carnitine has been researched along with Acidosis in 54 studies
Acidosis: A pathologic condition of acid accumulation or depletion of base in the body. The two main types are RESPIRATORY ACIDOSIS and metabolic acidosis, due to metabolic acid build up.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Propofol infusion syndrome is a rare but frequently fatal complication in critically ill children given long-term propofol infusions." | 7.71 | Impaired fatty acid oxidation in propofol infusion syndrome. ( Segar, P; Shield, J; Stone, J; Weir, P; Wolf, A, 2001) |
| " Laboratory data showed hypoglycemia and hypocarnitinemia." | 3.74 | Carnitine-associated encephalopathy caused by long-term treatment with an antibiotic containing pivalic acid. ( Ito, T; Koyama, N; Makino, Y; Sugiura, T; Sugiyama, N, 2007) |
| "Propofol infusion syndrome is a rare but frequently fatal complication in critically ill children given long-term propofol infusions." | 3.71 | Impaired fatty acid oxidation in propofol infusion syndrome. ( Segar, P; Shield, J; Stone, J; Weir, P; Wolf, A, 2001) |
| "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) |
| " Four dynamic syndromes are currently recognized: 1) defective carbohydrate utilization, due to block of glycogenolysis or glycolysis; 2) defective lipid utilization, due to deficiency of the mitochondrial translocation of long-chain fatty acids (carnitine palmityltransferase deficiency); 3) lactic acidosis, due to defects of mitochondrial electron transport enzymes and possibly other unidentified defects; and 4) abnormal adenine nucleotide metabolism, exemplified by adenylate deaminase deficiency." | 3.67 | Clinical disorders of muscle energy metabolism. ( Layzer, RB; Lewis, SF, 1984) |
| " Information on therapeutic usage, administration, and dosage was also recorded." | 1.36 | Setting up an emergency stock for metabolic diseases. ( Fernandez-Llamazares, CM; Manrique-Rodríguez, S; Sanjurjo-Sáez, M; Serrano, ML, 2010) |
| "Propionic acidemia is a hereditary metabolic disease caused by a deficiency of enzyme propionyl-CoA carboxylase, which is involved in the catabolism of ramified amino acids, odd-chain fatty acids, and other metabolites; the deficiency of this enzyme leads to an accumulation of toxic substances in the body." | 1.34 | Subacute presentation of propionic acidemia. ( de la Sierra García-Valdecasas, M; del Portal, LR; Delgado, C; Jiménez, LM; Macías, C; Pérez, M, 2007) |
| "A boy had neonatal seizure, lethargy, and metabolic acidosis at presentation." | 1.30 | Very long chain acyl-coenzyme A dehydrogenase deficiency in two siblings: evolution after prenatal diagnosis and prompt management. ( Brivet, M; Hubinont, C; Sluysmans, T; Tuerlinckx, D; Verellen-Dumoulin, C; Vianey-Saban, C, 1997) |
| "Carnitine conjugates were however detected." | 1.29 | The detection of 3-methylglutarylcarnitine and a new dicarboxylic conjugate, 3-methylglutaconylcarnitine, in 3-methylglutaconic aciduria. ( de Wet, WJ; Erasmus, E; Gibson, KM; Jooste, S; Mienie, LJ, 1994) |
