial Function in Children and Young Adults Receiving Continuous Renal Replacement Therapy[NCT01941823]		29 participants (Actual)	Observational	2014-12-31	Completed
Effect of Adding Lamotrigine to Sodium Valproate in Childhood Epilepsy: Clinicolabratory Study[NCT05881928]	Phase 4	50 participants (Anticipated)	Interventional	2023-07-25	Not yet recruiting
Fat and Sugar Metabolism During Exercise in Patients With Metabolic Myopathy[NCT02635269]		60 participants (Actual)	Interventional	2016-01-31	Active, not recruiting
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Article	Year
Clinical characteristics of primary carnitine deficiency: A structured review using a case-by-case approach. Journal of inherited metabolic disease, 2022, Volume: 45, Issue:3 Topics: Cardiomyopathies; Carnitine; Child, Preschool; Humans; Hyperammonemia; Infant, Newborn; Muscular Dis	2022
Valproic Acid Overdose: Case Report and Literature Review. The Journal of emergency medicine, 2022, Volume: 63, Issue:5 Topics: Anticonvulsants; Carnitine; Drug Overdose; Humans; Hyperammonemia; Male; Middle Aged; Valproic Acid	2022
Carnitine Inborn Errors of Metabolism. Molecules (Basel, Switzerland), 2019, Sep-06, Volume: 24, Issue:18 Topics: Aldehyde Oxidoreductases; Cardiomyopathies; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmi	2019
L-carnitine supplementation as a potential therapy for suspected hyperammonaemic encephalopathy. The journal of the Royal College of Physicians of Edinburgh, 2019, Volume: 49, Issue:4 Topics: Adult; Anticonvulsants; Blood Chemical Analysis; Carnitine; Cerebral Palsy; Dietary Supplements; Epi	2019
Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy. Nature reviews. Nephrology, 2020, Volume: 16, Issue:8 Topics: Arginine; Carnitine; Child; Child, Preschool; Continuous Renal Replacement Therapy; Delphi Technique	2020
Usefulness of Carnitine Supplementation for the Complications of Liver Cirrhosis. Nutrients, 2020, Jun-29, Volume: 12, Issue:7 Topics: Cardiomyopathies; Carnitine; Dietary Supplements; Humans; Hyperammonemia; Liver; Liver Cirrhosis; Mu	2020
The Importance of the Fatty Acid Transporter L-Carnitine in Non-Alcoholic Fatty Liver Disease (NAFLD). Nutrients, 2020, Jul-22, Volume: 12, Issue:8 Topics: Alanine Transaminase; Aspartate Aminotransferases; Cardiomyopathies; Carnitine; Dietary Supplements;	2020
The role of carnitine in maintenance dialysis therapy. Pediatric nephrology (Berlin, Germany), 2021, Volume: 36, Issue:8 Topics: Adult; Cardiomyopathies; Carnitine; Child; Humans; Hyperammonemia; Kidney Failure, Chronic; Peritone	2021
Significance of l-carnitine for human health. IUBMB life, 2017, Volume: 69, Issue:8 Topics: Cardiomyopathies; Carnitine; Carnitine Acyltransferases; Fatty Acids; Humans; Hyperammonemia; Liver;	2017
Brain carnitine deficiency causes nonsyndromic autism with an extreme male bias: A hypothesis. BioEssays : news and reviews in molecular, cellular and developmental biology, 2017, Volume: 39, Issue:8 Topics: Autistic Disorder; Blood-Brain Barrier; Brain; Cardiomyopathies; Carnitine; Female; Humans; Hyperamm	2017
Sudden valproate-induced hyperammonemia managed with L-carnitine in a medically healthy bipolar patient: Essential review of the literature and case report. Medicine, 2017, Volume: 96, Issue:39 Topics: Adult; Antimanic Agents; Bipolar Disorder; Carnitine; Humans; Hyperammonemia; Male; Substance-Relate	2017
A systematic review about prophylactic L-carnitine administration in parenteral nutrition of extremely preterm infants. Farmacia hospitalaria : organo oficial de expresion cientifica de la Sociedad Espanola de Farmacia Hospitalaria, 2018, 07-01, Volume: 42, Issue:4 Topics: Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Infant; Infant, Extremely Premature; Infant, Ne	2018
Effects of Levocarnitine on Cardiac Function and Renal Anemia in Hemodialysis Patients. Contributions to nephrology, 2018, Volume: 196 Topics: Anemia; Cardiomyopathies; Carnitine; Heart; Hematinics; Humans; Hyperammonemia; Kidney Failure, Chro	2018
Lipid storage myopathies: Current treatments and future directions. Progress in lipid research, 2018, Volume: 72 Topics: Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Lipid Metabolism; Lipid Metabolism, Inborn Erro	2018
Lesson of the month 1: Sodium valproate-induced encephalopathy. Clinical medicine (London, England), 2018, Volume: 18, Issue:5 Topics: Aged; Ammonia; Anticonvulsants; Brain Diseases; Carnitine; Consciousness Disorders; Epilepsy; Humans	2018
Carnitine Profile by Tandem Mass Spectrometry and Dialysis Patients. Contributions to nephrology, 2019, Volume: 198 Topics: Carboxylic Acids; Cardiomyopathies; Carnitine; Fatty Acids; Humans; Hyperammonemia; Muscular Disease	2019
[Valproic acid-induced hyperammonemic encephalopathy in a patient receiving valproic acid monotherapy]. Rinsho shinkeigaku = Clinical neurology, 2019, May-28, Volume: 59, Issue:5 Topics: Aged; Ammonia; Anticonvulsants; Biomarkers; Cardiomyopathies; Carnitine; Consciousness Disorders; Ep	2019
Carnitine deficiency in chronic critical illness. Current opinion in clinical nutrition and metabolic care, 2014, Volume: 17, Issue:2 Topics: Cardiomyopathies; Carnitine; Chronic Disease; Critical Illness; Fatty Acids; Humans; Hyperammonemia;	2014
Disorders of carnitine biosynthesis and transport. Molecular genetics and metabolism, 2015, Volume: 116, Issue:3 Topics: Animals; Autism Spectrum Disorder; Biological Transport; Cardiomyopathies; Carnitine; Fatty Acids; H	2015
[Correlation between serum L-carnitine concentration and neutrophil engraftment in patients treated with cord blood transplantation]. [Rinsho ketsueki] The Japanese journal of clinical hematology, 2016, Volume: 57, Issue:2 Topics: Cardiomyopathies; Carnitine; Cord Blood Stem Cell Transplantation; Fetal Blood; Graft vs Host Diseas	2016
Veterinary clinical nutrition: success stories: an overview. The Proceedings of the Nutrition Society, 2016, Volume: 75, Issue:3 Topics: Animal Nutritional Physiological Phenomena; Animals; Calculi; Cardiomyopathies; Cardiomyopathy, Dila	2016
Primary Carnitine Deficiency and Newborn Screening for Disorders of the Carnitine Cycle. Annals of nutrition & metabolism, 2016, Volume: 68 Suppl 3 Topics: Cardiomyopathies; Carnitine; Deficiency Diseases; Denmark; Dietary Supplements; Genetic Testing; Hum	2016
