Page last updated: 2024-10-16

carnitine and Acidosis

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.

Research Excerpts

ExcerptRelevanceReference
"Propofol infusion syndrome is a rare but frequently fatal complication in critically ill children given long-term propofol infusions."7.71Impaired fatty acid oxidation in propofol infusion syndrome. ( Segar, P; Shield, J; Stone, J; Weir, P; Wolf, A, 2001)
" Laboratory data showed hypoglycemia and hypocarnitinemia."3.74Carnitine-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."3.71Impaired 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."3.67Clinical disorders of muscle energy metabolism. ( Layzer, RB; Lewis, SF, 1984)
" Information on therapeutic usage, administration, and dosage was also recorded."1.36Setting 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."1.34Subacute 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."1.30Very 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."1.29The 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."1.29A 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."1.28Electron 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."1.27Ketosis, 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."1.27The 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."1.26Carnitine 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."1.26Hormonal control of ketogenesis. Biochemical considerations. ( Foster, DW; McGarry, JD, 1977)

Research

Studies (54)

TimeframeStudies, this research(%)All Research%
pre-199029 (53.70)18.7374
1990's11 (20.37)18.2507
2000's8 (14.81)29.6817
2010's5 (9.26)24.3611
2020's1 (1.85)2.80

Authors

AuthorsStudies
Tan, J3
Zheng, M3
Cai, R3
Zeng, T3
Yin, B3
Yang, J4
Wei, B3
Chang, R3
Jiang, Y3
Yuan, D3
Pan, L3
Huang, L3
Ning, H3
Wei, J3
Chen, D3
Liu, H1
Miao, JK1
Yu, CW1
Wan, KX1
Zhang, J1
Yuan, ZJ1
Wang, DJ1
Zeng, Y1
Zou, L1
Han, L1
Wu, S1
Ye, J1
Qiu, W1
Zhang, H1
Gao, X1
Wang, Y1
Gong, Z1
Jin, J1
Gu, X1
Kimmoun, A1
Abboud, G1
Strazeck, J1
Merten, M1
Guéant, JL1
Feillet, F1
Chace, DH1
Lim, T1
Hansen, CR1
Adam, BW1
Hannon, WH1
Fernandez-Llamazares, CM1
Serrano, ML1
Manrique-Rodríguez, S1
Sanjurjo-Sáez, M1
Rosa, M1
Pascarella, A1
Parenti, G1
Buono, S1
Romano, A1
Della Casa, R1
Andria, G1
Marino, M1
Riccio, MP1
Bravaccio, C1
Zwickler, T1
Haege, G1
Riderer, A1
Hörster, F1
Hoffmann, GF2
Burgard, P1
Kölker, S2
Schor, DS1
Feyh, P1
Wagner, L1
Jeffrey, I1
Pourfarzam, M1
Okun, JG1
Zschocke, J1
Baric, I1
Bain, MD1
Jakobs, C1
Chalmers, RA1
Distelmaier, F1
Vogel, M1
Spiekerkötter, U1
Gempel, K1
Klee, D1
Braunstein, S1
Groneck, HP1
Mayatepek, E1
Wendel, U1
Schwahn, B1
Makino, Y1
Sugiura, T1
Ito, T2
Sugiyama, N2
Koyama, N1
Delgado, C1
Macías, C1
de la Sierra García-Valdecasas, M1
Pérez, M1
del Portal, LR1
Jiménez, LM1
Beattie, MA2
Winder, WW2
Layzer, RB1
Lewis, SF1
Millington, DS2
Roe, CR3
Maltby, DA2
Morooka, K1
Hoppel, CL2
Genuth, SM1
Corr, PB1
Snyder, DW1
Cain, ME1
Crafford, WA1
Gross, RW1
Sobel, BE1
Peschechera, A1
Ferrari, LE1
Arrigoni-Martelli, E1
Hülsmann, WC1
Walter, JH1
Wraith, JE1
Cleary, MA1
Kumps, A1
Duez, P1
Mardens, Y1
Jooste, S1
Erasmus, E1
Mienie, LJ1
de Wet, WJ1
Gibson, KM2
Qureshi, IA1
Ratnakumari, L1
Michalak, A1
Giguère, R1
Cyr, D1
Butterworth, RF1
Kidouchi, K1
Kajita, M1
Chiba, T1
Niwa, T1
Wada, Y1
Fontaine, M1
Briand, G1
Ser, N1
Armelin, I1
Rolland, MO1
Degand, P1
Vamecq, J1
Sluysmans, T1
Tuerlinckx, D1
Hubinont, C1
Verellen-Dumoulin, C1
Brivet, M1
Vianey-Saban, C1
Weinberg, GL1
Laurito, CE1
Geldner, P1
Pygon, BH1
Burton, BK1
Eirís, J1
Ribes, A1
Fernández-Prieto, R1
Rodríguez-García, J1
Rodríguez-Segade, S1
Castro-Gago, M1
al-Essa, MA1
Rashed, MS1
Bakheet, SM1
Patay, ZJ1
Ozand, PT1
Wolf, A1
Weir, P1
Segar, P1
Stone, J1
Shield, J1
McGarry, JD3
Foster, DW3
Engel, AG2
Banker, BQ1
Eiben, RM1
Robles-Valdes, C2
Bell, RB1
Brownell, AK1
Goodman, SI1
Frerman, FE1
Seccombe, DW1
Snyder, FF1
Dasouki, M1
Buchanan, D1
Mercer, N1
Thoene, J1
Böhles, H1
Peters, WH1
Seim, H1
Löster, H1
Strack, E1
Lubs, H1
Kohnert, KD1
Carlin, JI1
Olson, EB1
Peters, HA1
Reddan, WG1
Rebouche, CJ1
Paulson, DJ1
Bahl, JJ1
Bressler, R2
Dugan, RE1
Schmidt, MJ1
Hoganson, GE1
Steele, J1
Gilles, BA1
Shug, AL1
Erfle, JD5
Sauer, F4
Sauer, FD1
Fisher, LJ3
Katz, R1
Wittels, B1
Tanaka, K1
Miller, EM1
Isselbacher, KJ1
Bremer, J1
Binns, MR1
Broekhuysen, J1
Baudine, A1
Deltour, G1

