carnitine has been researched along with Cardiomyopathies in 256 studies
Cardiomyopathies: A group of diseases in which the dominant feature is the involvement of the CARDIAC MUSCLE itself. Cardiomyopathies are classified according to their predominant pathophysiological features (DILATED CARDIOMYOPATHY; HYPERTROPHIC CARDIOMYOPATHY; RESTRICTIVE CARDIOMYOPATHY) or their etiological/pathological factors (CARDIOMYOPATHY, ALCOHOLIC; ENDOCARDIAL FIBROELASTOSIS).
| Excerpt | Relevance | Reference |
|---|---|---|
| "Fatigue is a common adverse event during lenvatinib treatment in patients with hepatocellular carcinoma." | 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) |
| "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) |
| "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) |
| "Carnitine deficiency was found in approximately 17% of patients with epilepsy and was significantly associated with carnitine-free enteral formula only by tube feeding, number of AEDs taken (independent of VPA use), body weight (BW), body height and Gross Motor Function Classification System (GMFCS) score." | 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) |
| "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) |
| "To evaluate the therapeutic efficacy of l-carnitine in heart failure, the myocardial carnitine levels and the therapeutic efficacy of l-carnitine were studied in cardiomyopathic BIO 14." | 7.68 | L-carnitine treatment for congestive heart failure--experimental and clinical study. ( Kobayashi, A; Masumura, Y; Yamazaki, N, 1992) |
| "Although cancer patients had carnitine deficiency and lower carnitine stores, these did not affect resting energy expenditure, total food intake, or plasma lysine and methionine levels." | 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) |
| "Therefore, patients with liver cirrhosis should be treated as if they are in a state of relative carnitine deficiency at the liver, skeletal muscle, and mitochondrial levels, even if the blood carnitine level is not decreased." | 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) |
| "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) |
| "Carnitine deficiency was revealed in 54." | 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) |
| "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) |
| "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) |
| "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) |
| "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) |
| "Fatigue is a common adverse event during lenvatinib treatment in patients with hepatocellular carcinoma." | 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) |
| "The thiamine therapy was then continued." | 5.28 | Neonatal cardiomyopathy and lactic acidosis responsive to thiamine. ( Abeling, NG; Bakker, HD; Lam, J; Luyt-Houwen, IE; Scholte, HR; van Gennip, AH, 1991) |
| "Fraternal twins who had fasting hypoglycemia, hypoketonemia, muscle weakness, and hepatic dysfunction are reported." | 5.27 | Hypoglycemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain acylcarnitine excess responsive to medium chain triglyceride diet. ( Bier, DM; Brown, BI; Dickie, M; Engel, AG; Glasgow, AM; Perry, LW; Todaro, J; Utter, MF, 1983) |
| " Because of its trophic action on the myocardial tissue, the use of L-carnitine has been evaluated during IL-2 therapy in advanced cancer patients with clinically important cardiac diseases." | 5.07 | Prevention by L-carnitine of interleukin-2 related cardiac toxicity during cancer immunotherapy. ( Barni, S; Galli, MA; Lissoni, P; Tancini, G, 1993) |
| "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) |
| "When patients suffer from hepato-encephalopathy, (cardio)myopathy, dystrophy, hypoglycaemia, some metabolic diseases and several other disease states, carnitine deficiency should be considered." | 4.78 | [Pathogenesis and pathophysiology of carnitine deficiency, a predictable risk]. ( Przyrembel, H; Rodrigues Pereira, R; Scholte, HR, 1990) |
| "Carnitine deficiency is probably not a major cause of general fatigue but may occur in patients with CML receiving TKI therapy." | 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) |
| "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) |
| " 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) |
| " The aim of the study was to investigate the role of MMPs and their inhibitors (TIMPs), in the pathogenesis of choline deficiency-induced cardiomyopathy, and the way they are affected by carnitine supplementation." | 3.83 | Immunohistochemical determination of the extracellular matrix modulation in a rat model of choline-deprived myocardium: the effects of carnitine. ( Giannopoulou, I; Karkalousos, P; Kriebardis, A; Lazaris, A; Liapi, C; Panayiotides, I; Papavdi, A; Perelas, A; Strilakou, A, 2016) |
| "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) |
| "Carnitine deficiency was found in approximately 17% of patients with epilepsy and was significantly associated with carnitine-free enteral formula only by tube feeding, number of AEDs taken (independent of VPA use), body weight (BW), body height and Gross Motor Function Classification System (GMFCS) score." | 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) |
| "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) |
| "This study investigated whether cyclophosphamide (CP) and ifosfamide (IFO) therapy alters the expression of the key genes engaged in long-chain fatty acid (LCFA) oxidation outside rat heart mitochondria, and if so, whether these alterations should be viewed as a mechanism during CP- and IFO-induced cardiotoxicity." | 3.80 | Inhibition of gene expression of carnitine palmitoyltransferase I and heart fatty acid binding protein in cyclophosphamide and ifosfamide-induced acute cardiotoxic rat models. ( Al-Harbi, MM; Al-Harbi, NO; Al-Hosaini, KA; Al-Shabanah, OA; Al-Sharary, SD; Aldelemy, ML; Hafez, MM; Sayed-Ahmed, MM, 2014) |
| "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) |
| " At 4 weeks after ascending aortic constriction, jvs/+ mice showed an exaggeration of cardiac hypertrophy and pulmonary congestion, further increased gene expression of atrial natriuretic peptide in the left ventricles, further deterioration of left ventricular fractional shortening, reduced myocardial phosphocreatine:adenosine triphosphate ratio, and increased mortality compared with wild-type mice; l-carnitine supplementation prevented these changes in jvs/+ mice subjected to ascending aortic constriction." | 3.74 | Pressure overload-induced cardiomyopathy in heterozygous carrier mice of carnitine transporter gene mutation. ( Asai, T; Matsui, H; Murakami, H; Murakami, R; Murohara, T; Numaguchi, Y; Okumura, K; Takahashi, R; Tsuzuki, M, 2007) |
| "Propionyl- l -carnitine (PLC) is a naturally occurring compound that has been considered for the treatment of many forms of cardiomyopathies." | 3.71 | Propionyl-L-carnitine as protector against adriamycin-induced cardiomyopathy. ( Abou El-Naga, SA; Calvani, M; Gaballah, HE; Nicolai, R; Salman, TM; Sayed-Ahmed, MM, 2001) |
| "To evaluate the therapeutic efficacy of l-carnitine in heart failure, the myocardial carnitine levels and the therapeutic efficacy of l-carnitine were studied in cardiomyopathic BIO 14." | 3.68 | L-carnitine treatment for congestive heart failure--experimental and clinical study. ( Kobayashi, A; Masumura, Y; Yamazaki, N, 1992) |
| "There was no significant improvement in muscle spasms, although decreased muscle cramping after L-carnitine treatment was reported by 31% of patients who had undergone HD for >4 years." | 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) |
| "L-carnitine infusion appears safe in vasopressor-dependent septic shock." | 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) |
| "Although cancer patients had carnitine deficiency and lower carnitine stores, these did not affect resting energy expenditure, total food intake, or plasma lysine and methionine levels." | 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) |
| "Carnitine plays a key role in energy production in the myocardium." | 2.77 | Carnitine supplementation improves cardiac strain rate in children on chronic hemodialysis. ( Ellini, A; Frank, L; Moudgil, A; Sable, C; Sgambat, K, 2012) |
| "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) |
| "Therefore, patients with liver cirrhosis should be treated as if they are in a state of relative carnitine deficiency at the liver, skeletal muscle, and mitochondrial levels, even if the blood carnitine level is not decreased." | 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) |
| "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) |
| "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) |
| " 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 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 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 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) |
| "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 has been identified in a variety of mammalian tissues and has an obligate role in the mitochondrial oxidation of long-chain fatty acids through the action of specialized acyltransferases." | 2.42 | The role of carnitine in normal and altered fatty acid metabolism. ( Hoppel, C, 2003) |
| "In patients with chronic renal failure, not yet undergoing hemodialysis (HD), plasma acylcarnitines accumulate in part due to a decreased renal clearance of esterified carnitine moieties." | 2.42 | Carnitine replacement in end-stage renal disease and hemodialysis. ( Amato, A; Benatti, P; Brass, EP; Calvani, M; D'Iddio, S; Giordano, V; Koverech, A; Mancinelli, A, 2004) |
| "glycogenosis type II and III), lysosomal storage diseases (e." | 2.41 | Metabolic cardiomyopathies. ( Guertl, B; Hoefler, G; Noehammer, C, 2000) |