Historical Perspective on Clinical Trials of Carnitine in Children and Adults. Annals of nutrition & metabolism, 2016, Volume: 68 Suppl 3 Topics: Administration, Intravenous; Adult; Cardiomyopathies; Carnitine; Carnitine Acyltransferases; Child;	2016
Carnitine in the treatment of valproic acid-induced toxicity. Clinical toxicology (Philadelphia, Pa.), 2009, Volume: 47, Issue:2 Topics: Animals; Anticonvulsants; Antidotes; Carnitine; Chemical and Drug Induced Liver Injury; Energy Metab	2009
Current and emerging strategies for treating hepatic encephalopathy. Critical care nursing clinics of North America, 2010, Volume: 22, Issue:3 Topics: Acarbose; Anti-Bacterial Agents; Carnitine; Dyskinesias; Electroencephalography; Flumazenil; GABA Mo	2010
State of the art in muscle lipid diseases. Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology, 2010, Volume: 29, Issue:2 Topics: Acyl-CoA Dehydrogenase, Long-Chain; Cardiomyopathies; Carnitine; Carnitine O-Palmitoyltransferase; H	2010
Levocarnitine for valproic-acid-induced hyperammonemic encephalopathy. American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists, 2012, Jan-01, Volume: 69, Issue:1 Topics: Carnitine; Humans; Hyperammonemia; Neurotoxicity Syndromes; Valproic Acid	2012
Pharmacological and pathophysiological roles of carnitine/organic cation transporters (OCTNs: SLC22A4, SLC22A5 and Slc22a21). Biopharmaceutics & drug disposition, 2013, Volume: 34, Issue:1 Topics: Animals; Cardiomyopathies; Carnitine; Carrier Proteins; Humans; Hyperammonemia; Intestinal Absorptio	2013
Science review: carnitine in the treatment of valproic acid-induced toxicity - what is the evidence? Critical care (London, England), 2005, Oct-05, Volume: 9, Issue:5 Topics: Anticonvulsants; Carnitine; Chemical and Drug Induced Liver Injury; Drug Overdose; Hepatic Encephalo	2005
Valproate-induced hyperammonemic encephalopathy. Acta neurologica Scandinavica, 2006, Volume: 114, Issue:1 Topics: Anticonvulsants; Brain; Brain Edema; Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing); Carnitine	2006
Hyperammonemia due to valproic acid in the psychiatric setting. The American journal of psychiatry, 2007, Volume: 164, Issue:7 Topics: Adult; Aged; Bipolar Disorder; Carnitine; Child; Female; Humans; Hyperammonemia; Liver; Middle Aged;	2007
Antagonizing effect of AP-1 on glucocorticoid induction of urea cycle enzymes: a study of hyperammonemia in carnitine-deficient, juvenile visceral steatosis mice. Molecular genetics and metabolism, 2000, Volume: 71, Issue:4 Topics: Animals; Carbamoyl-Phosphate Synthase (Ammonia); Carnitine; Fatty Acids; Gene Expression Regulation,	2000

Article	Year
Carnitine insufficiency is associated with fatigue during lenvatinib treatment in patients with hepatocellular carcinoma. PloS one, 2020, Volume: 15, Issue:3 Topics: Aged; Aged, 80 and over; Carcinoma, Hepatocellular; Cardiomyopathies; Carnitine; Dietary Supplements	2020
Effects of L-Carnitine Supplementation in Patients Receiving Hemodialysis or Peritoneal Dialysis. Nutrients, 2020, Nov-01, Volume: 12, Issue:11 Topics: Anemia; Cardiomyopathies; Carnitine; Dietary Supplements; Female; Heart; Humans; Hyperammonemia; Jap	2020
L-carnitine supplementation for muscle weakness and fatigue in children with neurofibromatosis type 1: A Phase 2a clinical trial. American journal of medical genetics. Part A, 2021, Volume: 185, Issue:10 Topics: Cardiomyopathies; Carnitine; Child; Dietary Supplements; Fatigue; Female; Humans; Hyperammonemia; Ma	2021
L-Carnitine Improves Skeletal Muscle Fat Oxidation in Primary Carnitine Deficiency. The Journal of clinical endocrinology and metabolism, 2018, 12-01, Volume: 103, Issue:12 Topics: Adult; Calorimetry, Indirect; Carbohydrate Metabolism; Cardiomyopathies; Carnitine; Exercise; Fatty	2018
Efficacy of L-carnitine supplementation for improving lean body mass and physical function in patients on hemodialysis: a randomized controlled trial. European journal of clinical nutrition, 2019, Volume: 73, Issue:2 Topics: Activities of Daily Living; Adult; Aged; Aged, 80 and over; Body Composition; Cardiomyopathies; Carn	2019
Molecular investigation in Chinese patients with primary carnitine deficiency. Molecular genetics & genomic medicine, 2019, Volume: 7, Issue:9 Topics: Adult; Asian People; Cardiomyopathies; Carnitine; China; Female; Humans; Hyperammonemia; INDEL Mutat	2019
Lower carnitine plasma values from malnutrition cancer patients. Journal of gastrointestinal cancer, 2013, Volume: 44, Issue:3 Topics: Adolescent; Adult; Biomarkers; Cardiomyopathies; Carnitine; Case-Control Studies; Female; Follow-Up	2013
Preliminary safety and efficacy of L-carnitine infusion for the treatment of vasopressor-dependent septic shock: a randomized control trial. JPEN. Journal of parenteral and enteral nutrition, 2014, Volume: 38, Issue:6 Topics: Aged; Black or African American; Body Mass Index; Cardiomyopathies; Carnitine; Dose-Response Relatio	2014
The risk of asymptomatic hyperammonemia in children with idiopathic epilepsy treated with valproate: relationship to blood carnitine status. Epilepsy research, 2009, Volume: 86, Issue:1 Topics: Adolescent; Ammonia; Anticonvulsants; Carnitine; Child; Epilepsy; Female; Humans; Hyperammonemia; Ma	2009

Article	Year
General anesthesia with cisatracurium and sevoflurane for a patient with primary carnitine deficiency receiving L-carnitine therapy: A case report. Medicine, 2021, Sep-24, Volume: 100, Issue:38 Topics: Adult; Anesthesia, General; Anesthetics, Inhalation; Atracurium; Cardiomyopathies; Carnitine; Female	2021
3-Methylglutaconic aciduria in carriers of primary carnitine deficiency. European journal of medical genetics, 2021, Volume: 64, Issue:12 Topics: Adult; Cardiomyopathies; Carnitine; Female; Heterozygote; Humans; Hyperammonemia; Infant; Infant, Ne	2021
[Analysis of metabolic profile and genetic variants for newborns with primary carnitine deficiency from Guangxi]. Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2021, Nov-10, Volume: 38, Issue:11 Topics: Cardiomyopathies; Carnitine; China; Humans; Hyperammonemia; Infant, Newborn; Metabolome; Muscular Di	2021
Evaluation of levocarnitine, lactulose, and combination therapy for the treatment of valproic acid-induced hyperammonemia in critically ill patients. Epilepsy research, 2021, Volume: 178 Topics: Carnitine; Cohort Studies; Critical Illness; Humans; Hyperammonemia; Lactulose; Retrospective Studie	2021