Clinical Trials (4)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
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)Observational2004-10-31Completed
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 142 participants Interventional2003-09-30Completed
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 240 participants (Actual)Interventional2008-04-30Completed
Multi-center Phase II Trial of Valproic Acid and Carnitine in Patients With Spinal Muscular Atrophy (SMA CARNI-VAL Trial)[NCT00227266]Phase 294 participants (Actual)Interventional2005-09-30Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Anthropometric Measures of Nutritional Status (Body Mass Index [BMI] Z-scores, Weight for Length Ratios, Lean/Fat Mass Via DEXA, Growth Parameters, and Triceps Skinfold Measures)

(NCT00661453)
Timeframe: -2 weeks, time 0, 3 months, 6 months

Interventiong (Mean)
Lean Mass BaselineLean Mass 3 monthsLean Mass 6 monthsFat Mass BaselineFat Mass 3 monthsFat Mass 6 months
SMA Type 14317.154993.925133.833011.373618.254316.08

Max CMAP Amplitude (Mean)

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)

,,
InterventionmV (Mean)
Baseline6 months
Cohort 1a Sitters Placebo Then Treatment2.282.32
Cohort 1b Sitters Treatment2.932.37
Cohort 2 Standers and Walkers - Treatment5.526.56

Max CMAP Amplitude Median

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)

,,
InterventionmV (Median)
Baseline6 months
Cohort 1a Sitters Placebo Then Treatment1.911.44
Cohort 1b Sitters Treatment2.21.8
Cohort 2 Standers and Walkers - Treatment5.35.85

Max CMAP Area (Mean)

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)

,,
InterventionmVms (Mean)
Baseline6 months
Cohort 1a Sitters Placebo Then Treatment5.465.28
Cohort 1b Sitters Treatment5.455.26
Cohort 2 Standers and Walkers - Treatment14.8516.26

Max CMAP Area (Median)

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)