| "The acute lipidosis most probably is explained by a slow oxidation of 22:1 acyl-CoA by the mitochondrial acyl-CoA dehydrogenase combined with an inhibitory effect of this CoA ester on the oxidation of acyl-CoA esters of a more "normal" chain length." | 2.36 | Metabolism of very long-chain monounsaturated fatty acids (22:1) and the adaptation to their presence in the diet. ( Bremer, J; Norum, KR, 1982) |
| "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 deficiency was revealed in 54." | 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) |
| "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) |
| "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) |
| "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) |
| "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) |
| "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 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) |
| "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) |
| "Carnitine deficiency has been associated with progressive cardiomyopathy due to compromised energy metabolism." | 1.38 | [Clinical presentation and therapeutic outcomes of carnitine deficiency-induced cardiomyopathy]. ( Chen, SB; Fu, LJ; Guo, Y; Han, LS; Huang, MR; Li, F; Zhao, PJ; Zhu, M, 2012) |
| "Treatment of mitochondrial disorders is currently inadequate, emphasizing the need for experimental models." | 1.38 | Tissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathy. ( Cheung, MM; Grubb, DR; Hardman, BM; Ke, BX; Koleff, J; Komen, JC; Laskowski, A; Lazarou, M; Pepe, S; Pitt, JJ; Rodda, FA; Ryan, MT; Smolich, JJ; Thorburn, DR, 2012) |
| "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) |
| "(1) Carnitine deficiency is a risk factor which is involved in CP-related cardiomyopathy; (2) serum and urinary carnitine levels should be monitored and viewed as indices of CP-induced multiple organ toxicity, and (3) carnitine supplementation, using PLC, prevents the development of CP-induced cardiotoxicity." | 1.36 | Carnitine deficiency aggravates cyclophosphamide-induced cardiotoxicity in rats. ( Al-Shabanah, OA; Aleisa, AM; Darweesh, AQ; Fatani, AG; Rizwan, L; Sayed-Ahmed, MM, 2010) |
| "L-carnitine may enhance HSP70 in a cellular-type manner." | 1.34 | Differential expression of HSP70 and ultrastructure of heart and liver tissues of rats treated with adriamycin: protective role of L-carnitine. ( Porras, N; Strauss, M, 2007) |
| "Carnitine treatment resulted in dramatic improvement of the cardiac symptoms, echocardiographic, and EKG findings in both cases." | 1.32 | Phenotypic manifestations of the OCTN2 V295X mutation: sudden infant death and carnitine-responsive cardiomyopathy in Roma families. ( Bene, J; Havasi, V; Kispál, G; Komlósi, K; Méhes, K; Melegh, B; Mogyorósy, G; Oláh, E; Pajor, L; Sumegi, B, 2004) |
| "l-Carnitine (CARN) plays a major role in fatty acid oxidation by translocating activated long-chain fatty acids into the matrix of mitochondria." | 1.32 | Effect of L-carnitine supplementation on cardiac carnitine palmitoyltransferase activities and plasma carnitine concentrations in adriamycin-treated rats. ( Bennett, MJ; Boriack, RL; Hong, YM; Yoon, HR, 2003) |
| "L-Carnitine-treated patients were younger than control patients and had poorer clinical functioning at baseline, yet they demonstrated lower mortality and a level of clinical functioning and clinical severity comparable to control patients on conventional therapy by the end of the study." | 1.31 | Metabolic aspects of myocardial disease and a role for L-carnitine in the treatment of childhood cardiomyopathy. ( Darragh, R; Francis, P; Fricker, FJ; Helton, E; Jue, K; Koch, G; Linn, LS; Mair, D; Pierpont, ME; Prochazka, JV; Winter, SC, 2000) |
| "Carnitine was administered i." | 1.30 | Carnitine promotes heat shock protein synthesis in adriamycin-induced cardiomyopathy in a neonatal rat experimental model. ( Anselmi, G; Hermoso, T; Strauss, M; Tejero, F, 1998) |
| "The thiamine therapy was then continued." | 1.28 | Neonatal cardiomyopathy and lactic acidosis responsive to thiamine. ( Abeling, NG; Bakker, HD; Lam, J; Luyt-Houwen, IE; Scholte, HR; van Gennip, AH, 1991) |
| "Patients with secondary cardiomyopathies tended to have normal carnitine values." | 1.27 | Plasma carnitine concentrations in cardiomyopathy patients. ( Shug, AL; Tripp, ME, 1984) |
| "Fraternal twins who had fasting hypoglycemia, hypoketonemia, muscle weakness, and hepatic dysfunction are reported." | 1.27 | Hypoglycemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain acylcarnitine excess responsive to medium chain triglyceride diet. ( Bier, DM; Brown, BI; Dickie, M; Engel, AG; Glasgow, AM; Perry, LW; Todaro, J; Utter, MF, 1983) |
| "Of the nine patients with encephalopathy, eight showed improvement in their mental status." | 1.27 | Plasma carnitine deficiency. Clinical observations in 51 pediatric patients. ( Curry, CJ; Hogue, R; Hutchinson, HT; Shug, A; Szabo-Aczel, S; Winter, SC, 1987) |
| "L-Carnitine was given intraperitoneally each day at a dose of 500 mg/kg." | 1.27 | Carnitine protection against adriamycin-induced cardiomyopathy in rats. ( Gilbert, EF; McFalls, EO; Paulson, DJ; Shug, AL, 1986) |
| "Carnitine was decreased in the skeletal muscles and the serum." | 1.27 | Successful carnitine treatment in two siblings having lipid storage myopathy with hypertrophic cardiomyopathy. ( Hirata, K; Hirose, F; Kato, H; Matsuishi, T; Nonaka, I; Ohta, K; Ohtaki, E; Sugiyama, N; Terasawa, K; Yoshino, M, 1985) |
| "Carnitine content was markedly reduced in muscle and moderately in plasma." | 1.26 | "Carnitine deficient" myopathy and cardiomyopathy with fatal outcome. ( Cornelio, F; Di Donato, S; Gori, G; Mora, M; Peluchetti, D; Rimoldi, M; Testa, D, 1980) |
| "Carnitine concentration was measured in plasma, muscle, and dialysate before and after haemodialysis in patients with renal failure and in plasma and muscle of healthy controls." | 1.26 | Carnitine deficiency induced during intermittent haemodialysis for renal failure. ( Bergrem, H; Bohmer, T; Eiklid, K, 1978) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 48 (18.75) | 18.7374 |
| 1990's | 26 (10.16) | 18.2507 |
| 2000's | 33 (12.89) | 29.6817 |
| 2010's | 104 (40.63) | 24.3611 |
| 2020's | 45 (17.58) | 2.80 |
| Authors | Studies |
|---|---|
| Ai, L | 1 |
| Fang, Y | 1 |
| Rücklová, K | 1 |
| Hrubá, E | 1 |
| Pavlíková, M | 1 |
| Hanák, P | 1 |
| Farolfi, M | 1 |
| Chrastina, P | 1 |
| Vlášková, H | 1 |
| Kousal, B | 1 |
| Smolka, V | 1 |
| Foltenová, H | 1 |
| Adam, T | 1 |
| Friedecký, D | 1 |
| Ješina, P | 1 |
| Zeman, J | 1 |
| Kožich, V | 1 |
| Honzík, T | 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 |
| Iriyama, N | 1 |
| Miura, K | 3 |
| 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 | 2 |
| Visser, G | 2 |
| Ferdinandusse, S | 2 |
| Wijburg, FA | 1 |
| Langeveld, M | 2 |
| Sjouke, B | 1 |
| Matsufuji, S | 1 |
| Shoji, T | 1 |
| Lee, S | 1 |
| Yamaguchi, M | 1 |
| Nishimura, M | 2 |
| 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 |
| Yu, HK | 1 |
| Ok, SH | 1 |
| Kim, S | 1 |
| Sohn, JT | 1 |
| Toyama, Y | 1 |
| Fujii, Y | 1 |
| Hori, SI | 1 |
| Yoshimura, K | 1 |
| Kaneko, K | 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 |
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| Özbay, B | 1 |
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| Canda, E | 1 |
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| Kalkan Uçar, S | 1 |
| Liammongkolkul, S | 1 |
| Boonyawat, B | 1 |
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| Tim-Aroon, T | 1 |
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| Song, W | 1 |
| Ye, S | 1 |
| Zheng, L | 1 |
| van den Heuvel, LM | 1 |
| Kater-Kuipers, A | 1 |
| van Dijk, T | 1 |
| Henneman, L | 1 |
| Zhang, Q | 1 |
| Yao, N | 1 |
| Liu, Z | 1 |
| Xu, C | 1 |
| Ding, Z | 1 |
| Gélinas, R | 1 |
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| Horvath, G | 1 |
| Laksman, Z | 1 |
| Almannai, M | 1 |
| Alfadhel, M | 1 |
| El-Hattab, AW | 2 |
| Li, X | 1 |
| Zhu, X | 1 |
| Jia, C | 1 |
| Ni, M | 1 |
| Li, Y | 2 |
| Zhang, L | 1 |
| Zhao, D | 1 |
| Ranea-Robles, P | 1 |
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| van Vlies, N | 1 |
| Vaz, FM | 3 |
| Houten, SM | 1 |
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| Bulman, D | 1 |
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| Tein, I | 3 |
| Geraghty, MT | 1 |
| Ishizuka, K | 1 |
| Kitagawa, R | 1 |
| Okubo, S | 1 |
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| Miyazaki, A | 1 |
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| Zhang, C | 2 |
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| Zhang, Y | 3 |
| Zhu, L | 1 |
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| Huang, X | 3 |
| Wang, S | 2 |
| Leng, J | 1 |
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| Zheng, R | 1 |
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| Lin, JC | 1 |
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| Nakata, J | 1 |
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| Suzuki, Y | 3 |
| Wang, K | 1 |
| Fu, C | 1 |
| Chen, D | 3 |
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| Teng, Y | 1 |
| Lin-Peng, S | 1 |
| Li, Z | 2 |
| Liang, D | 1 |
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| Imam, F | 1 |
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| Alasmari, AF | 1 |
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| Alanazi, MM | 1 |
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| Stenlid, R | 1 |
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| 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 | ||
| Phase 2 Study of Triheptanoin for Treatment of Long-Chain Fatty Acid Oxidation Disorders[NCT01379625] | Phase 2 | 32 participants (Actual) | Interventional | 2011-09-30 | Completed | ||