Relationship between Carnitine Deficiency and Tyrosine Kinase Inhibitor Use in Patients with Chronic Myeloid Leukemia. Chemotherapy, 2022, Volume: 67, Issue:2 Topics: Cardiomyopathies; Carnitine; Fatigue; Female; Humans; Hyperammonemia; Leukemia, Myelogenous, Chronic	2022
Biochemical and genetic characteristics of patients with primary carnitine deficiency identified through newborn screening. Orphanet journal of rare diseases, 2021, 12-04, Volume: 16, Issue:1 Topics: Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Infant, Newborn; Muscular Diseases; Mutation; N	2021
Association between Levocarnitine Treatment and the Change in Knee Extensor Strength in Patients Undergoing Hemodialysis: A Post-Hoc Analysis of the Osaka Dialysis Complication Study (ODCS). Nutrients, 2022, Jan-14, Volume: 14, Issue:2 Topics: Administration, Intravenous; Aged; Cardiomyopathies; Carnitine; Female; Humans; Hyperammonemia; Knee	2022
Evaluation of the relationship between serum carnitine levels and intradialytic complications in children with kidney failure. Pediatric nephrology (Berlin, Germany), 2022, Volume: 37, Issue:9 Topics: Adolescent; Cardiomyopathies; Carnitine; Child; Humans; Hyperammonemia; Hypotension; Kidney Failure,	2022
Lenvatinib causes reduced expression of carnitine/organic cation transporter 2 and carnitine deficiency in the skeletal muscle of rats. Toxicology letters, 2022, Aug-01, Volume: 366 Topics: Animals; Cardiomyopathies; Carnitine; Hyperammonemia; Muscle, Skeletal; Muscular Diseases; Organic C	2022
Systemic Primary Carnitine Deficiency: A Case Report with Homozygoys SLC22A5 Gene Mutation. Klinische Padiatrie, 2022, Volume: 234, Issue:4 Topics: Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Muscular Diseases; Mutation; Solute Carrier Fam	2022
Rare case of primary carnitine deficiency presenting as acute liver failure. BMJ case reports, 2022, Jul-19, Volume: 15, Issue:7 Topics: Adult; Cardiomyopathies; Carnitine; Child; Child, Preschool; Fatty Acids; Humans; Hyperammonemia; Li	2022
Carnitine deficiency, hearing loss and hydrochlorothiazide-induced diabetes mellitus associated with the recurrent p.Trp85Arg variant in HNF4A. American journal of medical genetics. Part A, 2022, Volume: 188, Issue:10 Topics: Cardiomyopathies; Carnitine; Diabetes Mellitus, Type 2; Hearing Loss; Hepatocyte Nuclear Factor 4; H	2022
Primary Carnitine Deficiency as a Treatable Cause of Heart Failure in Young Patients. Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir, 2022, Volume: 50, Issue:7 Topics: Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Heart Failure; Humans; Hyperammonemia; Male; M	2022
[Analysis of blood carnitine profile and SLC22A5 gene variants in 17 neonates with Primary carnitine deficiency]. Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2023, Feb-10, Volume: 40, Issue:2 Topics: Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Infant, Newborn; Muscular Diseases; Solute Carr	2023
Quantitative systems pharmacology Model to characterize valproic acid-induced hyperammonemia and the effect of L-carnitine supplementation. European journal of pharmaceutical sciences : official journal of the European Federation for Pharmaceutical Sciences, 2023, Apr-01, Volume: 183 Topics: Ammonia; Anticonvulsants; Carnitine; Dietary Supplements; Drug Overdose; Drug-Related Side Effects a	2023
Valproate induced carnitine deficiency and hyperammonaemia. Clinical medicine (London, England), 2023, Volume: 23, Issue:4 Topics: Amino Acid Metabolism, Inborn Errors; Anticonvulsants; Carnitine; Epilepsy; Humans; Hyperammonemia;	2023
Molecular Autopsy Implicates Primary Carnitine Deficiency in Sudden Unexplained Death and Reversible Short QT Syndrome. The Canadian journal of cardiology, 2019, Volume: 35, Issue:9 Topics: Adult; Autopsy; Cardiomyopathies; Carnitine; Death, Sudden, Cardiac; DNA; Fatal Outcome; Female; Gen	2019
[Clinical and genetic characteristics of primary carnitine deficiency identified by neonatal screening]. Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2019, Dec-10, Volume: 36, Issue:12 Topics: Cardiomyopathies; Carnitine; China; Female; Humans; Hyperammonemia; Infant, Newborn; Muscular Diseas	2019
Slc22a5 haploinsufficiency does not aggravate the phenotype of the long-chain acyl-CoA dehydrogenase KO mouse. Journal of inherited metabolic disease, 2020, Volume: 43, Issue:3 Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Cardiomyopathies; Carnitine; Disease Models, Animal; Fa	2020
Carnitine uptake defect due to a 5'UTR mutation in a pedigree with false positives and false negatives on Newborn screening. Molecular genetics and metabolism, 2020, Volume: 129, Issue:3 Topics: 5' Untranslated Regions; Actins; Biological Transport, Active; Cardiomyopathies; Carnitine; Cells, C	2020
Primary carnitine deficiency - diagnosis after heart transplantation: better late than never! Orphanet journal of rare diseases, 2020, 04-10, Volume: 15, Issue:1 Topics: Cardiomyopathies; Carnitine; Child; Heart Transplantation; Humans; Hyperammonemia; Infant, Newborn;	2020
Screening 3.4 million newborns for primary carnitine deficiency in Zhejiang Province, China. Clinica chimica acta; international journal of clinical chemistry, 2020, Volume: 507 Topics: Cardiomyopathies; Carnitine; China; Humans; Hyperammonemia; Infant, Newborn; Muscular Diseases; Neon	2020
Genetic characteristics and follow-up of patients with fatty acid β-oxidation disorders through expanded newborn screening in a Northern Chinese population. Journal of pediatric endocrinology & metabolism : JPEM, 2020, May-24, Volume: 33, Issue:6 Topics: Cardiomyopathies; Carnitine; Carnitine O-Palmitoyltransferase; China; Congenital Bone Marrow Failure	2020
Ventricular Fibrillation Caused by Primary Carnitine Deficiency. The Journal of emergency medicine, 2020, Volume: 59, Issue:1 Topics: Adult; Cardiomyopathies; Cardiopulmonary Resuscitation; Carnitine; Electric Countershock; Female; Hu	2020
Metabolic Serendipities of Expanded Newborn Screening. Genes, 2020, 08-29, Volume: 11, Issue:9 Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Cardiomyopathies; Carnitine; Dried	2020
Combined primary carnitine deficiency with neonatal intrahepatic cholestasis caused by citrin deficiency in a Chinese newborn. BMC pediatrics, 2020, 10-13, Volume: 20, Issue:1 Topics: Cardiomyopathies; Carnitine; China; Cholestasis, Intrahepatic; Citrullinemia; Humans; Hyperammonemia	2020