,,
InterventionmVms (Median)
Baseline6 months
Cohort 1a Sitters Placebo Then Treatment3.63.74
Cohort 1b Sitters Treatment4.63.4
Cohort 2 Standers and Walkers - Treatment13.6516.85

Modified Hammersmith Change From Baseline to 6 Months

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

,
InterventionScore (Mean)
Baseline visit (0 weeks)6 Month visit (V2)Change from Baseline
Cohort 1a Sitters Placebo Then Treatment20.020.60.6
Cohort 1b Sitters Treatment16.616.80.2

Modified Hammersmith Extend Baseline

"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)

InterventionScore (Mean)
Modified Hammersmith Extend at S1 (-4 weeks)Modified Hammersmith Extend at S2 (0 weeks)
Cohort 2 Experimental47.048.3

Reviews

3 reviews available for carnitine and Acidosis

ArticleYear
[Benign myopathy in children].
    Nihon rinsho. Japanese journal of clinical medicine, 1982, Volume: 40, Issue:7

    Topics: Acidosis; Adolescent; Adult; Age Factors; Carnitine; Carnitine O-Palmitoyltransferase; Child; Child,

1982
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
Carnitine metabolism and function in humans.
    Annual review of nutrition, 1986, Volume: 6

    Topics: Absorption; Acidosis; Adolescent; Adult; Aged; Animals; Biological Transport; Cardiomyopathies; Carn

1986
The pharmacology of carnitine.
    Annual review of pharmacology and toxicology, 1987, Volume: 27

    Topics: Acidosis; Animals; Cardiomyopathies; Carnitine; Humans; Infant, Newborn; Renal Dialysis; Reye Syndro

1987

Other Studies

51 other studies available for carnitine and Acidosis

ArticleYear
[Analysis of IVD gene variants in four children with isovalerate acidemia].
    Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2022, Dec-10, Volume: 39, Issue:12

    Topics: Acidosis; Carnitine; Child; Erythrocytes; High-Throughput Nucleotide Sequencing; Humans; Infant, New

2022
[Analysis of IVD gene variants in four children with isovalerate acidemia].
    Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2022, Dec-10, Volume: 39, Issue:12

    Topics: Acidosis; Carnitine; Child; Erythrocytes; High-Throughput Nucleotide Sequencing; Humans; Infant, New

2022
[Analysis of IVD gene variants in four children with isovalerate acidemia].
    Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2022, Dec-10, Volume: 39, Issue:12

    Topics: Acidosis; Carnitine; Child; Erythrocytes; High-Throughput Nucleotide Sequencing; Humans; Infant, New

2022
[Analysis of IVD gene variants in four children with isovalerate acidemia].
    Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2022, Dec-10, Volume: 39, Issue:12

    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.
    BMC pediatrics, 2019, 10-09, Volume: 19, Issue:1

    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.
    American journal of medical genetics. Part A, 2015, Volume: 167A, Issue:10

    Topics: Acetylcarnitine; Acidosis; Asian People; Asymptomatic Diseases; Carboxy-Lyases; Carnitine; Carrier P

2015
Acute decompensation of isovaleric acidemia induced by Graves' disease.
    Intensive care medicine, 2008, Volume: 34, Issue:12

    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.
    Clinica chimica acta; international journal of clinical chemistry, 2009, Volume: 402, Issue:1-2

    Topics: Acidosis; Carnitine; Humans; Infant, Newborn; Isotopes; Malonates; Reference Standards; Tandem Mass

2009
Setting up an emergency stock for metabolic diseases.
    La Clinica terapeutica, 2010, Volume: 161, Issue:6

    Topics: Acidosis; Amino Acids; Carnitine; Chelating Agents; Disease Management; Electronic Prescribing; Emer

2010
Developmental evolution in a patient with multiple acyl-coenzymeA dehydrogenase deficiency under pharmacological treatment.
    European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society, 2012, Volume: 16, Issue:2

    Topics: Acidosis; Acyl-CoA Dehydrogenase; Amino Acid Metabolism, Inborn Errors; Carnitine; Child Development

2012
Metabolic decompensation in methylmalonic aciduria: which biochemical parameters are discriminative?
    Journal of inherited metabolic disease, 2012, Volume: 35, Issue:5

