carnitine has been researched along with Fatty Liver with Encephalopathy in 65 studies
| Excerpt | Relevance | Reference |
|---|---|---|
| "Here, we report the case of a 17-year-old girl who presented in both ten months and five years of age a clinical picture characterized by lethargy leading to apnea and coma, hepatomegaly, hypoglycemia, metabolic acidosis, hyperammoniemia, elevated serum transaminases and absence of ketonuria." | 3.70 | [3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome]. ( Castro-Gago, M; Eirís, J; Fernández-Prieto, R; Ribes, A; Rodríguez-García, J; Rodríguez-Segade, S, 1998) |
| "Many inborn errors of metabolism (IEMs) may present as sudden infant death (SID)." | 2.53 | Inborn Errors of Metabolism That Cause Sudden Infant Death: A Systematic Review with Implications for Population Neonatal Screening Programmes. ( Bekhof, J; de Koning, TJ; Derks, TG; Heiner Fokkema, MR; Koolhaas, GD; Schielen, PC; van Rijt, WJ; van Spronsen, FJ; Visser, G, 2016) |
| " Idiosyncratic toxic responses are often not detected during this phase in development due to their relative rarity in incidence and differences in species sensitivity." | 2.41 | Metabolic, idiosyncratic toxicity of drugs: overview of the hepatic toxicity induced by the anxiolytic, panadiplon. ( Bacon, JA; Brass, EP; Cramer, CT; Petrella, DK; Sun, EL; Ulrich, RG, 2001) |
| "Reye syndrome and sudden death symptoms were caused by nimesulide-induced acute metabolic crisis." | 1.48 | [Reye syndrome and sudden death symptoms after oral administration of nimesulide due to upper respiratory tract infection in a boy]. ( Chen, XH; Feng, LF; Jin, Y; Li, DX; Li, XY; Song, JQ; Yang, YL, 2018) |
| "Carnitine is an essential cofactor for the oxidation of fatty acid in the mitochondria and an efficient therapeutics for primary carnitine deficiency." | 1.33 | Prolonged effect of single carnitine administration on fasted carnitine-deficient JVS mice regarding their locomotor activity and energy expenditure. ( Horiuchi, M; Kobayashi, K; Li, MX; Saheki, T; Yoshida, G, 2006) |
| "Free carnitine was measured by enzymatic UV-test." | 1.32 | Serum free carnitine in medium chain acyl-CoA dehydrogenase deficiency. ( Behulova, D; Bzduch, V; Fabriciova, K; Kozak, L; Ponec, J; Salingova, A, 2003) |
| "Riboflavin and carnitine treatment corrected the metabolic abnormalities and she improved clinically." | 1.28 | Electron transfer flavoprotein: ubiquinone oxidoreductase (ETF:QO) deficiency in an adult. ( Bell, RB; Brownell, AK; Engel, AG; Frerman, FE; Goodman, SI; Roe, CR; Seccombe, DW; Snyder, FF, 1990) |
| "In patients diagnosed as Reye syndrome, tissue carnitine deficiency was not always recognized and no decrease in the free/total carnitine ratio was found in the liver or muscle." | 1.28 | Carnitine deficiency in inherited organic acid disorders and Reye syndrome. ( Kidouchi, K; Kobayashi, M; Sugiyama, N; Wada, Y, 1990) |