Starting stiripentol in adults with Dravet syndrome? Watch for ammonia and carnitine. Epilepsia, 2020, Volume: 61, Issue:11 Topics: Adult; Ammonia; Anticonvulsants; Carnitine; Cohort Studies; Dioxolanes; Epilepsies, Myoclonic; Femal	2020
Long-term survival of a patient with acute neonatal-onset metabolic encephalopathy with carbamoyl phosphate synthetase 1 deficiency. European review for medical and pharmacological sciences, 2020, Volume: 24, Issue:19 Topics: Arginine; Brain Diseases, Metabolic; Carbamoyl-Phosphate Synthase I Deficiency Disease; Carnitine; F	2020
Newborn screening for primary carnitine deficiency in Quanzhou, China. Clinica chimica acta; international journal of clinical chemistry, 2021, Volume: 512 Topics: Cardiomyopathies; Carnitine; Child; China; Female; Humans; Hyperammonemia; Infant, Newborn; Muscular	2021
Biochemical and genetic characteristics of 40 neonates with carnitine deficiency. Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences, 2020, Oct-28, Volume: 45, Issue:10 Topics: Cardiomyopathies; Carnitine; Child; Humans; Hyperammonemia; Infant, Newborn; Muscular Diseases; Muta	2020
Role of carnitine in regulation of blood pressure (MAP/SBP) and gene expression of cardiac hypertrophy markers (α/β-MHC) during insulin-induced hypoglycaemia: Role of oxidative stress. Clinical and experimental pharmacology & physiology, 2021, Volume: 48, Issue:4 Topics: Animals; Blood Pressure; Cardiomyopathies; Carnitine; Hyperammonemia; Hypertension; Muscular Disease	2021
Hyperammonemia, Lactic Acidosis, and Arrhythmia in a Newborn. Clinical chemistry, 2021, 01-08, Volume: 67, Issue:1 Topics: Acidosis, Lactic; Arrhythmias, Cardiac; Carnitine; Female; Humans; Hyperammonemia; Infant, Newborn;	2021
Effectiveness of monitoring free carnitine levels for L-carnitine supplementation in hemodialysis patients to maintain carnitine sufficiency and nutritional factors. Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy, 2021, Volume: 25, Issue:5 Topics: Aged; Cardiomyopathies; Carnitine; Dietary Supplements; Female; Humans; Hyperammonemia; Kidney Failu	2021
Gene spectrum and clinical traits of 10 patients with primary carnitine deficiency. Molecular genetics & genomic medicine, 2021, Volume: 9, Issue:2 Topics: Adult; Cardiomyopathies; Carnitine; Female; Gene Frequency; Humans; Hyperammonemia; Infant; Male; Mu	2021
Hypoglycemia Due to Acquired Carnitine Deficiency in a Pediatric Patient Receiving Chemotherapy. Journal of pediatric hematology/oncology, 2022, Mar-01, Volume: 44, Issue:2 Topics: Cardiomyopathies; Carnitine; Child; Humans; Hyperammonemia; Hypoglycemia; Infant; Male; Malnutrition	2022
Increased detection of primary carnitine deficiency through second-tier newborn genetic screening. Orphanet journal of rare diseases, 2021, 03-23, Volume: 16, Issue:1 Topics: Cardiomyopathies; Carnitine; Genetic Testing; Humans; Hyperammonemia; Infant, Newborn; Muscular Dise	2021
Managing valproic acid toxicity-related hyperammonaemia: an unpredicted course. BMJ case reports, 2021, Apr-19, Volume: 14, Issue:4 Topics: Adult; Brain Diseases; Carnitine; Drug Overdose; Female; Humans; Hyperammonemia; Valproic Acid; Youn	2021
Altered mitochondrial metabolism in peripheral blood cells from patients with inborn errors of β-oxidation. Clinical and translational science, 2022, Volume: 15, Issue:1 Topics: Acyl-CoA Dehydrogenase, Long-Chain; Cardiomyopathies; Carnitine; Child; Child, Preschool; Female; Ge	2022
L-Carnitine supplementation to reverse hyperammonemia in a patient undergoing chronic valproic acid treatment: A case report. The Journal of international medical research, 2017, Volume: 45, Issue:3 Topics: Adult; Anticonvulsants; Carnitine; Female; Humans; Hyperammonemia; Seizures; Valproic Acid	2017
Biochemical characteristics of newborns with carnitine transporter defect identified by newborn screening in California. Molecular genetics and metabolism, 2017, Volume: 122, Issue:3 Topics: California; Cardiomyopathies; Carnitine; Dried Blood Spot Testing; False Positive Reactions; Female;	2017
Clinical features and genotyping of patients with primary carnitine deficiency identified by newborn screening. Journal of pediatric endocrinology & metabolism : JPEM, 2017, Aug-28, Volume: 30, Issue:8 Topics: Alleles; Cardiomyopathies; Carnitine; Female; Genotype; Humans; Hyperammonemia; Infant, Newborn; Mal	2017
Gene and environment interactions in autism risk: Reflections on the carnitine deficiency hypothesis by Beaudet (Comment on DOI 10.1002/bies.201700012). BioEssays : news and reviews in molecular, cellular and developmental biology, 2017, Volume: 39, Issue:10 Topics: Autistic Disorder; Carnitine; Gene-Environment Interaction; Humans; Hyperammonemia; Muscular Disease	2017
Functional and molecular studies in primary carnitine deficiency. Human mutation, 2017, Volume: 38, Issue:12 Topics: Amino Acid Substitution; Animals; Biological Transport; Cardiomyopathies; Carnitine; CHO Cells; Cric	2017
[Screening for fatty acid oxidation disorders of newborns in Zhejiang province:prevalence, outcome and follow-up]. Zhejiang da xue xue bao. Yi xue ban = Journal of Zhejiang University. Medical sciences, 2017, 05-25, Volume: 46, Issue:3 Topics: Acyl-CoA Dehydrogenase; Cardiomyopathies; Carnitine; China; Follow-Up Studies; Humans; Hyperammonemi	2017
Infant with hepatomegaly and hypoglycemia: A setting for fatty acid oxidation defects. Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology, 2017, Volume: 36, Issue:5 Topics: Cardiomyopathies; Carnitine; Chromatography, Gas; DNA Mutational Analysis; Early Diagnosis; Hepatome	2017
[Genetic diagnosis of 10 neonates with primary carnitine deficiency]. Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics, 2017, Volume: 19, Issue:11 Topics: Cardiomyopathies; Carnitine; Computational Biology; Genetic Counseling; Humans; Hyperammonemia; Infa	2017
Similarities between acylcarnitine profiles in large for gestational age newborns and obesity. Scientific reports, 2017, 11-24, Volume: 7, Issue:1 Topics: Cardiomyopathies; Carnitine; Female; Gestational Age; Humans; Hyperammonemia; Infant, Newborn; Infan	2017