    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.
    Neuropediatrics, 2003, Volume: 34, Issue:5

    Topics: Acidosis; Acute Disease; Adolescent; Anticonvulsants; Atrophy; Brain; Carnitine; DNA Mutational Anal

2003
Cystic renal dysplasia as a leading sign of inherited metabolic disease.
    Pediatric nephrology (Berlin, Germany), 2007, Volume: 22, Issue:12

    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.
    Pediatrics, 2007, Volume: 120, Issue:3

    Topics: Acidosis; Anti-Bacterial Agents; Brain Diseases, Metabolic; Carnitine; Cephalosporins; Humans; Hypog

2007
Subacute presentation of propionic acidemia.
    Journal of child neurology, 2007, Volume: 22, Issue:12

    Topics: Acidosis; Amino Acids; Biotin; Brain; Carnitine; Diagnosis, Differential; Diet, Protein-Restricted;

2007
Mechanism of training-induced attenuation of postexercise ketosis.
    The American journal of physiology, 1984, Volume: 247, Issue:5 Pt 2

    Topics: 3-Hydroxybutyric Acid; Acidosis; Animals; Carnitine; Cyclic AMP; Hydroxybutyrates; Ketosis; Liver; L

1984
Clinical disorders of muscle energy metabolism.
    Medicine and science in sports and exercise, 1984, Volume: 16, Issue:5

    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.
    Biomedical mass spectrometry, 1984, Volume: 11, Issue:5

    Topics: Acetylcarnitine; Acidosis; Carnitine; Humans; Magnetic Resonance Spectroscopy; Malonates; Mass Spect

1984
Urinary excretion of acetylcarnitine during human diabetic and fasting ketosis.
    The American journal of physiology, 1982, Volume: 243, Issue:2

    Topics: Acetylcarnitine; Acidosis; Adolescent; Adult; Aged; Body Weight; Carnitine; Child; Diabetic Ketoacid

1982
Carnitine and carnitine palmitoyltransferase in fatty acid oxidation and ketosis.
    Federation proceedings, 1982, Volume: 41, Issue:12

    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.
    Circulation research, 1981, Volume: 49, Issue:2

    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.
    Molecular and cellular biochemistry, 1995, Jan-26, Volume: 142, Issue:2

    Topics: Acidosis; Animals; Carnitine; Cattle; Cell Membrane; Cells, Cultured; Endothelium, Vascular; Female;

1995
Absence of acidosis in the initial presentation of propionic acidaemia.
    Archives of disease in childhood. Fetal and neonatal edition, 1995, Volume: 72, Issue:3

    Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carnitine; Food Pr

1995
Gas chromatographic profiling and determination of urinary acylcarnitines.
    Journal of chromatography. B, Biomedical applications, 1994, Aug-19, Volume: 658, Issue:2

    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.
    Clinica chimica acta; international journal of clinical chemistry, 1994, Oct-14, Volume: 230, Issue:1

    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.
    Biochemical medicine and metabolic biology, 1993, Volume: 50, Issue:2

    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.
    Journal of chromatography. B, Biomedical applications, 1995, Aug-18, Volume: 670, Issue:2

    Topics: Acidosis; Administration, Oral; Carnitine; Child, Preschool; Chromatography, Liquid; Female; Glucuro

1995
Metabolic studies in twin brothers with 2-methylacetoacetyl-CoA thiolase deficiency.
    Clinica chimica acta; international journal of clinical chemistry, 1996, Nov-15, Volume: 255, Issue:1

    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.
    The Journal of pediatrics, 1997, Volume: 131, Issue:3

    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.
    Journal of clinical anesthesia, 1997, Volume: 9, Issue:8

    Topics: Acidosis; Adolescent; Anesthesia, General; Anesthesia, Local; Anesthetics, Local; Bupivacaine; Carni

1997
[3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome].
    Revista de neurologia, 1998, Volume: 26, Issue:154

    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.
    Journal of perinatology : official journal of the California Perinatal Association, 2000, Volume: 20, Issue:2

    Topics: Acidosis; Age of Onset; Brain; Carnitine; Child; Consanguinity; Female; Glutarates; Humans; Infant;