| "Because of the paucity of causes of Reye's syndrome seen at any one centre, the clinical variability of the disease, and limited knowledge of definite aetiologic factors, controlled clinical trials are not easy to carry out or to interpret in human cases." | 1.28 | Evaluation of the possible role of glucose, carnitine, coenzyme Q10 and steroids in the treatment of Reye's syndrome using the margosa oil animal model. ( Baskaran, G; Pathmanathan, R; Sinniah, D; Sinniah, R; Yamashita, F; Yoshino, M, 1990) |
| "Thus, this disease is revealed by a coma due to hypoglycemia in a young child; the presence of dicarboxylic aciduria in such a situation is the main evidence for this diagnosis." | 1.27 | [Medium chain acyl-CoA dehydrogenase deficiency. Apropos of a case with demonstration of this enzyme deficiency]. ( Blanc, JF; Collet, JP; David, M; Divry, P; Guibaud, P; Hermier, M; Macabeo, V; Vibert, J, 1984) |
| "Third, epidemiologic experience with Reye's syndrome suggests that clusters of cases may occur more frequently when new influenza A or B viruses become epidemic." | 1.27 | From the National Institute of Allergy and Infectious Diseases: Summary of a workshop on disease mechanisms and prospects for prevention of Reye's syndrome. ( La Montagne, JR, 1983) |
| "Carnitine status was evaluated in 8 patients with partial ornithine transcarbamylase (OTC) deficiency and 19 patients with secondary carnitine deficiency, who were used as positive references." | 1.27 | Hyperammonemia related to carnitine metabolism with particular emphasis on ornithine transcarbamylase deficiency. ( Matsuda, I; Ohtani, Y; Ohyanagi, K; Yamamoto, S, 1987) |
| "Families with children who have had Reye syndrome or in which sudden infant death has occurred are at risk for MCAD deficiency." | 1.27 | Recognition of medium-chain acyl-CoA dehydrogenase deficiency in asymptomatic siblings of children dying of sudden infant death or Reye-like syndromes. ( Kinnebrew, P; Maltby, DA; Millington, DS; Roe, CR, 1986) |
| "She developed the typical features of Reye syndrome." | 1.26 | Decreased serum carnitine in valproate induced Reye syndrome. ( Böhles, H; Richter, K; Schäfer, H; Wagner-Thiessen, E, 1982) |
| "The clinical diagnosis of recurrent Reye's syndrome can be made only after a systemic exclusion of other diagnostic possibilities." | 1.26 | Recurrent Reye's syndrome. ( McCabe, ER; Pichichero, ME, 1978) |
| "Reye syndrome is biochemically distinct from the clinically similar syndrome of systemic carnitine deficiency." | 1.26 | Tissue carnitine in Reye syndrome. ( Chutorian, AM; DiMauro, S; Willner, JH, 1978) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 39 (60.00) | 18.7374 |
| 1990's | 17 (26.15) | 18.2507 |
| 2000's | 6 (9.23) | 29.6817 |
| 2010's | 3 (4.62) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Feng, LF | 1 |
| Chen, XH | 1 |
| Li, DX | 1 |
| Li, XY | 1 |
| Song, JQ | 1 |
| Jin, Y | 1 |
| Yang, YL | 1 |
| van Rijt, WJ | 1 |
| Koolhaas, GD | 1 |
| Bekhof, J | 1 |
| Heiner Fokkema, MR | 1 |
| de Koning, TJ | 1 |
| Visser, G | 1 |
| Schielen, PC | 1 |
| van Spronsen, FJ | 1 |
| Derks, TG | 1 |
| Sauer, SW | 1 |
| Okun, JG | 1 |
| Hoffmann, GF | 1 |
| Koelker, S | 1 |
| Morath, MA | 1 |
| Hori, T | 1 |
| Fukao, T | 1 |
| Kobayashi, H | 1 |