| "L-Carnitine treatment increased cerebral CoA-SH levels and both hepatic and cerebral acetyl-CoA levels in mutant mice." | 1.29 | A profile of cerebral and hepatic carnitine, ammonia, and energy metabolism in a model of organic aciduria: BALB/cByJ mouse with short-chain acyl-CoA dehydrogenase deficiency. ( Butterworth, RF; Cyr, D; Giguère, R; Michalak, A; Qureshi, IA; Ratnakumari, L, 1993) |
| "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) |
| "The overall frequency of ketonuria at (re)admission was 45% together with moderately elevated or high 3-hydroxybutyrate serum concentrations." | 1.27 | Ketosis, serum carnitine and its precursor amino acids in normal and diabetic ethiopians. ( Kohnert, KD; Löster, H; Lubs, H; Peters, WH; Seim, H; Strack, E, 1987) |
| "3." | 1.27 | The effects of post-exercise glucose and alanine ingestion on plasma carnitine and ketosis in humans. ( Carlin, JI; Olson, EB; Peters, HA; Reddan, WG, 1987) |
| "Carnitine is an essential factor in long-chain fatty acid oxidation." | 1.26 | Carnitine and carnitine palmitoyltransferase in fatty acid oxidation and ketosis. ( Hoppel, CL, 1982) |
| "Thus, ketosis is viewed as the result of increased mobilization of free fatty acids from adipose tissue (site 1) to the liver (site 2), coupled with simultaneous enhancement of the liver's capacity to convert these substrates into acetoacetic and beta-hydroxybutyric acids." | 1.26 | Hormonal control of ketogenesis. Biochemical considerations. ( Foster, DW; McGarry, JD, 1977) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 29 (53.70) | 18.7374 |
| 1990's | 11 (20.37) | 18.2507 |
| 2000's | 8 (14.81) | 29.6817 |
| 2010's | 5 (9.26) | 24.3611 |
| 2020's | 1 (1.85) | 2.80 |
| Authors | Studies |
|---|---|
| Tan, J | 3 |
| Zheng, M | 3 |
| Cai, R | 3 |
| Zeng, T | 3 |
| Yin, B | 3 |
| Yang, J | 4 |
| Wei, B | 3 |
| Chang, R | 3 |
| Jiang, Y | 3 |
| Yuan, D | 3 |
| Pan, L | 3 |
| Huang, L | 3 |
| Ning, H | 3 |
| Wei, J | 3 |
| Chen, D | 3 |
| Liu, H | 1 |
| Miao, JK | 1 |
| Yu, CW | 1 |
| Wan, KX | 1 |
| Zhang, J | 1 |
| Yuan, ZJ | 1 |
| Wang, DJ | 1 |
| Zeng, Y | 1 |
| Zou, L | 1 |
| Han, L | 1 |
| Wu, S | 1 |
| Ye, J | 1 |
| Qiu, W | 1 |
| Zhang, H | 1 |
| Gao, X | 1 |
| Wang, Y | 1 |
| Gong, Z | 1 |
| Jin, J | 1 |
| Gu, X | 1 |
| Kimmoun, A | 1 |
| Abboud, G | 1 |
| Strazeck, J | 1 |
| Merten, M | 1 |
| Guéant, JL | 1 |
| Feillet, F | 1 |
| Chace, DH | 1 |
| Lim, T | 1 |
| Hansen, CR | 1 |
| Adam, BW | 1 |
| Hannon, WH | 1 |
| Fernandez-Llamazares, CM | 1 |
| Serrano, ML | 1 |
| Manrique-Rodríguez, S | 1 |
| Sanjurjo-Sáez, M | 1 |
| Rosa, M | 1 |
| Pascarella, A | 1 |
| Parenti, G | 1 |
| Buono, S | 1 |
| Romano, A | 1 |
| Della Casa, R | 1 |
| Andria, G | 1 |
| Marino, M | 1 |
| Riccio, MP | 1 |
| Bravaccio, C | 1 |
| Zwickler, T | 1 |
| Haege, G | 1 |
| Riderer, A | 1 |
| Hörster, F | 1 |
| Hoffmann, GF | 2 |
| Burgard, P | 1 |
| Kölker, S | 2 |
| Schor, DS | 1 |
| Feyh, P | 1 |
| Wagner, L | 1 |
| Jeffrey, I | 1 |
| Pourfarzam, M | 1 |
| Okun, JG | 1 |
| Zschocke, J | 1 |
| Baric, I | 1 |
| Bain, MD | 1 |
| Jakobs, C | 1 |
| Chalmers, RA | 1 |
| Distelmaier, F | 1 |
| Vogel, M | 1 |
| Spiekerkötter, U | 1 |
| Gempel, K | 1 |
| Klee, D | 1 |
| Braunstein, S | 1 |
| Groneck, HP | 1 |
| Mayatepek, E | 1 |
| Wendel, U | 1 |
| Schwahn, B | 1 |
| Makino, Y | 1 |
| Sugiura, T | 1 |
| Ito, T | 2 |
| Sugiyama, N | 2 |