| A Phase 2A/2B Placebo-controlled Randomised Clinical Trial to Test the Ability of Triheptanoin to Protect Primary Airway Epithelial Cells Obtained From Participants With Ataxia-telangiectasia Against Death Induced by Glucose Deprivation[NCT04513002] | Phase 2 | 30 participants (Actual) | Interventional | 2022-03-15 | 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 | |||
| Fat and Sugar Metabolism During Exercise in Patients With Metabolic Myopathy[NCT02635269] | 60 participants (Actual) | Interventional | 2016-01-31 | Active, not recruiting | |||
| Triheptanoin's Effect on Fatty Acid Oxidation and Exercise Tolerance in Patients With Debrancher Deficiency, Glycogenin-1 Deficiency and Phosphofructoinase Deficiency at Rest and During Exercise. A Randomized, Double-blind, Placebo-controlled, Cross-over [NCT03642860] | Phase 2 | 3 participants (Actual) | Interventional | 2018-08-15 | Terminated (stopped due to Problems with recruitment) | ||
| [NCT00005391] | 0 participants | Observational | 1995-09-30 | Completed | |||
| In Vivo Study of Safety, Tolerability and Dosing Effect on SMN mRNA and Protein Levels of Valproic Acid in Patients With Spinal Muscular Atrophy[NCT00374075] | Phase 1 | 42 participants | Interventional | 2003-09-30 | Completed | ||
| Phase I/II Trial of Valproic Acid and Carnitine in Infants With Spinal Muscular Atrophy Type I (CARNI-VAL Type I)[NCT00661453] | Phase 1/Phase 2 | 40 participants (Actual) | Interventional | 2008-04-30 | Completed | ||
| Correlation Between Carnitine Deficiency and Hypoglycemic Events in Type I Diabetes; Effects of Carnitine Supplementation on Hypoglycemic Events in Type I Diabetes[NCT00351234] | 200 participants (Actual) | Observational | 2004-10-31 | Completed | |||
| Multi-center Phase II Trial of Valproic Acid and Carnitine in Patients With Spinal Muscular Atrophy (SMA CARNI-VAL Trial)[NCT00227266] | Phase 2 | 94 participants (Actual) | Interventional | 2005-09-30 | Completed | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
Change in resting ejection fraction over 4 month treatment period (NCT01379625)
Timeframe: 4 months
| Intervention | percent (Mean) |
|---|---|
| Medium Chain Triglyceride (MCT) | -1.91 |
| Triheptanoin | 2.14 |
Total energy expenditure will be measured by doubly labeled water and resting energy expenditure will be measured by indirect calorimetry at baseline and again after 4 months of either MCT or trihpetanoin treatment. (NCT01379625)
Timeframe: change from baseline after 4 months of treatment
| Intervention | kcal/day (Mean) |
|---|---|
| Medium Chain Triglyceride (MCT) | -73 |
| Triheptanoin | 107 |
Subjects will complete a submaximal treadmill exercise study at baseline. Exercise heart heart, ventilation and perceived exertion will be measured. Subjects will be randomized to MCT or triheptanoin supplementation for 4 months. At the end of treatment, the exercise test will be repeated keeping work performed constant. Change in exercise heart rate, ventilation and exertion will be compared between groups. (NCT01379625)
Timeframe: change from baseline to 4 months of treatment
| Intervention | beats per minute (Mean) |
|---|---|
| Medium Chain Triglyceride (MCT) | -0.1 |
| Triheptanoin | -12.6 |
(NCT00661453)
Timeframe: -2 weeks, time 0, 3 months, 6 months
| Intervention | g (Mean) | |||||
|---|---|---|---|---|---|---|
| Lean Mass Baseline | Lean Mass 3 months | Lean Mass 6 months | Fat Mass Baseline | Fat Mass 3 months | Fat Mass 6 months | |
| SMA Type 1 | 4317.15 | 4993.92 | 5133.83 | 3011.37 | 3618.25 | 4316.08 |
The maximum Compound Motor Action Potential (CMAP) is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This is done multiple times, the outcome used is the highest peak, or response observed. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)
| Intervention | mV (Mean) | |
|---|---|---|
| Baseline | 6 months | |
| Cohort 1a Sitters Placebo Then Treatment | 2.28 | 2.32 |
| Cohort 1b Sitters Treatment | 2.93 | 2.37 |
| Cohort 2 Standers and Walkers - Treatment | 5.52 | 6.56 |
The maximum Compound Motor Action Potential (CMAP) is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This is done multiple times, the outcome used is the highest peak, or response observed. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)
| Intervention | mV (Median) | |
|---|---|---|
| Baseline | 6 months | |
| Cohort 1a Sitters Placebo Then Treatment | 1.91 | 1.44 |
| Cohort 1b Sitters Treatment | 2.2 | 1.8 |
| Cohort 2 Standers and Walkers - Treatment | 5.3 | 5.85 |
The maximum Compound Motor Action Potential (CMAP) area is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This procedure is repeated multiple times. The maximum area is the response that results in the largest area under the response curve. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)
| Intervention | mVms (Mean) | |
|---|---|---|
| Baseline | 6 months | |
| Cohort 1a Sitters Placebo Then Treatment | 5.46 | 5.28 |
| Cohort 1b Sitters Treatment | 5.45 | 5.26 |
| Cohort 2 Standers and Walkers - Treatment | 14.85 | 16.26 |
The maximum Compound Motor Action Potential (CMAP) area is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This procedure is repeated multiple times. The maximum area is the response that results in the largest area under the response curve. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)
| Intervention | mVms (Median) | |
|---|---|---|
| Baseline | 6 months | |
| Cohort 1a Sitters Placebo Then Treatment | 3.6 | 3.74 |
| Cohort 1b Sitters Treatment | 4.6 | 3.4 |
| Cohort 2 Standers and Walkers - Treatment | 13.65 | 16.85 |
Comparison of Modified Hammersmith Change from baseline to 6 months. Scores range from 0 to 40. A higher score indicates a better outcome. This scale is used to assess gross motor abilities of non-ambulant children with SMA in multiple research trials as well as in clinical settings. (NCT00227266)
Timeframe: 0 months, 6 months
| Intervention | Score (Mean) | ||
|---|---|---|---|
| Baseline visit (0 weeks) | 6 Month visit (V2) | Change from Baseline | |
| Cohort 1a Sitters Placebo Then Treatment | 20.0 | 20.6 | 0.6 |
| Cohort 1b Sitters Treatment | 16.6 | 16.8 | 0.2 |
"Baseline Modified Hammersmith Extend testing. The baseline test is the score they receive during their screening visits. This scale ranges from 0 to 56. A higher score indicates a better outcome.~This scale is used to assess gross motor abilities of children with SMA in multiple research trials as well as in clinical settings." (NCT00227266)
Timeframe: 1 month prior to enrollment, at enrollment (0 months)
| Intervention | Score (Mean) | |
|---|---|---|
| Modified Hammersmith Extend at S1 (-4 weeks) | Modified Hammersmith Extend at S2 (0 weeks) | |
| Cohort 2 Experimental | 47.0 | 48.3 |
45 reviews available for carnitine and Cardiomyopathies
| Article | Year |
|---|---|
Clinical characteristics of primary carnitine deficiency: A structured review using a case-by-case approach.
Topics: Cardiomyopathies; Carnitine; Child, Preschool; Humans; Hyperammonemia; Infant, Newborn; Muscular Dis | 2022 |
Anesthetic management of patients with carnitine deficiency or a defect of the fatty acid β-oxidation pathway: A narrative review.
Topics: Anesthetics; Cardiomyopathies; Carnitine; Fatty Acids; Humans; Oxidation-Reduction | 2022 |
Carnitine Inborn Errors of Metabolism.
Topics: Aldehyde Oxidoreductases; Cardiomyopathies; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmi | 2019 |
Usefulness of Carnitine Supplementation for the Complications of Liver Cirrhosis.
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).
Topics: Alanine Transaminase; Aspartate Aminotransferases; Cardiomyopathies; Carnitine; Dietary Supplements; | 2020 |
The role of carnitine in maintenance dialysis therapy.
Topics: Adult; Cardiomyopathies; Carnitine; Child; Humans; Hyperammonemia; Kidney Failure, Chronic; Peritone | 2021 |
Significance of l-carnitine for human health.
Topics: Cardiomyopathies; Carnitine; Carnitine Acyltransferases; Fatty Acids; Humans; Hyperammonemia; Liver; | 2017 |
Brain carnitine deficiency causes nonsyndromic autism with an extreme male bias: A hypothesis.
Topics: Autistic Disorder; Blood-Brain Barrier; Brain; Cardiomyopathies; Carnitine; Female; Humans; Hyperamm | 2017 |
A systematic review about prophylactic L-carnitine administration in parenteral nutrition of extremely preterm infants.
Topics: Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Infant; Infant, Extremely Premature; Infant, Ne | 2018 |
Effects of Levocarnitine on Cardiac Function and Renal Anemia in Hemodialysis Patients.
Topics: Anemia; Cardiomyopathies; Carnitine; Heart; Hematinics; Humans; Hyperammonemia; Kidney Failure, Chro | 2018 |
Lipid storage myopathies: Current treatments and future directions.
Topics: Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Lipid Metabolism; Lipid Metabolism, Inborn Erro | 2018 |
Carnitine Profile by Tandem Mass Spectrometry and Dialysis Patients.
Topics: Carboxylic Acids; Cardiomyopathies; Carnitine; Fatty Acids; Humans; Hyperammonemia; Muscular Disease | 2019 |
[Valproic acid-induced hyperammonemic encephalopathy in a patient receiving valproic acid monotherapy].
Topics: Aged; Ammonia; Anticonvulsants; Biomarkers; Cardiomyopathies; Carnitine; Consciousness Disorders; Ep | 2019 |
Carnitine deficiency in chronic critical illness.
Topics: Cardiomyopathies; Carnitine; Chronic Disease; Critical Illness; Fatty Acids; Humans; Hyperammonemia; | 2014 |
Disorders of carnitine biosynthesis and transport.
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].
Topics: Cardiomyopathies; Carnitine; Cord Blood Stem Cell Transplantation; Fetal Blood; Graft vs Host Diseas | 2016 |
Veterinary clinical nutrition: success stories: an overview.