| Teramoto, T | 1 |
| Takayanagi, M | 2 |
| Hasegawa, Y | 1 |
| Yasuno, T | 1 |
| Yamaguchi, S | 2 |
| Kondo, N | 1 |
| Scaglia, F | 1 |
| Scheuerle, AE | 1 |
| Towbin, JA | 1 |
| Armstrong, DL | 1 |
| Sweetman, L | 1 |
| Wong, LJ | 1 |
| Hou, JW | 1 |
| Bzduch, V | 1 |
| Behulova, D | 1 |
| Salingova, A | 1 |
| Ponec, J | 1 |
| Fabriciova, K | 1 |
| Kozak, L | 1 |
| Li, MX | 1 |
| Yoshida, G | 1 |
| Horiuchi, M | 1 |
| Kobayashi, K | 1 |
| Saheki, T | 1 |
| Coulter, DL | 1 |
| Cannon, RA | 1 |
| Kobayashi, S | 2 |
| Ijima, H | 1 |
| Kamoshita, S | 1 |
| Sugiyama, N | 2 |
| Wada, Y | 2 |
| Duran, M | 2 |
| de Klerk, JB | 1 |
| Wadman, SK | 2 |
| Scholte, HR | 1 |
| Beekman, RP | 1 |
| Jennekens, FG | 1 |
| Coates, PM | 1 |
| Hale, DE | 2 |
| Stanley, CA | 1 |
| Glasgow, AM | 2 |
| Collet, JP | 1 |
| Divry, P | 2 |
| Blanc, JF | 1 |
| Guibaud, P | 1 |
| David, M | 1 |
| Macabeo, V | 1 |
| Vibert, J | 1 |
| Hermier, M | 1 |
| La Montagne, JR | 1 |
| Millington, DS | 4 |
| Roe, CR | 4 |
| Maltby, DA | 3 |
| Böhles, H | 1 |
| Richter, K | 1 |
| Wagner-Thiessen, E | 1 |
| Schäfer, H | 1 |
| Romshe, CA | 1 |
| Hilty, MD | 1 |
| McClung, HJ | 1 |
| Kerzner, B | 1 |
| Reiner, CB | 1 |
| Chapoy, P | 1 |
| Angelini, C | 4 |
| Cederbaum, S | 1 |
| Eng, G | 1 |
| Engel, AG | 2 |
| Hinshaw, WB | 1 |
| Glenn, JL | 1 |
| Hatch, KM | 1 |
| Chapoy, PR | 1 |
| Brown, WJ | 1 |
| Stiff, JE | 1 |
| Shug, AL | 2 |
| Cederbaum, SD | 1 |
| Visentin, M | 1 |
| Bellasio, R | 1 |
| Tacconi, MT | 1 |
| Bergman, AJ | 1 |
| Donckerwolcke, RA | 1 |
| Smeitink, JA | 1 |
| Mousson, B | 2 |
| Vianey-Saban, C | 2 |
| Poll-The, BT | 1 |
| Smith, ET | 1 |
| Davis, GJ | 1 |
| Shimizu, N | 1 |
| Orii, T | 1 |
| Bertrand, C | 1 |
| Stamm, D | 1 |
| Dumoulin, R | 1 |
| Zabot, MT | 1 |
| Floret, D | 1 |
| Mathieu, M | 1 |
| Eirís, J | 1 |
| Ribes, A | 1 |
| Fernández-Prieto, R | 1 |
| Rodríguez-García, J | 1 |
| Rodríguez-Segade, S | 1 |
| Castro-Gago, M | 1 |
| Tang, NL | 1 |
| Hui, J | 1 |
| Law, LK | 1 |
| To, KF | 1 |
| Ruiter, JP | 1 |
| IJlst, L | 1 |
| Wanders, RJ | 1 |
| Ho, CS | 1 |
| Fok, TF | 1 |
| Yuen, PM | 1 |
| Hjelm, NM | 1 |
| Clayton, PT | 1 |
| Doig, M | 1 |
| Ghafari, S | 1 |
| Meaney, C | 1 |
| Taylor, C | 1 |
| Leonard, JV | 1 |
| Morris, M | 1 |
| Johnson, AW | 1 |
| Matern, D | 1 |
| Strauss, AW | 1 |
| Hillman, SL | 1 |
| Mayatepek, E | 1 |
| Trefz, FK | 1 |
| Ulrich, RG | 1 |
| Bacon, JA | 1 |
| Brass, EP | 1 |
| Cramer, CT | 1 |
| Petrella, DK | 1 |
| Sun, EL | 1 |
| Pichichero, ME | 1 |
| McCabe, ER | 1 |
| Willner, JH | 1 |
| Chutorian, AM | 1 |
| DiMauro, S | 1 |
| Touma, EH | 1 |
| Charpentier, C | 1 |
| Bell, RB | 1 |
| Brownell, AK | 1 |
| Goodman, SI | 1 |
| Frerman, FE | 1 |
| Seccombe, DW | 1 |
| Snyder, FF | 1 |
| Sugimoto, T | 2 |
| Woo, M | 2 |
| Nishida, N | 2 |
| Araki, A | 1 |
| Murakami, K | 1 |
| Kobayashi, Y | 2 |
| Kimura, S | 2 |
| Amemiya, F | 1 |
| Kakinuma, H | 1 |
| Yamamoto, S | 2 |
| Nakajima, H | 1 |
| Kidouchi, K | 1 |
| Kobayashi, M | 1 |
| Matsuyuki, M | 1 |
| Sinniah, D | 2 |
| Sinniah, R | 1 |
| Baskaran, G | 1 |