| Koyama, N | 1 |
| Delgado, C | 1 |
| Macías, C | 1 |
| de la Sierra García-Valdecasas, M | 1 |
| Pérez, M | 1 |
| del Portal, LR | 1 |
| Jiménez, LM | 1 |
| Beattie, MA | 2 |
| Winder, WW | 2 |
| Layzer, RB | 1 |
| Lewis, SF | 1 |
| Millington, DS | 2 |
| Roe, CR | 3 |
| Maltby, DA | 2 |
| Morooka, K | 1 |
| Hoppel, CL | 2 |
| Genuth, SM | 1 |
| Corr, PB | 1 |
| Snyder, DW | 1 |
| Cain, ME | 1 |
| Crafford, WA | 1 |
| Gross, RW | 1 |
| Sobel, BE | 1 |
| Peschechera, A | 1 |
| Ferrari, LE | 1 |
| Arrigoni-Martelli, E | 1 |
| Hülsmann, WC | 1 |
| Walter, JH | 1 |
| Wraith, JE | 1 |
| Cleary, MA | 1 |
| Kumps, A | 1 |
| Duez, P | 1 |
| Mardens, Y | 1 |
| Jooste, S | 1 |
| Erasmus, E | 1 |
| Mienie, LJ | 1 |
| de Wet, WJ | 1 |
| Gibson, KM | 2 |
| Qureshi, IA | 1 |
| Ratnakumari, L | 1 |
| Michalak, A | 1 |
| Giguère, R | 1 |
| Cyr, D | 1 |
| Butterworth, RF | 1 |
| Kidouchi, K | 1 |
| Kajita, M | 1 |
| Chiba, T | 1 |
| Niwa, T | 1 |
| Wada, Y | 1 |
| Fontaine, M | 1 |
| Briand, G | 1 |
| Ser, N | 1 |
| Armelin, I | 1 |
| Rolland, MO | 1 |
| Degand, P | 1 |
| Vamecq, J | 1 |
| Sluysmans, T | 1 |
| Tuerlinckx, D | 1 |
| Hubinont, C | 1 |
| Verellen-Dumoulin, C | 1 |
| Brivet, M | 1 |
| Vianey-Saban, C | 1 |
| Weinberg, GL | 1 |
| Laurito, CE | 1 |
| Geldner, P | 1 |
| Pygon, BH | 1 |
| Burton, BK | 1 |
| Eirís, J | 1 |
| Ribes, A | 1 |
| Fernández-Prieto, R | 1 |
| Rodríguez-García, J | 1 |
| Rodríguez-Segade, S | 1 |
| Castro-Gago, M | 1 |
| al-Essa, MA | 1 |
| Rashed, MS | 1 |
| Bakheet, SM | 1 |
| Patay, ZJ | 1 |
| Ozand, PT | 1 |
| Wolf, A | 1 |
| Weir, P | 1 |
| Segar, P | 1 |
| Stone, J | 1 |
| Shield, J | 1 |
| McGarry, JD | 3 |
| Foster, DW | 3 |
| Engel, AG | 2 |
| Banker, BQ | 1 |
| Eiben, RM | 1 |
| Robles-Valdes, C | 2 |
| Bell, RB | 1 |
| Brownell, AK | 1 |
| Goodman, SI | 1 |
| Frerman, FE | 1 |
| Seccombe, DW | 1 |
| Snyder, FF | 1 |
| Dasouki, M | 1 |
| Buchanan, D | 1 |
| Mercer, N | 1 |
| Thoene, J | 1 |
| Böhles, H | 1 |
| Peters, WH | 1 |
| Seim, H | 1 |
| Löster, H | 1 |
| Strack, E | 1 |
| Lubs, H | 1 |
| Kohnert, KD | 1 |
| Carlin, JI | 1 |
| Olson, EB | 1 |
| Peters, HA | 1 |
| Reddan, WG | 1 |
| Rebouche, CJ | 1 |
| Paulson, DJ | 1 |
| Bahl, JJ | 1 |
| Bressler, R | 2 |
| Dugan, RE | 1 |
| Schmidt, MJ | 1 |
| Hoganson, GE | 1 |
| Steele, J | 1 |
| Gilles, BA | 1 |
| Shug, AL | 1 |
| Erfle, JD | 5 |
| Sauer, F | 4 |
| Sauer, FD | 1 |
| Fisher, LJ | 3 |
| Katz, R | 1 |
| Wittels, B | 1 |
| Tanaka, K | 1 |
| Miller, EM | 1 |
| Isselbacher, KJ | 1 |
| Bremer, J | 1 |
| Binns, MR | 1 |
| Broekhuysen, J | 1 |
| Baudine, A | 1 |
| Deltour, G | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| 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 | |||
| 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 |
3 reviews available for carnitine and Acidosis
| Article | Year |
|---|---|
[Benign myopathy in children].
Topics: Acidosis; Adolescent; Adult; Age Factors; Carnitine; Carnitine O-Palmitoyltransferase; Child; Child, | 1982 |
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 |
51 other studies available for carnitine and Acidosis
| Article | Year |
|---|---|
[Analysis of IVD gene variants in four children with isovalerate acidemia].
