carnitine has been researched along with Luft Disease in 38 studies
| Excerpt | Relevance | Reference |
|---|---|---|
| "To assess the role of carnitine in the skeletal myopathy present in anorexia nervosa." | 7.70 | Carnitine levels in patients with skeletal myopathy due to anorexia nervosa before and after refeeding. ( McLoughlin, DM; Morton, J; Russell, GF; Whiting, S, 1999) |
| "To assess the role of carnitine in the skeletal myopathy present in anorexia nervosa." | 3.70 | Carnitine levels in patients with skeletal myopathy due to anorexia nervosa before and after refeeding. ( McLoughlin, DM; Morton, J; Russell, GF; Whiting, S, 1999) |
| "The accumulation of arrhythmogenic intermediary metabolites of fatty acids, such as long-chain acylcarnitines, may be responsible for arrhythmias." | 3.70 | Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children. ( Bonnet, D; Brivet, M; Jouvet, P; Martin, D; Rabier, D; Saudubray, JM; Villain, E, 1999) |
| "The use of zidovudine (AZT) for the treatment of acquired immunodeficiency syndrome (AIDS) induces a DNA-depleting mitochondrial myopathy, which is histologically characterized by the presence of muscle fibers with "ragged-red"-like features, red-rimmed or empty cracks, granular degeneration, and rods (AZT fibers)." | 3.69 | Zidovudine-induced mitochondrial myopathy is associated with muscle carnitine deficiency and lipid storage. ( Bernardini, I; Dalakas, MC; Gahl, WA; Jay, CA; Leon-Monzon, ME, 1994) |
| "glycogenosis type II and III), lysosomal storage diseases (e." | 2.41 | Metabolic cardiomyopathies. ( Guertl, B; Hoefler, G; Noehammer, C, 2000) |
| "Although the Friedreich's ataxia phenotype results from decreased expression of a mitochondrially targeted protein, frataxin, mitochondrial myopathy has not been described as a feature of the disease." | 1.31 | Friedreich's ataxia associated with mitochondrial myopathy: clinicopathologic report. ( Beinlich, BR; Gallagher, CL; Harding, CO; Pandolfo, M; Pauli, RM; Poirer, J; Salamat, MS; Waclawik, AJ, 2002) |
| "Barth syndrome is an X-linked recessive condition characterized by skeletal myopathy, cardiomyopathy, proportionate short stature, and recurrent neutropenia, but with normal cognitive function." | 1.29 | Barth syndrome: clinical observations and genetic linkage studies. ( Becker, LE; Bridge, PJ; Christodoulou, J; Clarke, JT; Jay, V; Lehotay, DC; McInnes, RR; Platt, BA; Robinson, BH; Wilson, G, 1994) |
| "Treatment with riboflavin resulted in a clear clinical improvement in the two patients with the myopathic form of complex I deficiency." | 1.29 | Treatment of complex I deficiency with riboflavin. ( Bernsen, PL; Gabreëls, FJ; Hamburger, HL; Ruitenbeek, W, 1993) |
| "Six men and 7 women with inflammatory myopathy and 25 age-matched healthy controls were studied." | 1.29 | Abnormal carnitine distribution in the muscles of patients with idiopathic inflammatory myopathy. ( Arenas, J; Cabello, A; Campos, Y; Gomez-Reino, JJ; Gonzalez-Crespo, MR; Martin, MA, 1996) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 19 (50.00) | 18.2507 |
| 2000's | 11 (28.95) | 29.6817 |
| 2010's | 7 (18.42) | 24.3611 |
| 2020's | 1 (2.63) | 2.80 |
| Authors | Studies |
|---|---|
| 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 |
| Gillingham, MB | 1 |
| Heitner, SB | 1 |
| Martin, J | 1 |
| Rose, S | 1 |
| Goldstein, A | 1 |
| El-Gharbawy, AH | 1 |
| Deward, S | 1 |