Topics: Animal Nutritional Physiological Phenomena; Animals; Calculi; Cardiomyopathies; Cardiomyopathy, Dila | 2016 |
Primary Carnitine Deficiency and Newborn Screening for Disorders of the Carnitine Cycle.
Topics: Cardiomyopathies; Carnitine; Deficiency Diseases; Denmark; Dietary Supplements; Genetic Testing; Hum | 2016 |
Anthracycline-Induced Cardiotoxicity in Young Cancer Patients: The Role of Carnitine.
Topics: Anthracyclines; Antineoplastic Agents; Cardiomyopathies; Cardiotoxicity; Carnitine; Child; Deficienc | 2016 |
Historical Perspective on Clinical Trials of Carnitine in Children and Adults.
Topics: Administration, Intravenous; Adult; Cardiomyopathies; Carnitine; Carnitine Acyltransferases; Child; | 2016 |
State of the art in muscle lipid diseases.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Cardiomyopathies; Carnitine; Carnitine O-Palmitoyltransferase; H | 2010 |
Delineating the role of alterations in lipid metabolism to the pathogenesis of inherited skeletal and cardiac muscle disorders: Thematic Review Series: Genetics of Human Lipid Diseases.
Topics: Adult; Animals; Cardiolipins; Cardiomyopathies; Carnitine; Child, Preschool; Desmin; Dystrophin; Fab | 2012 |
Pharmacological and pathophysiological roles of carnitine/organic cation transporters (OCTNs: SLC22A4, SLC22A5 and Slc22a21).
Topics: Animals; Cardiomyopathies; Carnitine; Carrier Proteins; Humans; Hyperammonemia; Intestinal Absorptio | 2013 |
[Inherited and acquired disorders of mitochondrial DNA].
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Anti-HIV Agents; Cardiomyopathies; Carnitine; Child; Dia | 2002 |
The role of carnitine in normal and altered fatty acid metabolism.
Topics: Animals; Cardiomyopathies; Carnitine; Fatty Acids; Heart Failure; Humans; Kidney Failure, Chronic; L | 2003 |
The role of carnitine in myocardial dysfunction.
Topics: Cardiomyopathies; Carnitine; Fatty Acids; Humans; Kidney Diseases; Myocardium; Renal Dialysis | 2003 |
Carnitine replacement in end-stage renal disease and hemodialysis.
Topics: Anemia; Cardiomyopathies; Carnitine; Homeostasis; Humans; Kidney Failure, Chronic; Renal Dialysis | 2004 |
[Secondary mitochondrial dysfunction in the pathogenesis of acute myocardial dysfunction: possibilities of diagnostics and principles of medication].
Topics: Cardiomyopathies; Cardiotonic Agents; Carnitine; Cytoprotection; Humans; Mitochondria, Heart | 2007 |
Metabolism of very long-chain monounsaturated fatty acids (22:1) and the adaptation to their presence in the diet.
Topics: Acyl Coenzyme A; Adaptation, Physiological; Animals; Cardiomyopathies; Carnitine; Carnitine Acyltran | 1982 |
Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a severe fatty acid oxidation disorder.
Topics: 3-Hydroxyacyl CoA Dehydrogenases; Cardiomyopathies; Carnitine; Clinical Enzyme Tests; Fatal Outcome; | 1994 |
Infantile familial cardiomyopathy due to mitochondrial complex I and IV associated deficiency.
Topics: Biopsy; Cardiomyopathies; Carnitine; Consanguinity; Cytochrome-c Oxidase Deficiency; Electron Transp | 1993 |
Acylcarnitines in intermediary metabolism.
Topics: Acylation; Cardiomyopathies; Cardiotonic Agents; Carnitine; Humans; Metabolic Diseases | 1995 |
Carnitine deficiency-induced cardiomyopathy.
Topics: Animals; Cardiomyopathies; Carnitine; Disease Models, Animal; Humans; Kinetics; Models, Biological; | 1998 |
Echocardiographic features of genetic diseases: part 1. Cardiomyopathy.
Topics: Arrhythmogenic Right Ventricular Dysplasia; Beckwith-Wiedemann Syndrome; Cardiomyopathies; Cardiomyo | 2000 |
Cardiomyopathy in childhood, mitochondrial dysfunction, and the role of L-carnitine.
Topics: Cardiomyopathies; Carnitine; Child; Diagnosis, Differential; Energy Metabolism; Humans; Mitochondria | 2000 |
Metabolic cardiomyopathies.
Topics: Adult; Animals; Calcium; Cardiomegaly; Cardiomyopathies; Cardiomyopathy, Alcoholic; Carnitine; Diabe | 2000 |
Chronic cardiomyopathy and weakness or acute coma in children with a defect in carnitine uptake.
Topics: Biological Transport; Cardiomyopathies; Carnitine; Cells, Cultured; Child; Child, Preschool; Coma; F | 1991 |
Impaired skin fibroblast carnitine uptake in primary systemic carnitine deficiency manifested by childhood carnitine-responsive cardiomyopathy.
Topics: Biological Transport, Active; Cardiomyopathies; Carnitine; Child; Child, Preschool; Fatty Acids; Fem | 1990 |
[Pathogenesis and pathophysiology of carnitine deficiency, a predictable risk].
Topics: Adult; Cardiomyopathies; Carnitine; Child; Child, Preschool; Female; Hepatic Encephalopathy; Humans; | 1990 |
Diabetes-induced abnormalities in the myocardium.
Topics: Adenosine Triphosphatases; Animals; Blood Glucose; Calcium; Carbohydrate Metabolism; Cardiomyopathie | 1986 |
The role of the carnitine system in myocardial fatty acid oxidation: carnitine deficiency, failing mitochondria and cardiomyopathy.
Topics: Animals; Cardiomyopathies; Carnitine; Carnitine O-Palmitoyltransferase; Fatty Acids; Humans; Mitocho | 1987 |
Transport and function of L-carnitine and L-propionylcarnitine: relevance to some cardiomyopathies and cardiac ischemia.
Topics: Animals; Biological Transport; Cardiomyopathies; Carnitine; Coronary Disease; Humans; Myocardium; Sa | 1987 |
Role of carnitine-dependent metabolic pathways in heart disease without primary ischemia.
Topics: Amino Acids, Branched-Chain; Animals; Cardiomyopathies; Carnitine; Fatty Acids; Kidney; Lipidoses; L | 1987 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
Carnitine metabolism and function in humans.
Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn | 1986 |
The pharmacology of carnitine.
Topics: Acidosis; Animals; Cardiomyopathies; Carnitine; Humans; Infant, Newborn; Renal Dialysis; Reye Syndro | 1987 |
11 trials available for carnitine and Cardiomyopathies
| Article | Year |
|---|---|
Carnitine insufficiency is associated with fatigue during lenvatinib treatment in patients with hepatocellular carcinoma.
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.
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.
Topics: Cardiomyopathies; Carnitine; Child; Dietary Supplements; Fatigue; Female; Humans; Hyperammonemia; Ma | 2021 |
Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded, randomized controlled trial.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Caprylates; Cardiomyopathies; Carnitine; Chil | 2017 |
Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded, randomized controlled trial.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Caprylates; Cardiomyopathies; Carnitine; Chil | 2017 |
Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded, randomized controlled trial.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Caprylates; Cardiomyopathies; Carnitine; Chil | 2017 |
Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded, randomized controlled trial.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Caprylates; Cardiomyopathies; Carnitine; Chil | 2017 |
L-Carnitine Improves Skeletal Muscle Fat Oxidation in Primary Carnitine Deficiency.
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.
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.
Topics: Adult; Asian People; Cardiomyopathies; Carnitine; China; Female; Humans; Hyperammonemia; INDEL Mutat | 2019 |
Lower carnitine plasma values from malnutrition cancer patients.
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.
Topics: Aged; Black or African American; Body Mass Index; Cardiomyopathies; Carnitine; Dose-Response Relatio | 2014 |
Carnitine supplementation improves cardiac strain rate in children on chronic hemodialysis.
Topics: Adolescent; Cardiomyopathies; Carnitine; Child; Echocardiography; Female; Humans; Male; Renal Dialys | 2012 |
Prevention by L-carnitine of interleukin-2 related cardiac toxicity during cancer immunotherapy.
Topics: Adult; Aged; Cardiomyopathies; Carnitine; Drug Therapy, Combination; Female; Heart Diseases; Humans; | 1993 |
200 other studies available for carnitine and Cardiomyopathies
| Article | Year |
|---|---|
General anesthesia with cisatracurium and sevoflurane for a patient with primary carnitine deficiency receiving L-carnitine therapy: A case report.
Topics: Adult; Anesthesia, General; Anesthetics, Inhalation; Atracurium; Cardiomyopathies; Carnitine; Female | 2021 |
Impact of Newborn Screening and Early Dietary Management on Clinical Outcome of Patients with Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency and Medium Chain Acyl-CoA Dehydrogenase Deficiency-A Retrospective Nationwide Study.
Topics: 3-Hydroxyacyl CoA Dehydrogenases; Acyl-CoA Dehydrogenase; Cardiomyopathies; Carnitine; Child; Child, | 2021 |
3-Methylglutaconic aciduria in carriers of primary carnitine deficiency.
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].
Topics: Cardiomyopathies; Carnitine; China; Humans; Hyperammonemia; Infant, Newborn; Metabolome; Muscular Di | 2021 |
Relationship between Carnitine Deficiency and Tyrosine Kinase Inhibitor Use in Patients with Chronic Myeloid Leukemia.
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.
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).
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.
Topics: Adolescent; Cardiomyopathies; Carnitine; Child; Humans; Hyperammonemia; Hypotension; Kidney Failure, | 2022 |
L-carnitine rescue for neonatal intractable mitochondrial cardiomyopathy.
Topics: Cardiomyopathies; Carnitine; Humans; Infant, Newborn | 2022 |
Lenvatinib causes reduced expression of carnitine/organic cation transporter 2 and carnitine deficiency in the skeletal muscle of rats.