Dilated Cardiomyopathy With Short QT Interval Suggests Primary Carnitine Deficiency. Revista espanola de cardiologia (English ed.), 2018, Volume: 71, Issue:12 Topics: Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Child; Echocardiography; Electrocardiography;	2018
[Valproate-induced hyperammonemic encephalopathy]. Revista de neurologia, 2017, Dec-16, Volume: 65, Issue:12 Topics: Acetamides; Anticonvulsants; Brain Diseases, Metabolic; Carnitine; Coma; Drug Therapy, Combination;	2017
Multifactorial non-cirrhotic hyperammonaemic encephalopathy. BMJ case reports, 2018, Mar-09, Volume: 2018 Topics: Acetylcysteine; Aftercare; Anti-Bacterial Agents; Bacteremia; Carnitine; Female; Free Radical Scaven	2018
Carnitine levels and mutations in the SLC22A5 gene in Faroes patients with Parkinson's disease. Neuroscience letters, 2018, 05-14, Volume: 675 Topics: Adult; Aged; Aged, 80 and over; Cardiomyopathies; Carnitine; Case-Control Studies; Denmark; Female;	2018
SLC22A5 Mutations in a Patient With Systemic Primary Carnitine Deficiency and Cleft Palate-Successful Perioperative Management. The Journal of craniofacial surgery, 2018, Volume: 29, Issue:6 Topics: Cardiomyopathies; Carnitine; Cleft Palate; Fasting; Humans; Hyperammonemia; Infant; Male; Muscular D	2018
Primary carnitine deficiency in a 57-year-old patient with recurrent exertional rhabdomyolysis. BMJ case reports, 2018, Jun-11, Volume: 2018 Topics: Cardiomyopathies; Carnitine; Genetic Testing; Humans; Hyperammonemia; Lipid Metabolism; Male; Middle	2018
L-carnitine Improved the Cardiac Function via the Effect on Myocardial Fatty Acid Metabolism in a Hemodialysis Patient. Internal medicine (Tokyo, Japan), 2018, Dec-15, Volume: 57, Issue:24 Topics: Administration, Intravenous; Cardiomyopathies; Carnitine; Fatty Acids; Female; Heart; Humans; Hypera	2018
Follow-up of fatty acid β-oxidation disorders in expanded newborn screening era. European journal of pediatrics, 2019, Volume: 178, Issue:3 Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Amino Acid Metabolism, Inborn Errors; Ca	2019
Hyperammonaemia in Neonates and Young Children: Potential Metabolic Causes, Diagnostic Approaches and Clinical Consequences Irish medical journal, 2019, 01-15, Volume: 112, Issue:1 Topics: Ammonia; Arginine; Biomarkers; Brain Diseases, Metabolic, Inborn; Carnitine; Critical Illness; Diet,	2019
A newborn with seizures born to a mother diagnosed with primary carnitine deficiency. BMC pediatrics, 2019, 03-18, Volume: 19, Issue:1 Topics: Brain; Cardiomyopathies; Carnitine; Electroencephalography; Female; Fetal Diseases; Humans; Hyperamm	2019
Intravenous use of valproic acid in status epilepticus is associated with high risk of hyperammonemia. Seizure, 2019, Volume: 69 Topics: Adult; Aged; Aged, 80 and over; Anticonvulsants; Carnitine; Female; Humans; Hyperammonemia; Injectio	2019
A mutation creating an upstream translation initiation codon in SLC22A5 5'UTR is a frequent cause of primary carnitine deficiency. Human mutation, 2019, Volume: 40, Issue:10 Topics: 5' Untranslated Regions; Alleles; Amino Acid Sequence; Base Sequence; Biological Transport; Cardiomy	2019
Primary carnitine deficiency with severe acute hepatitis following rotavirus gastroenteritis. Journal of infection and chemotherapy : official journal of the Japan Society of Chemotherapy, 2019, Volume: 25, Issue:11 Topics: Acute Disease; Cardiomyopathies; Carnitine; Female; Gastroenteritis; Hepatitis; Humans; Hyperammonem	2019
Relapsing Hypoglycemia Associated with Hypocarnitinemia Following Treatment with Cefcapene Pivoxil in an Elderly Man. Internal medicine (Tokyo, Japan), 2019, Oct-01, Volume: 58, Issue:19 Topics: Administration, Oral; Aged, 80 and over; Blood Glucose; Cardiomyopathies; Carnitine; Cephalosporins;	2019
[SLC22A5 gene mutation analysis and prenatal diagnosis for a family with primary carnitine deficiency]. Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2019, Jul-10, Volume: 36, Issue:7 Topics: Cardiomyopathies; Carnitine; Child, Preschool; DNA Mutational Analysis; Female; Humans; Hyperammonem	2019
Increased risk of sudden death in untreated primary carnitine deficiency. Journal of inherited metabolic disease, 2020, Volume: 43, Issue:2 Topics: Adolescent; Adult; Arrhythmias, Cardiac; Cardiomyopathies; Carnitine; Child; Child, Preschool; Death	2020
Cardiac magnetic resonance findings in a case of carnitine deficiency. Texas Heart Institute journal, 2013, Volume: 40, Issue:1 Topics: Arrhythmias, Cardiac; Cardiomyopathies; Carnitine; Contrast Media; Death, Sudden, Cardiac; Defibrill	2013
Carnitine uptake defect (primary carnitine deficiency): risk in genotype-phenotype correlation. Human mutation, 2013, Volume: 34, Issue:4 Topics: Animals; Cardiomyopathies; Carnitine; Female; Humans; Hyperammonemia; Muscular Diseases; Organic Cat	2013
Response to chen et Al.: carnitine uptake defect (primary carnitine deficiency): risk in genotype-phenotype correlation. Human mutation, 2013, Volume: 34, Issue:4 Topics: Animals; Cardiomyopathies; Carnitine; Female; Humans; Hyperammonemia; Muscular Diseases; Organic Cat	2013
Successful early management of a female patient with a metabolic stroke due to ornithine transcarbamylase deficiency. Pediatric emergency care, 2013, Volume: 29, Issue:5 Topics: Adolescent; Arginine; Brain; Carnitine; Case Management; Citrulline; Coma; Combined Modality Therapy	2013
Carnitine levels in 26,462 individuals from the nationwide screening program for primary carnitine deficiency in the Faroe Islands. Journal of inherited metabolic disease, 2014, Volume: 37, Issue:2 Topics: Adolescent; Adult; Aged; Aged, 80 and over; Cardiomyopathies; Carnitine; Child; Child, Preschool; De	2014
Use of carglumic acid in the treatment of hyperammonaemia during metabolic decompensation of patients with propionic acidaemia. Molecular genetics and metabolism, 2013, Volume: 109, Issue:4 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Carnitine; Child, Preschool; Female; Glutamates; Huma	2013
Newborn screening for medium-chain acyl-CoA dehydrogenase deficiency: regional experience and high incidence of carnitine deficiency. Orphanet journal of rare diseases, 2013, Jul-10, Volume: 8 Topics: Acyl-CoA Dehydrogenase; Cardiomyopathies; Carnitine; Dietary Supplements; Female; Genetic Associatio	2013
Primary Carnitine deficiency in the Faroe Islands: health and cardiac status in 76 adult patients diagnosed by screening. Journal of inherited metabolic disease, 2014, Volume: 37, Issue:2 Topics: Adolescent; Adult; Arrhythmias, Cardiac; Cardiomyopathies; Carnitine; Denmark; Dietary Supplements;	2014