| Lasarev, MR | 1 |
| Pollaro, J | 1 |
| DeLany, JP | 1 |
| Burchill, LJ | 1 |
| Goodpaster, B | 1 |
| Shoemaker, J | 1 |
| Matern, D | 1 |
| Harding, CO | 2 |
| Vockley, J | 2 |
| Vissing, CR | 1 |
| Dunø, M | 1 |
| Wibrand, F | 1 |
| Christensen, M | 1 |
| Vissing, J | 1 |
| Haglind, CB | 1 |
| Nordenström, A | 1 |
| Ask, S | 1 |
| von Döbeln, U | 1 |
| Gustafsson, J | 1 |
| Stenlid, MH | 1 |
| Gimenes, AC | 1 |
| Bravo, DM | 1 |
| Nápolis, LM | 1 |
| Mello, MT | 1 |
| Oliveira, AS | 1 |
| Neder, JA | 1 |
| Nery, LE | 1 |
| Internullo, M | 1 |
| Bonini, M | 1 |
| Marinelli, P | 1 |
| Perli, E | 1 |
| Cerbelli, B | 1 |
| Palange, P | 1 |
| Yamamoto, Y | 1 |
| Matsui, N | 1 |
| Hiramatsu, Y | 1 |
| Miyazaki, Y | 1 |
| Nodera, H | 1 |
| Izumi, Y | 1 |
| Takashima, H | 1 |
| Kaji, R | 1 |
| Ogundare, O | 1 |
| Jumma, O | 1 |
| Turnbull, DM | 2 |
| Woywodt, A | 1 |
| Park, HD | 1 |
| Kim, SR | 1 |
| Ki, CS | 1 |
| Lee, SY | 1 |
| Chang, YS | 1 |
| Jin, DK | 1 |
| Park, WS | 1 |
| Listernick, R | 1 |
| Gallagher, CL | 1 |
| Waclawik, AJ | 1 |
| Beinlich, BR | 1 |
| Pauli, RM | 1 |
| Poirer, J | 1 |
| Pandolfo, M | 1 |
| Salamat, MS | 1 |
| Wasant, P | 1 |
| Matsumoto, I | 1 |
| Naylor, E | 1 |
| Liammongkolkul, S | 1 |
| Scaglia, F | 1 |
| Scheuerle, AE | 1 |
| Towbin, JA | 1 |
| Armstrong, DL | 1 |
| Sweetman, L | 1 |
| Wong, LJ | 1 |
| Bartlett, K | 1 |
| Pourfarzam, M | 2 |
| Walker, UA | 1 |
| Auré, K | 1 |
| Benoist, JF | 1 |
| Ogier de Baulny, H | 1 |
| Romero, NB | 1 |
| Rigal, O | 1 |
| Lombès, A | 1 |
| Olsen, RK | 1 |
| Olpin, SE | 1 |
| Andresen, BS | 1 |
| Miedzybrodzka, ZH | 1 |
| Merinero, B | 1 |
| Frerman, FE | 1 |
| Beresford, MW | 1 |
| Dean, JC | 1 |
| Cornelius, N | 1 |
| Andersen, O | 1 |
| Oldfors, A | 1 |
| Holme, E | 1 |
| Gregersen, N | 1 |
| Morris, AA | 1 |
| Semino-Mora, MC | 1 |
| Leon-Monzon, ME | 2 |
| Dalakas, MC | 2 |
| Largillière, C | 1 |
| Vianey-Saban, C | 1 |
| Fontaine, M | 1 |
| Bertrand, C | 1 |
| Kacet, N | 1 |
| Farriaux, JP | 1 |
| Sewell, AC | 1 |
| Bender, SW | 1 |
| Wirth, S | 1 |
| Münterfering, H | 1 |
| Ijlist, L | 1 |
| Wanders, RJ | 1 |
| Campos, Y | 4 |
| Arenas, J | 3 |
| Christodoulou, J | 2 |
| McInnes, RR | 1 |
| Jay, V | 1 |
| Wilson, G | 1 |
| Becker, LE | 2 |
| Lehotay, DC | 1 |
| Platt, BA | 1 |
| Bridge, PJ | 1 |
| Robinson, BH | 1 |
| Clarke, JT | 1 |
| Bernardini, I | 1 |
| Gahl, WA | 1 |
| Jay, CA | 1 |
| Bernsen, PL | 1 |
| Gabreëls, FJ | 1 |
| Ruitenbeek, W | 1 |
| Hamburger, HL | 1 |
| Reichmann, H | 1 |
| Huertas, R | 2 |
| Lorenzo, G | 2 |
| Bautista, J | 2 |
| Gutiérrez, E | 2 |
| Aparicio, M | 2 |
| Alesso, L | 2 |
| Segura, D | 1 |
| Villanueva, M | 1 |
| Cabello, A | 2 |
| Wada, H | 1 |
| Nishio, H | 1 |
| Nagaki, S | 1 |
| Yanagawa, H | 1 |
| Imamura, A | 1 |
| Yokoyama, S | 1 |
| Sano, T | 1 |
| Woo, M | 1 |
| Matsuo, M | 1 |
| Itoh, H | 1 |
| Nakamura, H | 1 |
| Gonzalez-Crespo, MR | 1 |
| Martin, MA | 1 |
| Gomez-Reino, JJ | 1 |
| Ogle, RF | 1 |
| Fagan, E | 1 |
| Blok, RB | 1 |
| Kirby, DM | 1 |
| Seller, KL | 1 |
| Dahl, HH | 1 |
| Thorburn, DR | 1 |
| Muñoz-Blanco, JL | 1 |
| Castro-Gago, M | 1 |
| Novo-Rodríguez, MI | 1 |
| Eirís-Puñal, J | 1 |
| Tein, I | 1 |
| Haslam, RH | 1 |
| Rhead, WJ | 1 |
| Bennett, MJ | 1 |
| Skyllouriotis, ML | 1 |
| Marx, M | 1 |
| Skyllouriotis, P | 1 |
| Bittner, R | 1 |
| Wimmer, M | 1 |
| Morton, J | 1 |
| McLoughlin, DM | 1 |
| Whiting, S | 1 |
| Russell, GF | 1 |