Topics: Animals; Cardiomyopathies; Carnitine; Hyperammonemia; Muscle, Skeletal; Muscular Diseases; Organic C | 2022 |
Systemic Primary Carnitine Deficiency: A Case Report with Homozygoys SLC22A5 Gene Mutation.
Topics: Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Muscular Diseases; Mutation; Solute Carrier Fam | 2022 |
Rare case of primary carnitine deficiency presenting as acute liver failure.
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.
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.
Topics: Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Heart Failure; Humans; Hyperammonemia; Male; M | 2022 |
Phenotypic and molecular features of Thai patients with primary carnitine deficiency.
Topics: Cardiomyopathies; Carnitine; Female; Humans; Infant, Newborn; Mutation; Neonatal Screening; Solute C | 2023 |
Cardiologic evaluation of Turkish mitochondrial fatty acid oxidation disorders.
Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Cardiomyopathies; Carnitine; Child; Fatt | 2022 |
[Analysis of blood carnitine profile and SLC22A5 gene variants in 17 neonates with Primary carnitine deficiency].
Topics: Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Infant, Newborn; Muscular Diseases; Solute Carr | 2023 |
A qualitative study on the perspectives of mothers who had been diagnosed with primary carnitine deficiency through newborn screening of their child.
Topics: Cardiomyopathies; Carnitine; Female; Humans; Infant, Newborn; Mothers; Muscular Diseases; Neonatal S | 2023 |
An Autopsy Analysis of a Patient With Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency Caused by Compound Heterozygous HADHA Gene Mutations.
Topics: Autopsy; Cardiomyopathies; Carnitine; Female; Humans; Mitochondrial Trifunctional Protein, alpha Sub | 2023 |
Molecular Autopsy Implicates Primary Carnitine Deficiency in Sudden Unexplained Death and Reversible Short QT Syndrome.
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].
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.
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.
Topics: 5' Untranslated Regions; Actins; Biological Transport, Active; Cardiomyopathies; Carnitine; Cells, C | 2020 |
Primary carnitine deficiency - diagnosis after heart transplantation: better late than never!
Topics: Cardiomyopathies; Carnitine; Child; Heart Transplantation; Humans; Hyperammonemia; Infant, Newborn; | 2020 |
Screening 3.4 million newborns for primary carnitine deficiency in Zhejiang Province, China.
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.
Topics: Cardiomyopathies; Carnitine; Carnitine O-Palmitoyltransferase; China; Congenital Bone Marrow Failure | 2020 |
Ventricular Fibrillation Caused by Primary Carnitine Deficiency.
Topics: Adult; Cardiomyopathies; Cardiopulmonary Resuscitation; Carnitine; Electric Countershock; Female; Hu | 2020 |
Molecular and clinical characteristics of very-long-chain acyl-CoA dehydrogenase deficiency: A single-center experience in Saudi Arabia.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Cardiomyopathies; Carnitine; Cohort Studies; Congenital Bone Mar | 2020 |
Metabolic Serendipities of Expanded Newborn Screening.
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.
Topics: Cardiomyopathies; Carnitine; China; Cholestasis, Intrahepatic; Citrullinemia; Humans; Hyperammonemia | 2020 |
Newborn screening for primary carnitine deficiency in Quanzhou, China.
Topics: Cardiomyopathies; Carnitine; Child; China; Female; Humans; Hyperammonemia; Infant, Newborn; Muscular | 2021 |
Biochemical and genetic characteristics of 40 neonates with carnitine deficiency.
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.
Topics: Animals; Blood Pressure; Cardiomyopathies; Carnitine; Hyperammonemia; Hypertension; Muscular Disease | 2021 |
Effectiveness of monitoring free carnitine levels for L-carnitine supplementation in hemodialysis patients to maintain carnitine sufficiency and nutritional factors.
Topics: Aged; Cardiomyopathies; Carnitine; Dietary Supplements; Female; Humans; Hyperammonemia; Kidney Failu | 2021 |
Gene spectrum and clinical traits of 10 patients with primary carnitine deficiency.
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.
Topics: Cardiomyopathies; Carnitine; Child; Humans; Hyperammonemia; Hypoglycemia; Infant; Male; Malnutrition | 2022 |
Increased detection of primary carnitine deficiency through second-tier newborn genetic screening.
Topics: Cardiomyopathies; Carnitine; Genetic Testing; Humans; Hyperammonemia; Infant, Newborn; Muscular Dise | 2021 |
Altered mitochondrial metabolism in peripheral blood cells from patients with inborn errors of β-oxidation.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Cardiomyopathies; Carnitine; Child; Child, Preschool; Female; Ge | 2022 |
Biochemical characteristics of newborns with carnitine transporter defect identified by newborn screening in California.
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.
Topics: Alleles; Cardiomyopathies; Carnitine; Female; Genotype; Humans; Hyperammonemia; Infant, Newborn; Mal | 2017 |
Functional and molecular studies in primary carnitine deficiency.
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].
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.
Topics: Cardiomyopathies; Carnitine; Chromatography, Gas; DNA Mutational Analysis; Early Diagnosis; Hepatome | 2017 |
[Genetic diagnosis of 10 neonates with primary carnitine deficiency].
Topics: Cardiomyopathies; Carnitine; Computational Biology; Genetic Counseling; Humans; Hyperammonemia; Infa | 2017 |
Similarities between acylcarnitine profiles in large for gestational age newborns and obesity.
Topics: Cardiomyopathies; Carnitine; Female; Gestational Age; Humans; Hyperammonemia; Infant, Newborn; Infan | 2017 |
Dilated Cardiomyopathy With Short QT Interval Suggests Primary Carnitine Deficiency.
Topics: Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Child; Echocardiography; Electrocardiography; | 2018 |
Carnitine levels and mutations in the SLC22A5 gene in Faroes patients with Parkinson's disease.
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.
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.
Topics: Cardiomyopathies; Carnitine; Genetic Testing; Humans; Hyperammonemia; Lipid Metabolism; Male; Middle | 2018 |
Tissue acylcarnitine status in a mouse model of mitochondrial β-oxidation deficiency during metabolic decompensation due to influenza virus infection.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Cardiomyopathies; Carnitine; Congenital Bone Marrow Fai | 2018 |
L-carnitine Improved the Cardiac Function via the Effect on Myocardial Fatty Acid Metabolism in a Hemodialysis Patient.
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.
Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Amino Acid Metabolism, Inborn Errors; Ca | 2019 |
A newborn with seizures born to a mother diagnosed with primary carnitine deficiency.
Topics: Brain; Cardiomyopathies; Carnitine; Electroencephalography; Female; Fetal Diseases; Humans; Hyperamm | 2019 |
A mutation creating an upstream translation initiation codon in SLC22A5 5'UTR is a frequent cause of primary carnitine deficiency.
Topics: 5' Untranslated Regions; Alleles; Amino Acid Sequence; Base Sequence; Biological Transport; Cardiomy | 2019 |
Primary carnitine deficiency with severe acute hepatitis following rotavirus gastroenteritis.
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.
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].
Topics: Cardiomyopathies; Carnitine; Child, Preschool; DNA Mutational Analysis; Female; Humans; Hyperammonem | 2019 |
Increased risk of sudden death in untreated primary carnitine deficiency.
Topics: Adolescent; Adult; Arrhythmias, Cardiac; Cardiomyopathies; Carnitine; Child; Child, Preschool; Death | 2020 |
Cardiac magnetic resonance findings in a case of carnitine deficiency.
Topics: Arrhythmias, Cardiac; Cardiomyopathies; Carnitine; Contrast Media; Death, Sudden, Cardiac; Defibrill | 2013 |
Carnitine uptake defect (primary carnitine deficiency): risk in genotype-phenotype correlation.
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.
Topics: Animals; Cardiomyopathies; Carnitine; Female; Humans; Hyperammonemia; Muscular Diseases; Organic Cat | 2013 |
Carnitine levels in 26,462 individuals from the nationwide screening program for primary carnitine deficiency in the Faroe Islands.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Cardiomyopathies; Carnitine; Child; Child, Preschool; De | 2014 |
Newborn screening for medium-chain acyl-CoA dehydrogenase deficiency: regional experience and high incidence of carnitine deficiency.
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.
Topics: Adolescent; Adult; Arrhythmias, Cardiac; Cardiomyopathies; Carnitine; Denmark; Dietary Supplements; | 2014 |
Inhibition of gene expression of carnitine palmitoyltransferase I and heart fatty acid binding protein in cyclophosphamide and ifosfamide-induced acute cardiotoxic rat models.
Topics: Animals; Antineoplastic Agents, Alkylating; Blotting, Western; Cardiomyopathies; Cardiotoxicity; Car | 2014 |
Carnitine and cardiac dysfunction in childhood cancer survivors treated with anthracyclines.
Topics: Adolescent; Adult; Anthracyclines; Cardiomyopathies; Cardiovascular Diseases; Carnitine; Child; Fema | 2014 |
Primary carnitine deficiency cardiomyopathy.
Topics: Adolescent; Cardiomyopathies; Carnitine; Child; Female; Humans; Hyperammonemia; Infant; Male; Muscul | 2014 |
Analysis of genetic mutations in Chinese patients with systemic primary carnitine deficiency.
Topics: Asian People; Cardiomyopathies; Carnitine; Child; Child, Preschool; DNA Mutational Analysis; Exons; | 2014 |
Increased and early lipolysis in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency during fast.
Topics: 3-Hydroxyacyl CoA Dehydrogenases; Age Factors; Biomarkers; Blood Glucose; Calorimetry, Indirect; Car | 2015 |
[Genetics and pedigree analysis of primary carnitine deficiency cardiomyopathy in 6 cases].