Exacerbation of mood symptoms associated to primary and secondary carnitine deficiency: a case report. Boletin de la Asociacion Medica de Puerto Rico, 2013, Volume: 105, Issue:3 Topics: Adult; Antidepressive Agents; Antimanic Agents; Attention Deficit Disorder with Hyperactivity; Benzo	2013
[The association of hypocarnitinemia with enteral diets and antiepileptic drugs in children and adults with severe physical and mental disabilities]. No to hattatsu = Brain and development, 2013, Volume: 45, Issue:6 Topics: Adolescent; Adult; Anticonvulsants; Carnitine; Child; Child, Preschool; Enteral Nutrition; Female; H	2013
Valproate-induced hyperammonemia in juvenile ceroid lipofuscinosis (Batten disease). Seizure, 2014, Volume: 23, Issue:6 Topics: Adolescent; Anticonvulsants; Brain Diseases; Carnitine; Drug Therapy, Combination; Female; Humans; H	2014
Carnitine and cardiac dysfunction in childhood cancer survivors treated with anthracyclines. Cancer epidemiology, biomarkers & prevention : a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive Oncology, 2014, Volume: 23, Issue:6 Topics: Adolescent; Adult; Anthracyclines; Cardiomyopathies; Cardiovascular Diseases; Carnitine; Child; Fema	2014
Primary carnitine deficiency cardiomyopathy. International journal of cardiology, 2014, Jun-01, Volume: 174, Issue:1 Topics: Adolescent; Cardiomyopathies; Carnitine; Child; Female; Humans; Hyperammonemia; Infant; Male; Muscul	2014
Emerging trends in management of propionic acidemia. Arquivos brasileiros de endocrinologia e metabologia, 2014, Volume: 58, Issue:3 Topics: Adolescent; Anti-Infective Agents; Biotin; Carnitine; Child; Child, Preschool; Diet, Protein-Restric	2014
Hyperammonemia associated with valproic acid concentrations. BioMed research international, 2014, Volume: 2014 Topics: Adolescent; Adult; Ammonia; Bipolar Disorder; Carnitine; Child; Dietary Supplements; Dose-Response R	2014
Analysis of genetic mutations in Chinese patients with systemic primary carnitine deficiency. European journal of medical genetics, 2014, Volume: 57, Issue:10 Topics: Asian People; Cardiomyopathies; Carnitine; Child; Child, Preschool; DNA Mutational Analysis; Exons;	2014
[Genetics and pedigree analysis of primary carnitine deficiency cardiomyopathy in 6 cases]. Zhonghua er ke za zhi = Chinese journal of pediatrics, 2014, Volume: 52, Issue:7 Topics: Adolescent; Base Sequence; Cardiomyopathies; Carnitine; Child; Child, Preschool; DNA Mutational Anal	2014
[Primary carnitine deficiency in an infant]. Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics, 2014, Volume: 16, Issue:9 Topics: Cardiomyopathies; Carnitine; Female; Humans; Hyperammonemia; Infant; Muscular Diseases	2014
Ammonia encephalopathy and awake craniotomy for brain language mapping: cause of failed awake craniotomy. Revista espanola de anestesiologia y reanimacion, 2015, Volume: 62, Issue:5 Topics: Anesthesia, General; Anesthesia, Local; Anticonvulsants; Aphasia; Benzodiazepines; Brain Diseases; B	2015
Carnitine deficiency: Risk factors and incidence in children with epilepsy. Brain & development, 2015, Volume: 37, Issue:8 Topics: Adolescent; Anticonvulsants; Cardiomyopathies; Carnitine; Case-Control Studies; Child; Child, Presch	2015
3-HMG Coenzyme A Lyase Deficiency: Macrocephaly and Left Ventricular Noncompaction with a Novel Mutation. Indian journal of pediatrics, 2015, Volume: 82, Issue:7 Topics: Acetyl-CoA C-Acetyltransferase; Amino Acid Metabolism, Inborn Errors; Carnitine; Diet, Fat-Restricte	2015
Carnitine is associated with fatigue following chemoradiotherapy for head and neck cancer. Acta oto-laryngologica, 2015, Volume: 135, Issue:8 Topics: Animals; Cardiomyopathies; Carnitine; Chemoradiotherapy; Female; Head and Neck Neoplasms; Humans; Hy	2015
Importance of molecular diagnosis in the accurate diagnosis of systemic carnitine deficiency. Journal of genetics, 2015, Volume: 94, Issue:1 Topics: Base Sequence; Cardiomyopathies; Carnitine; Diagnosis, Differential; DNA Mutational Analysis; Female	2015
Improvement of regressive autism symptoms in a child with TMLHE deficiency following carnitine supplementation. American journal of medical genetics. Part A, 2015, Volume: 167A, Issue:9 Topics: Autism Spectrum Disorder; Autistic Disorder; Cardiomyopathies; Carnitine; Child Development Disorder	2015
Primary systemic carnitine deficiency: a Turkish case with a novel homozygous SLC22A5 mutation and 14 years follow-up. Journal of pediatric endocrinology & metabolism : JPEM, 2015, Volume: 28, Issue:9-10 Topics: Adolescent; Cardiomyopathies; Carnitine; Child; Child, Preschool; Hormone Replacement Therapy; Human	2015
Carnitine deficiency induces a short QT syndrome. Heart rhythm, 2016, Volume: 13, Issue:1 Topics: Adult; Animals; Arrhythmias, Cardiac; Cardiomyopathies; Carnitine; Child, Preschool; Echocardiograph	2016
Carnitine insufficiency in children with inborn errors of metabolism: prevalence and treatment efficacy. Journal of pediatric endocrinology & metabolism : JPEM, 2015, Nov-01, Volume: 28, Issue:11-12 Topics: Cardiomyopathies; Carnitine; Child; Child, Preschool; Female; Humans; Hyperammonemia; Male; Metaboli	2015
[Genetic and prenatal diagnosis for a Chinese family with primary carnitine deficiency]. Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2015, Volume: 32, Issue:4 Topics: Adult; Asian People; Base Sequence; Cardiomyopathies; Carnitine; China; Exons; Female; Genotype; Hum	2015
Carnitine deficiency in children receiving continuous renal replacement therapy. Hemodialysis international. International Symposium on Home Hemodialysis, 2016, Volume: 20, Issue:1 Topics: Acute Kidney Injury; Adolescent; Adult; Cardiomyopathies; Carnitine; Child; Child, Preschool; Female	2016
Hyperammonemia Syndrome After Lung Transplantation: A Single Center Experience. Transplantation, 2016, Volume: 100, Issue:3 Topics: Aged; Ammonia; Arginine; Biomarkers; Carnitine; Combined Modality Therapy; Decontamination; Female;	2016
Reversible weakness and encephalopathy while on long-term valproate treatment due to carnitine deficiency. BMJ case reports, 2015, Sep-02, Volume: 2015 Topics: Adult; Antimanic Agents; Brain Diseases; Cardiomyopathies; Carnitine; Female; Humans; Hyperammonemia	2015