| Bonnet, D | 1 |
| Martin, D | 1 |
| Villain, E | 1 |
| Jouvet, P | 1 |
| Rabier, D | 1 |
| Brivet, M | 1 |
| Saudubray, JM | 1 |
| Winter, SC | 1 |
| Buist, NR | 1 |
| Guertl, B | 1 |
| Noehammer, C | 1 |
| Hoefler, G | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| 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 | ||
| 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) | ||
| Estudio clínico Fase III Para Evaluar la Eficacia terapéutica en Pacientes Mexicanos Con Dislipidemia Mediante el Uso vía Oral de L-Carnitina + Atorvastatina Comparado Con Atorvastatina[NCT03696940] | Phase 3 | 120 participants (Actual) | Interventional | 2018-05-28 | Active, not recruiting | ||
| [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 |
8 reviews available for carnitine and Luft Disease
| Article | Year |
|---|---|
Defects of beta-oxidation including carnitine deficiency.
Topics: Biological Transport; Carnitine; Carnitine O-Palmitoyltransferase; Deficiency Diseases; Humans; Meta | 2002 |
[Inherited and acquired disorders of mitochondrial DNA].
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Anti-HIV Agents; Cardiomyopathies; Carnitine; Child; Dia | 2002 |
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 |
[Therapy of metabolic myopathies].
Topics: Carbohydrate Metabolism, Inborn Errors; Carnitine; Carnitine O-Palmitoyltransferase; Electron Transp | 1993 |
[Lipidic myopathies].
Topics: Carnitine; Carnitine O-Palmitoyltransferase; Electron Transport; Fatty Acid Desaturases; Humans; Lip | 1998 |
[Treatment of mitochondrial diseases in childhood and adolescence].
Topics: Adolescent; Carnitine; Carnitine O-Palmitoyltransferase; Child, Preschool; Humans; Infant; Mitochond | 1998 |
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 |
2 trials available for carnitine and Luft Disease
| Article | Year |
|---|---|
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 |
Effect of L-carnitine on exercise performance in patients with mitochondrial myopathy.
Topics: Adult; Carnitine; Cross-Over Studies; Double-Blind Method; Exercise Test; Exercise Tolerance; Female | 2015 |
28 other studies available for carnitine and Luft Disease
| Article | Year |
|---|---|
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 |
Hydroxylated Long-Chain Acylcarnitines are Biomarkers of Mitochondrial Myopathy.
Topics: Acyl Coenzyme A; Acyl-CoA Dehydrogenase; Adult; Biomarkers; Carnitine; Cohort Studies; Denmark; Fema | 2019 |
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 |
A 22-year-old woman with unexplained exertional dyspnoea.
Topics: Carnitine; Dyspnea; Exercise; Exercise Test; Female; Humans; Mitochondrial Myopathies; Riboflavin; R | 2016 |
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 |
Searching for the needle in the Haystacks.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Age of Onset; Biopsy; Carnitine; Creatine Kinase; Creatinine; Hi | 2009 |
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 |
A 14-year-old boy with vomiting.
Topics: Abdominal Pain; Adolescent; Brain Ischemia; Carnitine; Cognition Disorders; Diagnosis, Differential; | 2012 |
Friedreich's ataxia associated with mitochondrial myopathy: clinicopathologic report.
Topics: Adolescent; Autopsy; Biopsy; Carnitine; Disease Progression; DNA, Mitochondrial; Frataxin; Friedreic | 2002 |
Mitochondrial fatty acid oxidation disorders in Thai infants: a report of 3 cases.