Topics: Adolescent; Base Sequence; Cardiomyopathies; Carnitine; Child; Child, Preschool; DNA Mutational Anal | 2014 |
[Primary carnitine deficiency in an infant].
Topics: Cardiomyopathies; Carnitine; Female; Humans; Hyperammonemia; Infant; Muscular Diseases | 2014 |
Carnitine deficiency: Risk factors and incidence in children with epilepsy.
Topics: Adolescent; Anticonvulsants; Cardiomyopathies; Carnitine; Case-Control Studies; Child; Child, Presch | 2015 |
Carnitine is associated with fatigue following chemoradiotherapy for head and neck cancer.
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.
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.
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.
Topics: Adolescent; Cardiomyopathies; Carnitine; Child; Child, Preschool; Hormone Replacement Therapy; Human | 2015 |
Carnitine deficiency induces a short QT syndrome.
Topics: Adult; Animals; Arrhythmias, Cardiac; Cardiomyopathies; Carnitine; Child, Preschool; Echocardiograph | 2016 |
Carnitine insufficiency in children with inborn errors of metabolism: prevalence and treatment efficacy.
Topics: Cardiomyopathies; Carnitine; Child; Child, Preschool; Female; Humans; Hyperammonemia; Male; Metaboli | 2015 |
[Genetic and prenatal diagnosis for a Chinese family with primary carnitine deficiency].
Topics: Adult; Asian People; Base Sequence; Cardiomyopathies; Carnitine; China; Exons; Female; Genotype; Hum | 2015 |
Carnitine deficiency in children receiving continuous renal replacement therapy.
Topics: Acute Kidney Injury; Adolescent; Adult; Cardiomyopathies; Carnitine; Child; Child, Preschool; Female | 2016 |
Reversible weakness and encephalopathy while on long-term valproate treatment due to carnitine deficiency.
Topics: Adult; Antimanic Agents; Brain Diseases; Cardiomyopathies; Carnitine; Female; Humans; Hyperammonemia | 2015 |
Elevation of pivaloylcarnitine by sivelestat sodium in two children.
Topics: Amino Acid Metabolism, Inborn Errors; Cardiomyopathies; Carnitine; Child, Preschool; Chromatography, | 2015 |
Immunohistochemical determination of the extracellular matrix modulation in a rat model of choline-deprived myocardium: the effects of carnitine.
Topics: Administration, Oral; Animals; Cardiomyopathies; Carnitine; Choline Deficiency; Disease Models, Anim | 2016 |
A Moderate Carnitine Deficiency Exacerbates Isoproterenol-Induced Myocardial Injury in Rats.
Topics: Animals; Cardiomyopathies; Carnitine; Diastole; Heart; Heart Diseases; Heart Rate; Hyperammonemia; I | 2016 |
Hemodialysis Associated with Severe and Unpredictable Hypoglycemia.
Topics: Aged; Autopsy; Blood Glucose; Cardiomyopathies; Carnitine; Diabetes Mellitus, Type 2; Fatal Outcome; | 2016 |
Lipolysis and lipophagy in lipid storage myopathies.
Topics: Adolescent; Adult; Aged; Autophagy; Cardiomyopathies; Carnitine; Carnitine O-Palmitoyltransferase; C | 2016 |
The cardioprotective effects of L-carnitine on rat cardiac injury, apoptosis, and oxidative stress caused by amethopterin.
Topics: Animals; Antineoplastic Agents; Apoptosis; Cardiomyopathies; Cardiotonic Agents; Cardiotoxicity; Car | 2016 |
Primary Carnitine Deficiency - A Rare Treatable Cause of Cardiomyopathy and Massive Hepatomegaly.
Topics: Cardiomyopathies; Carnitine; Hepatomegaly; Humans; Hyperammonemia; Infant; Male; Muscular Diseases | 2017 |
Dilated Cardiomyopathy as the Only Clinical Manifestation of Carnitine Transporter Deficiency.
Topics: Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Child, Preschool; Diagnosis, Differential; Fem | 2017 |
Round Table Discussion.
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.
Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Boston; Cardiomyopath | 2017 |
Mitochondrial trifunctional protein deficiency: an adult patient with similar progress to Charcot-Marie-Tooth disease.
Topics: Biomarkers; Cardiomyopathies; Carnitine; Charcot-Marie-Tooth Disease; Diagnosis, Differential; Disea | 2017 |
[Mutational analysis of SLC22A5 gene in eight patients with systemic primary carnitine deficiency].
Topics: Adult; Amino Acid Sequence; Base Sequence; Cardiomyopathies; Carnitine; DNA Mutational Analysis; Fem | 2017 |
Exome sequencing identifies primary carnitine deficiency in a family with cardiomyopathy and sudden death.
Topics: Adult; Cardiomyopathies; Carnitine; Codon, Terminator; Death, Sudden; Exome; Female; Humans; Infant; | 2017 |
Anesthetic management of a patient with 3-methylcrotonyl-CoA carboxylase deficiency.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Anesthesia, General; Carbon-Carbon Ligases; Cardiomyopa | 2008 |
Positive inotropic effect of coenzyme Q10, omega-3 fatty acids and propionyl-L-carnitine on papillary muscle force-frequency responses of BIO TO-2 cardiomyopathic Syrian hamsters.
Topics: Animals; Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Cricetinae; Fatty Acids, Omega-3; Mal | 2008 |
Two novel HADHB gene mutations in a Korean patient with mitochondrial trifunctional protein deficiency.
Topics: 3-Hydroxyacyl CoA Dehydrogenases; Cardiomyopathies; Carnitine; DNA Mutational Analysis; Humans; Infa | 2009 |
Carnitine deficiency aggravates cyclophosphamide-induced cardiotoxicity in rats.
Topics: Acetyl Coenzyme A; Adenosine Triphosphate; Animals; Antineoplastic Agents, Alkylating; Cardiomyopath | 2010 |
Use of a long-chain triglyceride-restricted/medium-chain triglyceride-supplemented diet in a case of malonyl-CoA decarboxylase deficiency with cardiomyopathy.
Topics: Angiotensin-Converting Enzyme Inhibitors; Captopril; Carboxy-Lyases; Cardiomyopathies; Carnitine; Ch | 2010 |
Glycosylation of the OCTN2 carnitine transporter: study of natural mutations identified in patients with primary carnitine deficiency.
Topics: Amino Acid Substitution; Animals; Biological Transport; Blotting, Western; Cardiomyopathies; Carniti | 2011 |
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.
Topics: Animals; Cardiomyopathies; Carnitine; Disease Models, Animal; Female; Glucose; Hyperammonemia; Male; | 2011 |
Genotype-phenotype correlation in primary carnitine deficiency.
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.
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.
Topics: Adult; Cardiomyopathies; Cardiomyopathy, Hypertrophic; Carnitine; Dietary Supplements; Follow-Up Stu | 2012 |
Acquired encephalopathy associated with carnitine deficiency after cefditoren pivoxil administration.
Topics: Anti-Bacterial Agents; Brain Diseases, Metabolic; Cardiomyopathies; Carnitine; Cephalosporins; Diagn | 2012 |
[A cause of dilated cardiomyopathy in a child: primary carnitine deficiency].
Topics: Biomarkers; Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Child, Preschool; Dyspnea; Humans; | 2014 |
Tissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathy.
Topics: Adenosine Triphosphate; Animals; Animals, Newborn; Blotting, Western; Cardiomyopathies; Carnitine; C | 2012 |
Primary carnitine deficiency and pivalic acid exposure causing encephalopathy and fatal cardiac events.
Topics: Adult; Anti-Bacterial Agents; Arrhythmias, Cardiac; Cardiomyopathies; Carnitine; Child, Preschool; D | 2013 |
Primary carnitine deficiency dilated cardiomyopathy: 28 years follow-up.
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.
Topics: Adult; Aged; Aged, 80 and over; Cardiomyopathies; Carnitine; Dietary Supplements; Female; Follow-Up | 2013 |
Inhibition of gene expression of organic cation/carnitine transporter and antioxidant enzymes in oxazaphosphorines-induced acute cardiomyopathic rat models.
Topics: Animals; Cardiomyopathies; Carnitine; Creatine Kinase; Cyclophosphamide; Gene Expression; Ifosfamide | 2012 |
Malonyl coenzyme A decarboxylase deficiency: early dietary restriction and time course of cardiomyopathy.
Topics: Alleles; Carboxy-Lyases; Cardiomyopathies; Carnitine; Chromosome Aberrations; Chromosome Deletion; C | 2012 |
[Primary carnitine deficiency in 17 patients: diagnosis, treatment and follow up].
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.
Topics: Cardiomyopathies; Carnitine; Cleft Lip; Cleft Palate; Energy Metabolism; Humans; Hyperammonemia; Inf | 2013 |
SLC22A5 mutations in a patient with systemic primary carnitine deficiency: the first Korean case confirmed by biochemical and molecular investigation.
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.
Topics: Biological Transport; Cardiomyopathies; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyl | 2013 |
[Clinical presentation and therapeutic outcomes of carnitine deficiency-induced cardiomyopathy].
Topics: Adolescent; Cardiomyopathies; Cardiotonic Agents; Carnitine; Child; Child, Preschool; Electrocardiog | 2012 |
[Primary carnitine deficiency in a neonate].
Topics: Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Infant, Newborn; Male; Muscular Diseases | 2013 |
Primary carnitine deficiency: novel mutations and insights into the cardiac phenotype.
Topics: Base Sequence; Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; Child; Child, Preschool; Codon, | 2014 |
Novel OCTN2 mutations: no genotype-phenotype correlations: early carnitine therapy prevents cardiomyopathy.