Elevation of pivaloylcarnitine by sivelestat sodium in two children. Molecular genetics and metabolism, 2015, Volume: 116, Issue:3 Topics: Amino Acid Metabolism, Inborn Errors; Cardiomyopathies; Carnitine; Child, Preschool; Chromatography,	2015
A Moderate Carnitine Deficiency Exacerbates Isoproterenol-Induced Myocardial Injury in Rats. Cardiovascular drugs and therapy, 2016, Volume: 30, Issue:2 Topics: Animals; Cardiomyopathies; Carnitine; Diastole; Heart; Heart Diseases; Heart Rate; Hyperammonemia; I	2016
Usefulness of Levocarnitine and/or Branched-Chain Amino Acids during Invasive Treatment for Hepatocellular Carcinoma. Journal of nutritional science and vitaminology, 2015, Volume: 61, Issue:6 Topics: Ablation Techniques; Aged; Aged, 80 and over; Albumins; Amino Acids, Branched-Chain; Ammonia; Carcin	2015
Hemodialysis Associated with Severe and Unpredictable Hypoglycemia. Internal medicine (Tokyo, Japan), 2016, Volume: 55, Issue:4 Topics: Aged; Autopsy; Blood Glucose; Cardiomyopathies; Carnitine; Diabetes Mellitus, Type 2; Fatal Outcome;	2016
Lipolysis and lipophagy in lipid storage myopathies. Biochimica et biophysica acta, 2016, Volume: 1862, Issue:7 Topics: Adolescent; Adult; Aged; Autophagy; Cardiomyopathies; Carnitine; Carnitine O-Palmitoyltransferase; C	2016
Primary Carnitine Deficiency - A Rare Treatable Cause of Cardiomyopathy and Massive Hepatomegaly. Indian journal of pediatrics, 2017, Volume: 84, Issue:1 Topics: Cardiomyopathies; Carnitine; Hepatomegaly; Humans; Hyperammonemia; Infant; Male; Muscular Diseases	2017
Dilated Cardiomyopathy as the Only Clinical Manifestation of Carnitine Transporter Deficiency. Indian journal of pediatrics, 2017, Volume: 84, Issue:3 Topics: Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Child, Preschool; Diagnosis, Differential; Fem	2017
Round Table Discussion. Annals of nutrition & metabolism, 2016, Volume: 68 Suppl 3 Topics: Adolescent; Autistic Disorder; Biomedical Research; Cardiomyopathies; Carnitine; Child; Congresses a	2016
Long-term outcome of expanded newborn screening at Boston children's hospital: benefits and challenges in defining true disease. Journal of inherited metabolic disease, 2017, Volume: 40, Issue:2 Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Boston; Cardiomyopath	2017
L-Arginine in the treatment of valproate overdose - five clinical cases. Clinical toxicology (Philadelphia, Pa.), 2017, Volume: 55, Issue:4 Topics: Acyl Coenzyme A; Adult; Amino-Acid N-Acetyltransferase; Ammonia; Arginine; Carnitine; Coma; Drug Ove	2017
[Mutational analysis of SLC22A5 gene in eight patients with systemic primary carnitine deficiency]. Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2017, Feb-10, Volume: 34, Issue:1 Topics: Adult; Amino Acid Sequence; Base Sequence; Cardiomyopathies; Carnitine; DNA Mutational Analysis; Fem	2017
Outpatient treatment of propionic acidemia-associated hyperammonemia with N-carbamoyl-L-glutamate in an infant. Clinical therapeutics, 2010, Volume: 32, Issue:4 Topics: Carnitine; Diet; Female; Glutamates; Humans; Hyperammonemia; Infant; Propionic Acidemia	2010
Glycosylation of the OCTN2 carnitine transporter: study of natural mutations identified in patients with primary carnitine deficiency. Biochimica et biophysica acta, 2011, Volume: 1812, Issue:3 Topics: Amino Acid Substitution; Animals; Biological Transport; Blotting, Western; Cardiomyopathies; Carniti	2011
Setting up an emergency stock for metabolic diseases. La Clinica terapeutica, 2010, Volume: 161, Issue:6 Topics: Acidosis; Amino Acids; Carnitine; Chelating Agents; Disease Management; Electronic Prescribing; Emer	2010
Induction of PDK4 in the heart muscle of JVS mice, an animal model of systemic carnitine deficiency, does not appear to reduce glucose utilization by the heart. Molecular genetics and metabolism, 2011, Volume: 102, Issue:3 Topics: Animals; Cardiomyopathies; Carnitine; Disease Models, Animal; Female; Glucose; Hyperammonemia; Male;	2011
The consequences of valproate overdose. Clinical chemistry, 2011, Volume: 57, Issue:9 Topics: Acid-Base Equilibrium; Acidosis, Lactic; Adult; Anticonvulsants; Carnitine; Drug Overdose; Epilepsy;	2011
Carnitine supplementation for valproate-related hyperammonemia to maintain therapeutic valproate level. Journal of clinical psychopharmacology, 2011, Volume: 31, Issue:5 Topics: Adult; Anticonvulsants; Antisocial Personality Disorder; Carnitine; Humans; Hyperammonemia; Male; Sc	2011
Genotype-phenotype correlation in primary carnitine deficiency. Human mutation, 2012, Volume: 33, Issue:1 Topics: Adult; Animals; Asymptomatic Diseases; Biological Transport; Cardiomyopathies; Carnitine; Child; Chi	2012
Primary carnitine deficiency and sudden death: in vivo evidence of myocardial lipid peroxidation and sulfonylation of sarcoendoplasmic reticulum calcium ATPase 2. Cardiology, 2011, Volume: 120, Issue:1 Topics: Cardiomyopathies; Carnitine; Death, Sudden, Cardiac; Defibrillators, Implantable; Electrocardiograph	2011
Thirty-year follow-up of carnitine supplementation in two siblings with hypertrophic cardiomyopathy caused by primary systemic carnitine deficiency. International journal of cardiology, 2012, Aug-09, Volume: 159, Issue:1 Topics: Adult; Cardiomyopathies; Cardiomyopathy, Hypertrophic; Carnitine; Dietary Supplements; Follow-Up Stu	2012
Acquired encephalopathy associated with carnitine deficiency after cefditoren pivoxil administration. Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2012, Volume: 33, Issue:6 Topics: Anti-Bacterial Agents; Brain Diseases, Metabolic; Cardiomyopathies; Carnitine; Cephalosporins; Diagn	2012
[A cause of dilated cardiomyopathy in a child: primary carnitine deficiency]. Annales de cardiologie et d'angeiologie, 2014, Volume: 63, Issue:2 Topics: Biomarkers; Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Child, Preschool; Dyspnea; Humans;	2014
Primary carnitine deficiency and pivalic acid exposure causing encephalopathy and fatal cardiac events. Journal of inherited metabolic disease, 2013, Volume: 36, Issue:1 Topics: Adult; Anti-Bacterial Agents; Arrhythmias, Cardiac; Cardiomyopathies; Carnitine; Child, Preschool; D	2013
Primary carnitine deficiency dilated cardiomyopathy: 28 years follow-up. International journal of cardiology, 2013, Jan-10, Volume: 162, Issue:2 Topics: Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Child, Preschool; Echocardiography; Female; Fo	2013