Topics: 3-Hydroxyacyl CoA Dehydrogenases; Cardiomyopathy, Hypertrophic; Carnitine; Fatal Outcome; Fatty Acid | 2002 |
Neonatal presentation of ventricular tachycardia and a Reye-like syndrome episode associated with disturbed mitochondrial energy metabolism.
Topics: Biomarkers; Carnitine; DNA, Mitochondrial; Female; Glutarates; Humans; Infant, Newborn; Mitochondria | 2002 |
Progression despite replacement of a myopathic form of coenzyme Q10 defect.
Topics: Benzoquinones; Carnitine; Cerebellar Ataxia; Cerebellum; Child, Preschool; Disease Progression; Drug | 2004 |
ETFDH mutations as a major cause of riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency.
Topics: Acyl-CoA Dehydrogenase; Adolescent; Adult; Brain Diseases, Metabolic; Carnitine; Child; Child, Presc | 2007 |
Effect of L-carnitine on the zidovudine-induced destruction of human myotubes. Part I: L-carnitine prevents the myotoxicity of AZT in vitro.
Topics: Carnitine; Culture Techniques; Dose-Response Relationship, Drug; Histocytochemistry; Humans; Immunoh | 1994 |
Mitochondrial very long chain acyl-CoA dehydrogenase deficiency--a new disorder of fatty acid oxidation.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Carnitine; Diet, Fat-Restricted; Female; Humans; Infant, Newborn | 1995 |
Muscle carnitine deficiency associated with zidovudine-induced mitochondrial myopathy.
Topics: Carnitine; Humans; Mitochondrial Myopathies; Muscles; Zidovudine | 1994 |
Barth syndrome: clinical observations and genetic linkage studies.
Topics: Abnormalities, Multiple; Acids; Cardiomyopathy, Dilated; Carnitine; Diseases in Twins; Dwarfism; Ele | 1994 |
Zidovudine-induced mitochondrial myopathy is associated with muscle carnitine deficiency and lipid storage.
Topics: Acquired Immunodeficiency Syndrome; Adult; Carnitine; Humans; Lipid Metabolism; Male; Middle Aged; M | 1994 |
Treatment of complex I deficiency with riboflavin.
Topics: Adolescent; Adult; Carnitine; Child; Child, Preschool; Electrophysiology; Female; Humans; Lactates; | 1993 |
Plasma carnitine insufficiency and effectiveness of L-carnitine therapy in patients with mitochondrial myopathy.
Topics: Adolescent; Adult; Carnitine; Child; Child, Preschool; Female; Humans; Male; Middle Aged; Mitochondr | 1993 |
Muscle carnitine deficiency and lipid storage myopathy in patients with mitochondrial myopathy.
Topics: Adolescent; Adult; Carnitine; Child; Child, Preschool; Female; Humans; Infant; Male; Middle Aged; Mi | 1993 |
[Benign infantile mitochondrial myopathy caused by reversible cytochrome c oxidase deficiency].
Topics: Carnitine; Cytochrome-c Oxidase Deficiency; Female; Humans; Infant; Mitochondrial Myopathies; Ubiqui | 1996 |
Abnormal carnitine distribution in the muscles of patients with idiopathic inflammatory myopathy.
Topics: Adolescent; Adult; Aged; Carnitine; Creatine Kinase; Female; Histocytochemistry; Humans; Male; Middl | 1996 |
Mitochondrial myopathy with tRNA(Leu(UUR)) mutation and complex I deficiency responsive to riboflavin.
Topics: Carnitine; Child, Preschool; DNA, Mitochondrial; Female; Humans; Infant; Male; Mitochondrial Myopath | 1997 |
Short-chain acyl-CoA dehydrogenase deficiency: a cause of ophthalmoplegia and multicore myopathy.
Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Adolescent; Carnitine; Cells, Cultured; Diagnosis, | 1999 |
Nemaline myopathy and cardiomyopathy.
Topics: Acyl-CoA Dehydrogenase; Biopsy; Cardiomyopathy, Hypertrophic; Carnitine; Child, Preschool; Diet, Fat | 1999 |
Carnitine levels in patients with skeletal myopathy due to anorexia nervosa before and after refeeding.
Topics: Adult; Anorexia Nervosa; Body Mass Index; Carnitine; Female; Humans; Mitochondrial Myopathies; Muscl | 1999 |
Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children.
Topics: 3-Hydroxyacyl CoA Dehydrogenases; Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Arrhyt | 1999 |