2000
Impaired fatty acid oxidation in propofol infusion syndrome.
    Lancet (London, England), 2001, Feb-24, Volume: 357, Issue:9256

    Topics: Acidosis; Carnitine; Critical Care; Electron Transport; Fatty Acids; Hemofiltration; Humans; Hypnoti

2001
Hormonal control of ketogenesis. Biochemical considerations.
    Archives of internal medicine, 1977, Volume: 137, Issue:4

    Topics: Acetoacetates; Acidosis; Adipose Tissue; Alcoholism; Animals; Carnitine; Carnitine Acyltransferases;

1977
Carnitine deficiency: clinical, morphological, and biochemical observations in a fatal case.
    Journal of neurology, neurosurgery, and psychiatry, 1977, Volume: 40, Issue:4

    Topics: Acidosis; Carnitine; Child; Hepatomegaly; Humans; Male; Mitochondria, Muscle; Muscles; Muscular Dise

1977
Maternal-fetal carnitine relationship and neonatal ketosis in the rat.
    The Journal of biological chemistry, 1976, Oct-10, Volume: 251, Issue:19

    Topics: Acidosis; Aging; Animals; Animals, Newborn; Body Weight; Carnitine; Fasting; Female; Fetus; Ketone B

1976
Glucagon and ketogenesis.
    Metabolism: clinical and experimental, 1976, Volume: 25, Issue:11 Suppl 1

    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.
    Neurology, 1990, Volume: 40, Issue:11

    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.
    Journal of inherited metabolic disease, 1987, Volume: 10, Issue:2

    Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Carnitine; Dietary Fats; Dietary Proteins; Female; G

1987
Attenuation of postexercise ketosis in fasted endurance-trained rats.
    The American journal of physiology, 1985, Volume: 248, Issue:1 Pt 2

    Topics: 3-Hydroxybutyric Acid; Acidosis; Adaptation, Physiological; Animals; Carnitine; Cyclic AMP; Fasting;

1985
[When is parenteral carnitine administration indicated?].
    Beitrage zu Infusionstherapie und klinische Ernahrung, 1986, Volume: 13

    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.
    Experimental and clinical endocrinology, 1987, Volume: 90, Issue:1

    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.
    Biomedical & environmental mass spectrometry, 1987, Volume: 14, Issue:12

    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.
    The Journal of physiology, 1987, Volume: 390

    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.
    Analytical biochemistry, 1987, Feb-01, Volume: 160, Issue:2

    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.
    The Journal of biological chemistry, 1967, May-10, Volume: 242, Issue:9

    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.
    Journal of dairy science, 1974, Volume: 57, Issue:6

    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.
    Journal of dairy science, 1971, Volume: 54, Issue:5

    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.
    Annals of the New York Academy of Sciences, 1965, Oct-08, Volume: 131, Issue:1

    Topics: Acetoacetates; Acidosis; Adipose Tissue; Animals; Carnitine; Cholesterol; Citrates; Coenzyme A; Diph

1965
Hypoglycin A: a specific inhibitor of isovaleryl CoA dehydrogenase.
    Proceedings of the National Academy of Sciences of the United States of America, 1971, Volume: 68, Issue:1

    Topics: Acidosis; Alanine; Amino Acid Metabolism, Inborn Errors; Animals; Butyrates; Caproates; Carbon Dioxi

1971
Pathogenesis of ketonemia.
    Scandinavian journal of clinical and laboratory investigation, 1969, Volume: 23, Issue:2

    Topics: Acetoacetates; Acidosis; Animals; Carnitine; Coenzyme A; Diabetes Mellitus; Fasting; Gluconeogenesis

1969
Carnitine and acetylcarnitine in the milk of normal and ketotic cows.
    Journal of dairy science, 1970, Volume: 53, Issue:4

    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.
    European journal of biochemistry, 1970, Volume: 17, Issue:2

    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.
    Biochimica et biophysica acta, 1965, Jul-07, Volume: 106, Issue:1

    Topics: Acidosis; Animals; Carnitine; Dogs; Infusions, Parenteral; Ketone Bodies; Lipids; Starvation

1965