Effect of hemodialysis session on the dynamics of carnitine ester profile changes in L-carnitine pretreated end-stage renal disease patients. International urology and nephrology, 2013, Volume: 45, Issue:3 Topics: Adult; Aged; Aged, 80 and over; Cardiomyopathies; Carnitine; Dietary Supplements; Female; Follow-Up	2013
[Primary carnitine deficiency in 17 patients: diagnosis, treatment and follow up]. Zhonghua er ke za zhi = Chinese journal of pediatrics, 2012, Volume: 50, Issue:6 Topics: Cardiomyopathies; Carnitine; Child, Preschool; DNA Mutational Analysis; Female; Follow-Up Studies; H	2012
Successful perioperative management of a patient with primary systemic carnitine deficiency: a case report. Journal of anesthesia, 2013, Volume: 27, Issue:1 Topics: Cardiomyopathies; Carnitine; Cleft Lip; Cleft Palate; Energy Metabolism; Humans; Hyperammonemia; Inf	2013
[L-carnitine in valproic acid-induced hyperammonemia]. Medicina intensiva, 2014, Volume: 38, Issue:2 Topics: Adult; Anticonvulsants; Carnitine; Humans; Hyperammonemia; Male; Valproic Acid; Vitamin B Complex	2014
SLC22A5 mutations in a patient with systemic primary carnitine deficiency: the first Korean case confirmed by biochemical and molecular investigation. Annals of clinical and laboratory science, 2012,Fall, Volume: 42, Issue:4 Topics: Ammonia; Base Sequence; Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Infant, Newborn; Lactic	2012
Intracellular in vitro probe acylcarnitine assay for identifying deficiencies of carnitine transporter and carnitine palmitoyltransferase-1. Analytical and bioanalytical chemistry, 2013, Volume: 405, Issue:4 Topics: Biological Transport; Cardiomyopathies; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyl	2013
[Primary carnitine deficiency in a neonate]. Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics, 2013, Volume: 15, Issue:1 Topics: Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Infant, Newborn; Male; Muscular Diseases	2013
Primary carnitine deficiency: novel mutations and insights into the cardiac phenotype. Clinical genetics, 2014, Volume: 85, Issue:2 Topics: Base Sequence; Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Child; Child, Preschool; Codon,	2014
Hyperammonemia in carnitine-deficient adult JVS mice used by starvation. Metabolic brain disease, 2002, Volume: 17, Issue:4 Topics: Aging; Amino Acids; Ammonia; Animals; Animals, Newborn; Carbamoyl-Phosphate Synthase (Ammonia); Carn	2002
The effect of zinc and potassium in the nutrition of Tenebrio molitor, with observations on the expression of a carnitine deficiency. The Journal of nutrition, 1958, Jul-10, Volume: 65, Issue:3 Topics: Animals; Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Muscular Diseases; Potassium; Tenebrio	1958
Relative carnitine deficiency in autism. Journal of autism and developmental disorders, 2004, Volume: 34, Issue:6 Topics: Adolescent; Alanine; Autistic Disorder; Carnitine; Child; Child, Preschool; Diagnostic and Statistic	2004
Ammonia-mediated LTP inhibition: effects of NMDA receptor antagonists and L-carnitine. Neurobiology of disease, 2005, Volume: 20, Issue:2 Topics: Adenosine Triphosphate; Ammonia; Animals; Carnitine; Cell Respiration; Dose-Response Relationship, D	2005
Acute valproate poisoning: pharmacokinetics, alteration in fatty acid metabolism, and changes during therapy. Journal of clinical psychopharmacology, 2005, Volume: 25, Issue:4 Topics: Anticonvulsants; Brain Edema; Carnitine; Drug Overdose; Fatty Acids; Female; Humans; Hyperammonemia;	2005
Taurine rescues hippocampal long-term potentiation from ammonia-induced impairment. Neurobiology of disease, 2006, Volume: 23, Issue:3 Topics: Ammonium Chloride; Animals; Carnitine; Energy Metabolism; Hepatic Encephalopathy; Hippocampus; Hyper	2006
Prospective treatment in carnitine-acylcarnitine translocase deficiency. Journal of inherited metabolic disease, 2007, Volume: 30, Issue:5 Topics: Carnitine; Carnitine Acyltransferases; Dicarboxylic Acids; Diet, Fat-Restricted; Dietary Supplements	2007
Two cases of valproic acid poisoning treated with L-carnitine. Human & experimental toxicology, 2007, Volume: 26, Issue:12 Topics: Adolescent; Adult; Anticonvulsants; Antidotes; Carnitine; Depression; Drug Overdose; Female; Humans;	2007
Simultaneous side effects of both clozapine and valproate. Intensive care medicine, 2001, Volume: 27, Issue:5 Topics: Adult; Anticonvulsants; Antipsychotic Agents; Carnitine; Clozapine; Coma; Diabetic Ketoacidosis; Dru	2001
L-carnitine administration reverses acute mental status changes in a chronic hemodialysis patient with hepatitis C infection. Clinical nephrology, 2002, Volume: 57, Issue:5 Topics: Carnitine; Consciousness Disorders; Hepatitis C; Humans; Hyperammonemia; Male; Middle Aged; Renal Di	2002

carnitine and Hyperammonemia

Research Excerpts

Research

Studies (177)

Authors

Clinical Trials (7)

Trial Overview

Reviews

Trials

Other Studies

Authors	Studies
Ai, L	1
Fang, Y	1
Ziats, CA	1
Burns, WB	1
Tedder, ML	1
Pollard, L	1
Wood, T	1
Champaigne, NL	1
Geng, G	1
Yang, Q	1
Fan, X	1
Lin, C	3
Wu, L	3
Chen, S	3
Luo, J	1
Nicholson, C	1
Fowler, M	1
Mullen, C	1
Cunningham, B	1
Iriyama, N	1
Miura, K	1
Uchino, Y	1
Takahashi, H	1
Nakagawa, M	1
Iizuka, K	1
Hamada, T	1
Koike, T	1
Kurihara, K	1
Nakayama, T	1
Takei, M	1
Hatta, Y	1
Nakamura, H	1
Lin, Y	6
Lin, B	1
Chen, Y	4
Zheng, Z	4
Fu, Q	4
Lin, W	5
Zhang, W	2
Crefcoeur, LL	1
Visser, G	1
Ferdinandusse, S	2
Wijburg, FA	1
Langeveld, M	1
Sjouke, B	1
Matsufuji, S	1
Shoji, T	1
Lee, S	1
Yamaguchi, M	1
Nishimura, M	1
Tsujimoto, Y	1
Nakatani, S	1
Morioka, T	1
Mori, K	1
Emoto, M	1
Bazargani, B	1
Mojtahedi, SY	1
Fahimi, D	1
Askarian, F	1
Moghtaderi, M	1
Abbasi, A	1
Samimi, M	1
Bakhtiari Koohsorkhi, M	1
Jing, Z	1
Okubo, H	2
Morishige, JI	1
Xu, P	1
Hasan, N	1
Nagata, N	1
Ando, H	3
Yildiz, D	1
Yazici, MU	1
Oguz, MM	1
Torun, EG	1
Sezer, A	1
Kiliç, M	1
Jain, S	1
Kumar, K	1
Malhotra, S	1
Sibal, A	1
Mattman, A	1
Masoudi, R	1
Stockler-Ipsiroglu, S	1
Zivkovic, I	1
Lehman, A	1
Dionne, JM	1
Kayıkçıoğlu, M	1
Özbay, B	1
Yağmur, B	1
Canda, E	1
Bayraktaroğlu, S	1
Şimşek, E	1
Kalkan Uçar, S	1
Patel, J	1
Berezowski, I	1
Mazer-Amirshahi, M	1
Frasure, SE	1
Tran, QK	1
Pourmand, A	1
Song, W	1
Ye, S	1
Zheng, L	1
Schiavo, A	1
Maldonado, C	3
Vázquez, M	3
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