Topics: Cardiomyopathies; Carnitine; Carrier Proteins; Child; Child, Preschool; Female; Heart; Humans; Male; | 2002 |
Very long-chain acyl-CoA dehydrogenase deficiency in an infant presenting with massive hepatomegaly.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Cardiomyopathies; Carnitine; Diet; Gastroenteritis; Hepatomegaly | 2002 |
Differential long-term subcellular responses in heart and liver to adriamycin stress. Exogenous L-carnitine cardiac and hepatic protection.
Topics: Administration, Oral; Animals; Antineoplastic Agents; Cardiomyopathies; Carnitine; Chemical and Drug | 2002 |
Effect of L-carnitine supplementation on cardiac carnitine palmitoyltransferase activities and plasma carnitine concentrations in adriamycin-treated rats.
Topics: Animals; Antibiotics, Antineoplastic; Cardiomyopathies; Carnitine; Carnitine O-Palmitoyltransferase; | 2003 |
The effect of zinc and potassium in the nutrition of Tenebrio molitor, with observations on the expression of a carnitine deficiency.
Topics: Animals; Cardiomyopathies; Carnitine; Humans; Hyperammonemia; Muscular Diseases; Potassium; Tenebrio | 1958 |
Phenotypic manifestations of the OCTN2 V295X mutation: sudden infant death and carnitine-responsive cardiomyopathy in Roma families.
Topics: Amino Acid Sequence; Base Sequence; Cardiomyopathies; Carnitine; Child, Preschool; DNA; DNA Mutation | 2004 |
Antimony-induced cardiomyopathy in guinea-pig and protection by L-carnitine.
Topics: Action Potentials; Animals; Antimony; Cardiomyopathies; Cardiotonic Agents; Carnitine; Drug Administ | 2005 |
Accumulation of long-chain acylcarnitine and 3-hydroxy acylcarnitine molecular species in diabetic myocardium: identification of alterations in mitochondrial fatty acid processing in diabetic myocardium by shotgun lipidomics.
Topics: Animals; Base Sequence; Cardiomyopathies; Carnitine; Cricetinae; Diabetes Mellitus, Experimental; DN | 2005 |
Cardiac carnitine leakage is promoted by cardiomyopathy.
Topics: Adult; Aged; Cardiac Catheterization; Cardiomyopathies; Carnitine; Heart; Humans; Male; Middle Aged; | 2005 |
Biochemical, clinical and molecular findings in LCHAD and general mitochondrial trifunctional protein deficiency.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Cardiomyopathies; Carnitine; Exons; Fatty Acids; Fibroblasts; Ho | 2005 |
Clinical, pathological, and biochemical studies in a patient with propionic acidemia and fatal cardiomyopathy.
Topics: Amino Acid Metabolism, Inborn Errors; Cardiomyopathies; Carnitine; Child; Electrocardiography; Fatal | 2005 |
Propionyl-L-carnitine prevents the progression of cisplatin-induced cardiomyopathy in a carnitine-depleted rat model.
Topics: Adenosine Triphosphate; Animals; Cardiomyopathies; Carnitine; Cisplatin; Disease Models, Animal; Glu | 2006 |
Asymptomatic cardiomyopathy in children and adolescents with type 1 diabetes mellitus: association of echocardiographic indicators with duration of diabetes mellitus and metabolic parameters.
Topics: Adolescent; Adult; Blood Glucose; Cardiomyopathies; Carnitine; Child; Child, Preschool; Cholesterol; | 2006 |
Differential expression of HSP70 and ultrastructure of heart and liver tissues of rats treated with adriamycin: protective role of L-carnitine.
Topics: Animals; Cardiomyopathies; Cardiotonic Agents; Carnitine; Doxorubicin; Drug Evaluation, Preclinical; | 2007 |
Pressure overload-induced cardiomyopathy in heterozygous carrier mice of carnitine transporter gene mutation.
Topics: Adenosine Triphosphate; Animals; Aorta; Atrial Natriuretic Factor; Blood Pressure; Cardiomegaly; Car | 2007 |
Carnitine membrane transporter deficiency: a rare treatable cause of cardiomyopathy and anemia.
Topics: Anemia, Iron-Deficiency; Cardiomyopathies; Carnitine; Humans; Infant; Male; Metabolism, Inborn Error | 2008 |
Cardiomyopathy and carnitine deficiency.
Topics: Animals; Cardiomyopathies; Carnitine; CHO Cells; Cricetinae; Cricetulus; Exons; Gene Expression; Het | 2008 |
An X-linked mitochondrial disease affecting cardiac muscle, skeletal muscle and neutrophil leucocytes.
Topics: Adenosine Triphosphatases; Adult; Aged; Ca(2+) Mg(2+)-ATPase; Cardiomyopathies; Carnitine; Cytochrom | 1983 |
[Uremic cardiomyopathy].
Topics: Calcium; Calcium Phosphates; Cardiomegaly; Cardiomyopathies; Carnitine; Humans; Hyperparathyroidism; | 1981 |
Mitochondrial cardiomyopathy with involvement of skeletal muscles.
Topics: Autopsy; Cardiomyopathies; Carnitine; Female; Glycogen Storage Disease Type II; Humans; Infant; Micr | 1983 |
Hamster cardiomyopathy. A defect in oxidative phosphorylation in the cardiac interfibrillar mitochondria.
Topics: Adenosine Triphosphatases; Animals; Cardiomyopathies; Carnitine; Coenzyme A; Cricetinae; Hydrolases; | 1982 |
Plasma carnitine concentrations in cardiomyopathy patients.
Topics: Adolescent; Adult; Cardiomyopathies; Cardiomyopathy, Dilated; Cardiomyopathy, Hypertrophic; Carnitin | 1984 |
Hypoglycemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain acylcarnitine excess responsive to medium chain triglyceride diet.
Topics: Cardiomyopathies; Carnitine; Dietary Fats; Diseases in Twins; Female; Humans; Hypoglycemia; Infant; | 1983 |
Effects of L-carnitine on adriamycin-induced cardiomyopathy in rabbit.
Topics: Animals; Cardiomyopathies; Carnitine; Doxorubicin; Male; Mitochondria, Heart; Myocardium; Rabbits | 1984 |
["Metabolic" myocardiopathies in childhood (author's transl)].
Topics: Cardiomyopathies; Carnitine; Diabetes Mellitus; Echocardiography; Glycogen Storage Disease; Humans; | 1981 |
[Myocardiopathy with lipid overload and leukocyte palmityl carnitine transferase (PCT) deficiency].
Topics: Acyltransferases; Adult; Cardiomyopathies; Carnitine; Carnitine O-Palmitoyltransferase; Humans; Leuk | 1982 |
Cardiac carnitine deficiency and altered carnitine transport in cardiomyopathic hamsters.
Topics: Animals; Biological Transport; Cardiomyopathies; Carnitine; Cricetinae; Disease Models, Animal; Meso | 1983 |
[Myocardial disease caused by adriamycin. Experimental animal models and possible pharmacologic prevention].
Topics: Animals; Cardiomyopathies; Carnitine; Disease Models, Animal; Doxorubicin; Mice; Rabbits; Rats; Razo | 1982 |
[Carnitine deficiency: a treatable cause of cardiomyopathy in children (author's transl)].
Topics: Cardiomyopathies; Carnitine; Child, Preschool; Female; Humans; Infant; Lipid Metabolism; Male; Micro | 1982 |
Carnitine deficiency presenting as familial cardiomyopathy: a treatable defect in carnitine transport.
Topics: Cardiomyopathies; Carnitine; Child, Preschool; Diagnosis, Differential; Heart Failure; Humans; Male | 1982 |
[Systemic carnitine deficiency: its place in Reye's syndrome (author's transl)].
Topics: Cardiomyopathies; Carnitine; Child, Preschool; Diagnosis, Differential; Hepatic Encephalopathy; Huma | 1981 |
"Carnitine deficient" myopathy and cardiomyopathy with fatal outcome.
Topics: Adolescent; Anesthesia, General; Cardiomyopathies; Carnitine; Carnitine O-Palmitoyltransferase; Hear | 1980 |
Six years' experience with carnitine supplementation in a patient with an inherited defective carnitine transport system.
Topics: Biological Transport; Cardiomyopathies; Cardiotonic Agents; Carnitine; Cells, Cultured; Fibroblasts; | 1995 |
[Carnitine-deficient cardiomyopathy].
Topics: Administration, Oral; Cardiomyopathies; Carnitine; Echocardiography, Doppler; Female; Humans; Infant | 1994 |
[Cardiomyopathy caused by carnitine deficiency].
Topics: Cardiomyopathies; Carnitine; Humans; Infant; Male; Prognosis | 1993 |
Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency--diagnosis, plasma carnitine fractions and management in a further patient.
Topics: 3-Hydroxyacyl CoA Dehydrogenases; Cardiomyopathies; Carnitine; Female; Gastrointestinal Diseases; Hu | 1993 |
The role of L-carnitine in pediatric cardiomyopathy.
Topics: Cardiomyopathies; Carnitine; Child; Child, Preschool; Female; Humans; Infant; Male; Retrospective St | 1995 |
Increased expression of carnitine palmitoyltransferase I gene is repressed by administering L-carnitine in the hearts of carnitine-deficient juvenile visceral steatosis mice.
Topics: Amino Acid Sequence; Animals; Base Sequence; Brain; Cardiomyopathies; Carnitine; Carnitine O-Palmito | 1996 |
Isolated isobutyryl-CoA dehydrogenase deficiency: an unrecognized defect in human valine metabolism.
Topics: Cardiomyopathies; Carnitine; Child, Preschool; Female; Fibroblasts; Humans; Infant; Male; Oxidoreduc | 1998 |
Carnitine promotes heat shock protein synthesis in adriamycin-induced cardiomyopathy in a neonatal rat experimental model.
Topics: Animals; Antibiotics, Antineoplastic; Cardiomyopathies; Carnitine; Doxorubicin; Drug Antagonism; Fem | 1998 |
Reversal of doxorubicin-induced cardiac metabolic damage by L-carnitine.