Topics: Acidosis; Carnitine; Child; Erythrocytes; High-Throughput Nucleotide Sequencing; Humans; Infant, New | 2022 |
[Analysis of IVD gene variants in four children with isovalerate acidemia].
Topics: Acidosis; Carnitine; Child; Erythrocytes; High-Throughput Nucleotide Sequencing; Humans; Infant, New | 2022 |
[Analysis of IVD gene variants in four children with isovalerate acidemia].
Topics: Acidosis; Carnitine; Child; Erythrocytes; High-Throughput Nucleotide Sequencing; Humans; Infant, New | 2022 |
[Analysis of IVD gene variants in four children with isovalerate acidemia].
Topics: Acidosis; Carnitine; Child; Erythrocytes; High-Throughput Nucleotide Sequencing; Humans; Infant, New | 2022 |
Severe clinical manifestation of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase deficiency associated with two novel mutations: a case report.
Topics: Acidosis; Acyl Coenzyme A; Adipates; Carnitine; Diarrhea; Dicarboxylic Acids; Exome Sequencing; Fata | 2019 |
Biochemical, molecular and outcome analysis of eight chinese asymptomatic individuals with methyl malonic acidemia detected through newborn screening.
Topics: Acetylcarnitine; Acidosis; Asian People; Asymptomatic Diseases; Carboxy-Lyases; Carnitine; Carrier P | 2015 |
Acute decompensation of isovaleric acidemia induced by Graves' disease.
Topics: Acidosis; Carnitine; Diet, Protein-Restricted; Female; Graves Disease; Hemiterpenes; Humans; Isovale | 2008 |
Quantification of malonylcarnitine in dried blood spots by use of MS/MS varies by stable isotope internal standard composition.
Topics: Acidosis; Carnitine; Humans; Infant, Newborn; Isotopes; Malonates; Reference Standards; Tandem Mass | 2009 |
Setting up an emergency stock for metabolic diseases.
Topics: Acidosis; Amino Acids; Carnitine; Chelating Agents; Disease Management; Electronic Prescribing; Emer | 2010 |
Developmental evolution in a patient with multiple acyl-coenzymeA dehydrogenase deficiency under pharmacological treatment.
Topics: Acidosis; Acyl-CoA Dehydrogenase; Amino Acid Metabolism, Inborn Errors; Carnitine; Child Development | 2012 |
Metabolic decompensation in methylmalonic aciduria: which biochemical parameters are discriminative?
Topics: Acid-Base Equilibrium; Acidosis; Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; B | 2012 |
Glutaryl-CoA dehydrogenase deficiency: region-specific analysis of organic acids and acylcarnitines in post mortem brain predicts vulnerability of the putamen.
Topics: Acidosis; Acute Disease; Adolescent; Anticonvulsants; Atrophy; Brain; Carnitine; DNA Mutational Anal | 2003 |
Cystic renal dysplasia as a leading sign of inherited metabolic disease.
Topics: Abnormalities, Multiple; Acidosis; Carnitine; Carnitine O-Palmitoyltransferase; Fatal Outcome; Femal | 2007 |
Carnitine-associated encephalopathy caused by long-term treatment with an antibiotic containing pivalic acid.
Topics: Acidosis; Anti-Bacterial Agents; Brain Diseases, Metabolic; Carnitine; Cephalosporins; Humans; Hypog | 2007 |
Subacute presentation of propionic acidemia.
Topics: Acidosis; Amino Acids; Biotin; Brain; Carnitine; Diagnosis, Differential; Diet, Protein-Restricted; | 2007 |
Mechanism of training-induced attenuation of postexercise ketosis.
Topics: 3-Hydroxybutyric Acid; Acidosis; Animals; Carnitine; Cyclic AMP; Hydroxybutyrates; Ketosis; Liver; L | 1984 |
Clinical disorders of muscle energy metabolism.
Topics: Acidosis; Adenine Nucleotides; Carbohydrate Metabolism; Carnitine; Energy Metabolism; Humans; Lactat | 1984 |
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 |
Urinary excretion of acetylcarnitine during human diabetic and fasting ketosis.
Topics: Acetylcarnitine; Acidosis; Adolescent; Adult; Aged; Body Weight; Carnitine; Child; Diabetic Ketoacid | 1982 |
Carnitine and carnitine palmitoyltransferase in fatty acid oxidation and ketosis.