| Pathmanathan, R | 1 |
| Yamashita, F | 3 |
| Yoshino, M | 3 |
| Kuwajima, M | 1 |
| Kono, N | 1 |
| Tarui, S | 1 |
| Miyake, S | 1 |
| Murphy, JV | 1 |
| Marquardt, KM | 1 |
| Kobayashi, J | 1 |
| Ichimura, M | 1 |
| Sugie, Y | 1 |
| Sugie, H | 1 |
| Igarashi, Y | 1 |
| Matsuda, I | 2 |
| Ohtani, Y | 2 |
| Ninomiya, N | 1 |
| Tracey, BM | 1 |
| Cheng, KN | 1 |
| Rosankiewicz, J | 1 |
| Stacey, TE | 1 |
| Chalmers, RA | 2 |
| Shahar, E | 1 |
| Brand, N | 1 |
| Shapira, Y | 1 |
| Barash, V | 1 |
| Gutman, A | 1 |
| Matsuishi, T | 1 |
| Yuge, K | 1 |
| Aramaki, S | 1 |
| Yoshida, I | 2 |
| Kopieczna-Grzebieniak, E | 1 |
| Tarnawski, R | 1 |
| Toborek, M | 1 |
| Jakubowska, D | 1 |
| Ohyanagi, K | 1 |
| Bahl, JJ | 1 |
| Bressler, R | 1 |
| Koga, Y | 1 |
| Kimura, A | 1 |
| Takeuchi, T | 1 |
| Yasuhara, A | 1 |
| Sakane, Y | 1 |
| Taubman, B | 1 |
| Kelley, RI | 1 |
| Stumpf, DA | 1 |
| Parker, WD | 1 |
| Shaw, W | 1 |
| Legge, M | 1 |
| Kinnebrew, P | 1 |
| Bougnères, PF | 1 |
| Rocchiccioli, F | 1 |
| Kølvraa, S | 1 |
| Hadchouel, M | 1 |
| Lalau-Keraly, J | 1 |
| Chaussain, JL | 1 |
| Gregersen, N | 1 |
| Bohan, TP | 1 |
| Kahler, SG | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| 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 | ||
| 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] | |||||||
(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 |
7 reviews available for carnitine and Fatty Liver with Encephalopathy
| Article | Year |
|---|---|
Inborn Errors of Metabolism That Cause Sudden Infant Death: A Systematic Review with Implications for Population Neonatal Screening Programmes.
Topics: Amino Acids; Autopsy; Carnitine; Humans; Infant; Infant, Newborn; Metabolism, Inborn Errors; Neonata | 2016 |
Rate-dependent distal renal tubular acidosis and carnitine palmitoyltransferase I deficiency.
Topics: Acidosis, Renal Tubular; Biological Transport; Carnitine; Carnitine O-Palmitoyltransferase; Female; | 1994 |
Carnitine palmitoyl transferase I deficiency presenting as a Reye-like syndrome without hypoglycaemia.
Topics: Carnitine; Carnitine O-Palmitoyltransferase; Child, Preschool; Fatty Acids; Female; Fibroblasts; Hum | 1993 |
Metabolic, idiosyncratic toxicity of drugs: overview of the hepatic toxicity induced by the anxiolytic, panadiplon.
Topics: Animals; Anti-Anxiety Agents; Carnitine; Cell Hypoxia; Cells, Cultured; Chemical and Drug Induced Li | 2001 |
Medium chain acyl-CoA dehydrogenase deficiency.
Topics: Acyl-CoA Dehydrogenases; Blood Glucose; Carnitine; Child, Preschool; Fasting; Fatty Acids; Female; H | 1992 |
The pharmacology of carnitine.
Topics: Acidosis; Animals; Cardiomyopathies; Carnitine; Humans; Infant, Newborn; Renal Dialysis; Reye Syndro | 1987 |
Carnitine deficiency, organic acidemias, and Reye's syndrome.
Topics: Acids; Acyl Coenzyme A; Carnitine; Humans; Metabolic Diseases; Reye Syndrome | 1985 |
1 trial available for carnitine and Fatty Liver with Encephalopathy
| Article | Year |
|---|---|
Screening for medium chain acyl-CoA dehydrogenase deficiency using electrospray ionisation tandem mass spectrometry.
Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Adolescent; Age Distribution; Aging; Carnitine; Chi | 1998 |
57 other studies available for carnitine and Fatty Liver with Encephalopathy
| Article | Year |
|---|---|
[Reye syndrome and sudden death symptoms after oral administration of nimesulide due to upper respiratory tract infection in a boy].
Topics: Acyl-CoA Dehydrogenase; Administration, Oral; Carnitine; Child; Death, Sudden; Humans; Male; Respira | 2018 |
Impact of short- and medium-chain organic acids, acylcarnitines, and acyl-CoAs on mitochondrial energy metabolism.
Topics: Acids; Acyl Coenzyme A; Animals; Carnitine; Cattle; Electron Transport Complex III; Energy Metabolis | 2008 |
Carnitine palmitoyltransferase 2 deficiency: the time-course of blood and urinary acylcarnitine levels during initial L-carnitine supplementation.
Topics: Acetylcarnitine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Blood Chemical Analysis; Carniti | 2010 |
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 |
Primary systemic carnitine deficiency presenting as recurrent Reye-like syndrome and dilated cardiomyopathy.
Topics: Cardiomyopathy, Dilated; Carnitine; Child; Female; Humans; Recurrence; Reye Syndrome | 2002 |
Serum free carnitine in medium chain acyl-CoA dehydrogenase deficiency.
Topics: Acyl-CoA Dehydrogenase; Carnitine; Child, Preschool; Humans; Male; Reye Syndrome | 2003 |
Prolonged effect of single carnitine administration on fasted carnitine-deficient JVS mice regarding their locomotor activity and energy expenditure.
Topics: Animals; Carnitine; Disease Models, Animal; Energy Metabolism; Fasting; Fatty Acids; Lipid Metabolis | 2006 |
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid | 1984 |
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid | 1984 |
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid | 1984 |
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid | 1984 |
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid | 1984 |
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid | 1984 |
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid | 1984 |
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid | 1984 |
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid | 1984 |
Reye's syndrome or its metabolic mimics?
Topics: Administration, Oral; Biopsy; Carnitine; Diagnosis, Differential; Female; Humans; Infant; Kwashiorko | 1984 |
[An autopsy case of Reye's syndrome associated with deficient muscle carnitine].
Topics: Carnitine; Histocytochemistry; Humans; Infant; Male; Muscles; Reye Syndrome | 1984 |
Systemic carnitine deficiency: benefit of oral carnitine supplements vs. persisting biochemical abnormalities.
Topics: Carnitine; Fatty Acids; Humans; Infant; Liver; Male; Muscles; Reye Syndrome | 1984 |
Systemic carnitine deficiency simulating Reye syndrome.
Topics: Carnitine; Child; Diagnosis, Differential; Humans; Reye Syndrome | 1984 |
[Medium chain acyl-CoA dehydrogenase deficiency. Apropos of a case with demonstration of this enzyme deficiency].
Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Carnitine; Cells, Cultured; Coma; Diagnosis, Differ | 1984 |
From the National Institute of Allergy and Infectious Diseases: Summary of a workshop on disease mechanisms and prospects for prevention of Reye's syndrome.
Topics: Adolescent; Adult; Animals; Biological Transport; Calcium; Carnitine; Child; Child, Preschool; Disea | 1983 |
Application of high resolution fast atom bombardment and constant B/E ratio linked scanning to the identification and analysis of acylcarnitines in metabolic disease.
Topics: Acetylcarnitine; Acidosis; Carnitine; Humans; Magnetic Resonance Spectroscopy; Malonates; Mass Spect | 1984 |
Decreased serum carnitine in valproate induced Reye syndrome.
Topics: Carnitine; Child, Preschool; Female; Humans; Reye Syndrome; Valproic Acid | 1982 |
Amino acid pattern in Reye syndrome: comparison with clinically similar entities.
Topics: Amino Acids; Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing); Carnitine; Diagnosis, Differentia | 1981 |
[Systemic carnitine deficiency: its place in Reye's syndrome (author's transl)].
Topics: Cardiomyopathies; Carnitine; Child, Preschool; Diagnosis, Differential; Hepatic Encephalopathy; Huma | 1981 |
Systemic carnitine deficiency.
Topics: Biological Transport; Carnitine; Diagnosis, Differential; Fatty Acids; Humans; Infant; Male; Metabol | 1981 |
Systemic carnitine deficiency simulating recurrent Reye syndrome.