Topics: Animals; Antineoplastic Agents; Carcinoma, Ehrlich Tumor; Cardiomyopathies; Carnitine; Cell Division | 1999 |
Diagnosis of mitochondrial trifunctional protein deficiency in a blood spot from the newborn screening card by tandem mass spectrometry and DNA analysis.
Topics: 3-Hydroxyacyl CoA Dehydrogenases; Base Sequence; Cardiomyopathies; Carnitine; DNA; Female; Genetic T | 1999 |
Very long chain acyl coenzyme A dehydrogenase deficiency in a 5-month-old Korean boy: identification of a novel mutation.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Arginine; Cardiomyopathies; Carnitine; Codon, Terminator; Fatal | 1999 |
Metabolic aspects of myocardial disease and a role for L-carnitine in the treatment of childhood cardiomyopathy.
Topics: Cardiomyopathies; Carnitine; Child; Child, Preschool; Dietary Supplements; Female; Humans; Male; Ret | 2000 |
Propionyl-L-carnitine as protector against adriamycin-induced cardiomyopathy.
Topics: Animals; Antineoplastic Agents; Carcinoma, Ehrlich Tumor; Cardiomyopathies; Cardiotonic Agents; Carn | 2001 |
Characterization of altered myocardial fatty acid metabolism in patients with inherited cardiomyopathy.
Topics: Acetates; Adolescent; Adult; Cardiomyopathies; Carnitine; Child; Child, Preschool; Coronary Circulat | 2001 |
Serum lipid and fatty acid profiles in adriamycin-treated rats after administration of L-carnitine.
Topics: Animals; Antibiotics, Antineoplastic; Cardiomyopathies; Carnitine; Cholesterol; Doxorubicin; Fatty A | 2002 |
Myocardial and hepatic free carnitine concentrations in pups of diabetic female rats.
Topics: Animals; Animals, Newborn; Cardiomyopathies; Carnitine; Diabetes Mellitus, Experimental; Dietary Sup | 2002 |
Age-associated cardiomyopathy in heterozygous carrier mice of a pathological mutation of carnitine transporter gene, OCTN2.
Topics: Adult; Aging; Animals; Cardiomyopathies; Carnitine; Carrier Proteins; Female; Glucose; Heterozygote; | 2002 |
Carnitine deficiency induced during intermittent haemodialysis for renal failure.
Topics: Cardiomyopathies; Carnitine; Humans; Kidney Failure, Chronic; Muscles; Myocardium; Renal Dialysis | 1978 |
[Lipidic myopathy with severe cardiomyopathy caused by a generalized carnitine deficiency. Favourable course during carnitine hydrochloride treatment].
Topics: Biopsy; Cardiomyopathies; Carnitine; Child; Female; Follow-Up Studies; Humans; Lipid Metabolism; Lip | 1979 |
Failure of carnitine to prevent adriamycin cardiomyopathy.
Topics: Animals; Cardiomyopathies; Carnitine; Doxorubicin; Kidney; Rabbits | 1979 |
Muscle carnitine deficiency and fatal cardiomyopathy.
Topics: Cardiomyopathies; Carnitine; Child, Preschool; Humans; Male; Muscles; Muscular Diseases; Myocardium | 1978 |
Carnitine effect on heart steatosis induced in rats by rapeseed oil.
Topics: Animals; Cardiomyopathies; Carnitine; Cholesterol; Dietary Fats; Erucic Acids; Fatty Acids; Lipidose | 1977 |
Effect of propionyl-L-carnitine on experimental induced cardiomyopathy in rats.
Topics: Animals; Blood Pressure; Cardiomyopathies; Carnitine; Diabetes Mellitus, Experimental; Erucic Acids; | 1992 |
Amelioration of cardiac function by L-carnitine administration in patients on haemodialysis.
Topics: Adult; Cardiomyopathies; Carnitine; Heart; Humans; Renal Dialysis; Stroke Volume | 1992 |
L-carnitine treatment for congestive heart failure--experimental and clinical study.
Topics: Adult; Angina Pectoris; Animals; Cardiomyopathies; Carnitine; Chronic Disease; Cricetinae; Drug Eval | 1992 |
Effect of prolonged treatment with propionyl-L-carnitine on erucic acid-induced myocardial dysfunction in rats.
Topics: Adenosine Triphosphate; Animals; Cardiomyopathies; Carnitine; Erucic Acids; Heart; Lipid Metabolism; | 1992 |
Neonatal cardiomyopathy and lactic acidosis responsive to thiamine.
Topics: Acidosis, Lactic; Cardiomyopathies; Carnitine; Child; Child, Preschool; Female; Humans; Infant; Keto | 1991 |
Primary carnitine deficiency: heterozygote and intrafamilial phenotypic variation.
Topics: Cardiomyopathies; Carnitine; Child; Child, Preschool; Genetic Variation; Heterozygote; Humans; Male; | 1991 |
Carnitine deficiency with cardiomyopathy presenting as neonatal hydrops: successful response to carnitine therapy.
Topics: Cardiomyopathies; Carnitine; Humans; Hydrops Fetalis; Infant, Newborn; Infant, Small for Gestational | 1990 |
[L-carnitine therapy and myocardial function in children treated with chronic hemodialysis].
Topics: Administration, Oral; Adolescent; Cardiac Output; Cardiomyopathies; Carnitine; Child; Child, Prescho | 1989 |
Value of endomyocardial biopsy.
Topics: Amyloid; Biopsy; Calcium-Transporting ATPases; Cardiomyopathies; Cardiomyopathy, Dilated; Carnitine; | 1986 |
Experimental uremic cardiomyopathy--fact or fiction?
Topics: Animals; Carbachol; Cardiomegaly; Cardiomyopathies; Carnitine; Catecholamines; Isometric Contraction | 1986 |
[Familial cardiomyopathy caused by carnitine deficiency].
Topics: Adolescent; Cardiomyopathies; Carnitine; Child, Preschool; Female; Humans; Male | 1986 |
[Primary cardiomyopathies and disorders of myocardial energy metabolism: causes or consequences?].
Topics: Animals; Antibiotics, Antineoplastic; Cardiomyopathies; Cardiomyopathy, Alcoholic; Cardiomyopathy, D | 1988 |
Cardiomyopathy associated with carnitine loss in kidneys and small intestine.
Topics: Cardiomyopathies; Carnitine; Child, Preschool; Humans; Infant; Intestine, Small; Kidney; Male; Mitoc | 1988 |
Carnitine metabolism. Role in acute ischemia and chronic myocardial disease.
Topics: Cardiomyopathies; Carnitine; Coronary Disease; Humans; Myocardium | 1987 |
Protection from adriamycin-induced cardiomyopathy in rats.
Topics: Animals; Cardiomyopathies; Carnitine; Doxorubicin; Male; Myocardium; Perfusion; Rats; Rats, Inbred S | 1987 |
Plasma carnitine deficiency. Clinical observations in 51 pediatric patients.
Topics: Adolescent; Brain Diseases; Cardiomyopathies; Carnitine; Child; Child, Preschool; Failure to Thrive; | 1987 |
L-carnitine treatment improves cardiac performance and restores high-energy phosphate pools in cardiomyopathic Syrian hamster.
Topics: Adenine Nucleotides; Animals; Cardiomyopathies; Carnitine; Coenzyme A; Cricetinae; Heart; Hemodynami | 1987 |
Muscle carnitine deficiency presenting as familial fatal cardiomyopathy.
Topics: Cardiomyopathies; Carnitine; Child, Preschool; Female; Humans; Infant; Male; Microscopy, Electron; M | 1987 |
[Carnitine deficiency: a treatable cardiomyopathy].
Topics: Cardiomyopathies; Carnitine; Child; Child, Preschool; Humans; Male | 1986 |
Effects of L-carnitine and verapamil on myocardial carnitine concentration and histopathology of Syrian hamster BIO 14.6.
Topics: Animals; Calcinosis; Cardiomyopathies; Carnitine; Cricetinae; Fibrosis; Mesocricetus; Myocardium; Ne | 1986 |
Carnitine protection against adriamycin-induced cardiomyopathy in rats.
Topics: Animals; Cardiac Output; Cardiomyopathies; Carnitine; Doxorubicin; Male; Microscopy, Electron; Myoca | 1986 |
Successful carnitine treatment in two siblings having lipid storage myopathy with hypertrophic cardiomyopathy.
Topics: Cardiomyopathies; Carnitine; Child; Child, Preschool; Humans; Lipidoses; Male; Muscles; Muscular Dis | 1985 |
omega-123I-hexadecanoic acid metabolic probe of cardiomyopathy.
Topics: Adolescent; Adult; Aged; Alcoholism; Atrial Fibrillation; Cardiomyopathies; Cardiomyopathy, Dilated; | 1985 |
Carnitine derivatives in hereditary cardiomyopathic animals.
Topics: Animals; Cardiomyopathies; Carnitine; Cricetinae; Heart Ventricles; Liver; Mice; Mice, Mutant Strain | 1985 |
Depressed fatty acid and acetate oxidation and other metabolic defects in homogenates from hearts of hamsters with hereditary cardiomyopathy.
Topics: Acetates; Acyltransferases; Adenosine Triphosphatases; Animals; Butyrates; Caprylates; Carbon Dioxid | 1974 |
Oxidative phosphorylation in cardiomyopathic hamsters.
Topics: Adenosine Diphosphate; Animals; Cardiomyopathies; Carnitine; Cricetinae; Glutamates; Heart Failure; | 1972 |
Protective effect of L-carnitine in experimental intoxication with diphtheria toxin.
Topics: Animals; Arteriovenous Fistula; Blood Pressure; Cardiac Catheterization; Cardiac Output; Cardiomyopa | 1971 |