Topics: Acidosis; Acyltransferases; Animals; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltra | 1982 |
Electrophysiological effects of amphiphiles on canine purkinje fibers. Implications for dysrhythmia secondary to ischemia.
Topics: Acidosis; Animals; Arrhythmias, Cardiac; Carnitine; Coronary Disease; Dogs; Electrophysiology; Fatty | 1981 |
Uptake and release of carnitine by vascular endothelium in culture; effects of protons and oxygen free radicals.
Topics: Acidosis; Animals; Carnitine; Cattle; Cell Membrane; Cells, Cultured; Endothelium, Vascular; Female; | 1995 |
Absence of acidosis in the initial presentation of propionic acidaemia.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carnitine; Food Pr | 1995 |
Gas chromatographic profiling and determination of urinary acylcarnitines.
Topics: Acidosis; Carnitine; Chromatography, Gas; Chromatography, Ion Exchange; Humans; Indicators and Reage | 1994 |
The detection of 3-methylglutarylcarnitine and a new dicarboxylic conjugate, 3-methylglutaconylcarnitine, in 3-methylglutaconic aciduria.
Topics: Acidosis; Carnitine; Chromatography, Gas; Female; Fibroblasts; Glutarates; Glycine; Humans; Hydro-Ly | 1994 |
A profile of cerebral and hepatic carnitine, ammonia, and energy metabolism in a model of organic aciduria: BALB/cByJ mouse with short-chain acyl-CoA dehydrogenase deficiency.
Topics: Acidosis; Acyl-CoA Dehydrogenase, Long-Chain; Acyltransferases; Ammonia; Animals; Brain Chemistry; C | 1993 |
Liquid chromatographic-atmospheric pressure chemical ionization mass spectrometric analysis of glycine conjugates and urinary isovalerylglycine in isovaleric acidemia.
Topics: Acidosis; Administration, Oral; Carnitine; Child, Preschool; Chromatography, Liquid; Female; Glucuro | 1995 |
Metabolic studies in twin brothers with 2-methylacetoacetyl-CoA thiolase deficiency.
Topics: Acetyl-CoA C-Acyltransferase; Acidosis; Biomarkers; Carnitine; Cells, Cultured; Child, Preschool; Di | 1996 |
Very long chain acyl-coenzyme A dehydrogenase deficiency in two siblings: evolution after prenatal diagnosis and prompt management.
Topics: Acidosis; Acyl-CoA Dehydrogenase, Long-Chain; Amniocentesis; Carnitine; Diagnosis, Differential; Fat | 1997 |
Malignant ventricular dysrhythmias in a patient with isovaleric acidemia receiving general and local anesthesia for suction lipectomy.
Topics: Acidosis; Adolescent; Anesthesia, General; Anesthesia, Local; Anesthetics, Local; Bupivacaine; Carni | 1997 |
[3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome].
Topics: Acidosis; Adolescent; Apnea; Carnitine; Coma; Diagnosis, Differential; Fatty Liver; Female; Fibrobla | 1998 |
Glutaric aciduria type II: observations in seven patients with neonatal- and late-onset disease.
Topics: Acidosis; Age of Onset; Brain; Carnitine; Child; Consanguinity; Female; Glutarates; Humans; Infant; | 2000 |
Impaired fatty acid oxidation in propofol infusion syndrome.
Topics: Acidosis; Carnitine; Critical Care; Electron Transport; Fatty Acids; Hemofiltration; Humans; Hypnoti | 2001 |
Hormonal control of ketogenesis. Biochemical considerations.
Topics: Acetoacetates; Acidosis; Adipose Tissue; Alcoholism; Animals; Carnitine; Carnitine Acyltransferases; | 1977 |
Carnitine deficiency: clinical, morphological, and biochemical observations in a fatal case.
Topics: Acidosis; Carnitine; Child; Hepatomegaly; Humans; Male; Mitochondria, Muscle; Muscles; Muscular Dise | 1977 |
Maternal-fetal carnitine relationship and neonatal ketosis in the rat.
Topics: Acidosis; Aging; Animals; Animals, Newborn; Body Weight; Carnitine; Fasting; Female; Fetus; Ketone B | 1976 |
Glucagon and ketogenesis.
Topics: Acidosis; Age Factors; Animals; Animals, Newborn; Carnitine; Female; Glucagon; Ketone Bodies; Ketosi | 1976 |
Electron transfer flavoprotein: ubiquinone oxidoreductase (ETF:QO) deficiency in an adult.