Topics: Carnitine; Child; Child, Preschool; Diagnosis, Differential; Fasting; Humans; Infant; Reye Syndrome; | 1980 |
Serum carnitine in Reye's syndrome.
Topics: Carnitine; Humans; Reye Syndrome | 1980 |
Systemic carnitine deficiency--a treatable inherited lipid-storage disease presenting as Reye's syndrome.
Topics: Carnitine; Child, Preschool; Diagnosis, Differential; Humans; Lipid Metabolism, Inborn Errors; Liver | 1980 |
Reye syndrome model in rats: protection against liver abnormalities by L-carnitine and acetyl-L-carnitine.
Topics: Acetylcarnitine; Acyl Coenzyme A; Animals; Aspirin; Carnitine; Coenzyme A; Disease Models, Animal; K | 1995 |
Medium-chain acylcoenzyme-A dehydrogenase deficiency. Not just another Reye syndrome.
Topics: Acyl-CoA Dehydrogenase; Blood Chemical Analysis; Brain Edema; Carnitine; Cause of Death; Chromosomes | 1993 |
A study of urinary metabolites in patients with dicarboxylic aciduria for differential diagnosis.
Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Child, Preschool; Diagnosis, Differential; Dicarbox | 1994 |
[3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome].
Topics: Acidosis; Adolescent; Apnea; Carnitine; Coma; Diagnosis, Differential; Fatty Liver; Female; Fibrobla | 1998 |
Primary plasmalemmal carnitine transporter defect manifested with dicarboxylic aciduria and impaired fatty acid oxidation.
Topics: Alanine Transaminase; Carnitine; Carnitine Acyltransferases; Dicarboxylic Acids; Fatty Acids; Humans | 1998 |
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 |
Recurrent Reye's syndrome.
Topics: Adolescent; Carnitine; Diagnosis, Differential; Female; Humans; Ornithine Carbamoyltransferase; Recu | 1978 |
Tissue carnitine in Reye syndrome.
Topics: Acyltransferases; Carnitine; Carnitine O-Palmitoyltransferase; Child; Humans; Liver; Muscles; Reye S | 1978 |
Electron transfer flavoprotein: ubiquinone oxidoreductase (ETF:QO) deficiency in an adult.
Topics: Acidosis; Adult; Carnitine; Electron-Transferring Flavoproteins; Fatty Acid Desaturases; Female; Glu | 1990 |
The effect of L-carnitine supplementation in 4 pentenoic acid treated rats.
Topics: Ammonia; Animals; Blood Glucose; Carnitine; Disease Models, Animal; Fatty Acids, Monounsaturated; Fa | 1990 |
Brain and liver pathology in a patient with carnitine deficiency.
Topics: Astrocytes; Brain; Brain Diseases, Metabolic; Brain Edema; Carnitine; Humans; Infant; Liver; Male; R | 1990 |
Carnitine metabolism and urinary organic acid excretion in Reye's syndrome and salicylate intoxication.
Topics: Aspirin; Carnitine; Child, Preschool; Dicarboxylic Acids; Fatty Acids; Female; Humans; Infant; Male; | 1990 |
Carnitine deficiency in inherited organic acid disorders and Reye syndrome.
Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Child, Preschool; Humans; Infant; Male; Metabolism, | 1990 |
Mitochondrial changes and carnitine status in fasting rats.
Topics: Animals; Body Weight; Carnitine; Fasting; Fatty Acids, Nonesterified; Male; Mitochondria, Liver; Rat | 1990 |
Evaluation of the possible role of glucose, carnitine, coenzyme Q10 and steroids in the treatment of Reye's syndrome using the margosa oil animal model.
Topics: Animals; Carnitine; Coenzymes; Dexamethasone; Disease Models, Animal; Female; Glucose; Glycerides; M | 1990 |
[Alterations of mitochondria in metabolic diseases. Carnitine deficiency, carnitine palmitoyltransferase deficiency and beta oxidation].
Topics: Acyltransferases; Carnitine; Carnitine O-Palmitoyltransferase; Fatty Acids; Humans; Metabolic Diseas | 1989 |
[Significance of carnitine analysis in clinical tests].