Topics: Acidosis; Adult; Carnitine; Electron-Transferring Flavoproteins; Fatty Acid Desaturases; Female; Glu | 1990 |
3-Hydroxy-3-methylglutaric aciduria: response to carnitine therapy and fat and leucine restriction.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Carnitine; Dietary Fats; Dietary Proteins; Female; G | 1987 |
Attenuation of postexercise ketosis in fasted endurance-trained rats.
Topics: 3-Hydroxybutyric Acid; Acidosis; Adaptation, Physiological; Animals; Carnitine; Cyclic AMP; Fasting; | 1985 |
[When is parenteral carnitine administration indicated?].
Topics: Acidosis; Adult; Ammonia; Carnitine; Fat Emulsions, Intravenous; Humans; Infant, Newborn; Infant, Pr | 1986 |
Ketosis, serum carnitine and its precursor amino acids in normal and diabetic ethiopians.
Topics: Acidosis; Adolescent; Adult; Amino Acids; Carnitine; Diabetes Mellitus, Type 1; Diabetic Ketoacidosi | 1987 |
Characterization of new diagnostic acylcarnitines in patients with beta-ketothiolase deficiency and glutaric aciduria type I using mass spectrometry.
Topics: Acetyl-CoA C-Acyltransferase; Acidosis; Acyltransferases; Carnitine; Gas Chromatography-Mass Spectro | 1987 |
The effects of post-exercise glucose and alanine ingestion on plasma carnitine and ketosis in humans.
Topics: 3-Hydroxybutyric Acid; Acidosis; Adult; Alanine; Carnitine; Fasting; Fatty Acids, Nonesterified; Glu | 1987 |
High-performance liquid chromatography of coenzyme A esters formed by transesterification of short-chain acylcarnitines: diagnosis of acidemias by urinary analysis.
Topics: Acidosis; Carnitine; Chromatography, High Pressure Liquid; Coenzyme A; Esterification; Humans | 1987 |
Acetyl coenzyme A and acetylcarnitine concentration and turnover rates in muscle and liver of the ketotic rat and guinea pig.
Topics: Acetates; Acidosis; Acyltransferases; Animals; Carbon Dioxide; Carbon Isotopes; Carnitine; Coenzyme | 1967 |
Interrelationships between milk carnitine and blood and milk components and tissue carnitine in normal and ketotic cows.
Topics: Acetoacetates; Acidosis; Animals; Blood Glucose; Brain; Carnitine; Cattle; Cattle Diseases; Fatty Ac | 1974 |
Effect of infusion of carnitine and glucose on blood glucose, ketones, and free fatty acids of ketotic cows.
Topics: Acetates; Acidosis; Animals; Blood Glucose; Butyrates; Carnitine; Cattle; Cattle Diseases; Fatty Aci | 1971 |
The role of carnitine in the intracellular translocation of acyl coenzyme-a derivatives.
Topics: Acetoacetates; Acidosis; Adipose Tissue; Animals; Carnitine; Cholesterol; Citrates; Coenzyme A; Diph | 1965 |
Hypoglycin A: a specific inhibitor of isovaleryl CoA dehydrogenase.
Topics: Acidosis; Alanine; Amino Acid Metabolism, Inborn Errors; Animals; Butyrates; Caproates; Carbon Dioxi | 1971 |
Pathogenesis of ketonemia.
Topics: Acetoacetates; Acidosis; Animals; Carnitine; Coenzyme A; Diabetes Mellitus; Fasting; Gluconeogenesis | 1969 |
Carnitine and acetylcarnitine in the milk of normal and ketotic cows.
Topics: Acetoacetates; Acidosis; Animals; Blood Glucose; Carnitine; Cattle; Cattle Diseases; Fatty Acids; Ke | 1970 |
Turnover rates and intracellular pool size distribution of citrate cycle intermediates in normal, diabetic and fat-fed rats estimated by computer analysis from specific activity decay data of 14C-labeled citrate cycle acids.
Topics: Acetates; Acetoacetates; Acidosis; Animals; Aspartic Acid; Carbon Isotopes; Carnitine; Chromatograph | 1970 |
Effect of carnitine on acidosis and ketosis induced by lipid perfusions in dogs during starvation.
Topics: Acidosis; Animals; Carnitine; Dogs; Infusions, Parenteral; Ketone Bodies; Lipids; Starvation | 1965 |