Topics: Carnitine; Fatty Acid Desaturases; Humans; Reference Values; Reye Syndrome; Tissue Distribution | 1989 |
Type II fiber myolysis in a patient with hypocarnitinemia.
Topics: Biopsy; Carnitine; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Intellectual Disabili | 1989 |
Valproic acid associated abnormalities of carnitine metabolism.
Topics: Carnitine; Child; Child, Preschool; Humans; Infant; Reye Syndrome; Valproic Acid | 1985 |
[A case of phosphoglucomutase deficiency with decreased muscle and serum carnitine].
Topics: Carnitine; Glycogen; Humans; Infant; Male; Muscles; Phosphoglucomutase; Reye Syndrome | 1986 |
Renal handling of carnitine in children with carnitine deficiency and hyperammonemia associated with valproate therapy.
Topics: Ammonia; Carnitine; Epilepsy; Humans; Infant; Intellectual Disability; Kidney; Metabolic Clearance R | 1986 |
What is the best treatment for Reye's syndrome?
Topics: Adolescent; Barbiturates; Blood Transfusion; Carnitine; Child; Critical Care; Diuretics; Exchange Tr | 1986 |
Urinary C6-C12 dicarboxylic acylcarnitines in Reye's syndrome.
Topics: Carnitine; Child, Preschool; Dicarboxylic Acids; Female; Humans; Reye Syndrome | 1988 |
Familial carnitine deficiency: further evidence for autosomal recessive transmission with variable expression.
Topics: Carnitine; Child, Preschool; Chromosome Aberrations; Chromosome Disorders; Female; Gene Expression R | 1988 |
Lipid storage myopathy associated with recurrent Reye syndrome-like attacks, but with a normal carnitine level.
Topics: Carnitine; Child; Female; Humans; Lipid Metabolism; Metabolic Diseases; Muscles; Reye Syndrome | 1988 |
[Hyperammonemias of childhood. III. Acquired diseases].
Topics: Ammonia; Carnitine; Diagnosis, Differential; Erythroblastosis, Fetal; Humans; Infant, Newborn; Reye | 1988 |
Hyperammonemia related to carnitine metabolism with particular emphasis on ornithine transcarbamylase deficiency.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carnitine; Child; Dietary Pr | 1987 |
Inhibition of mitochondrial functions by margosa oil: possible implications in the pathogenesis of Reye's syndrome.
Topics: Animals; Carnitine; Glycerides; In Vitro Techniques; Male; Mitochondria, Liver; Oxidative Phosphoryl | 1987 |
Serum and urinary carnitine and organic acids in Reye syndrome and Reye-like syndrome.
Topics: Acids; Adolescent; Carnitine; Child; Child, Preschool; Female; Humans; Infant; Male; Reye Syndrome | 1986 |
Familial Reye-like syndrome: a presentation of medium-chain acyl-coenzyme A dehydrogenase deficiency.
Topics: Acyl-CoA Dehydrogenase; Carboxylic Acids; Carnitine; Diagnosis, Differential; Fatty Acid Desaturases | 1987 |
Possible role of lysolecithins and nonesterified fatty acids in the pathogenesis of Reye's syndrome, sudden infant death syndrome, acute pancreatitis, and diabetic ketoacidosis.
Topics: Acute Disease; Barbiturates; Blood Transfusion; Carnitine; Diabetic Ketoacidosis; Fasting; Fatty Aci | 1985 |
Systemic carnitine deficiency as the cause of a prolonged illness and sudden death in a six-year-old child.
Topics: Acids; Carnitine; Child; Death, Sudden; Diagnostic Errors; Female; Humans; Metabolism, Inborn Errors | 1985 |
Recognition of medium-chain acyl-CoA dehydrogenase deficiency in asymptomatic siblings of children dying of sudden infant death or Reye-like syndromes.
Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Adult; Breast Feeding; Carnitine; Child, Preschool; | 1986 |
Medium-chain acyl-CoA dehydrogenase deficiency in two siblings with a Reye-like syndrome.
Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Blood Glucose; Caprylates; Carnitine; Fatty Acids, | 1985 |
Diagnostic and therapeutic implications of medium-chain acylcarnitines in the medium-chain acyl-coA dehydrogenase deficiency.
Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Carnitine; Dicarboxylic Acids; Female; Gas Chromato | 1985 |