Page last updated: 2024-10-17

lactic acid and Carbohydrate Metabolism, Inborn Error

lactic acid has been researched along with Carbohydrate Metabolism, Inborn Error in 9 studies

Lactic Acid: A normal intermediate in the fermentation (oxidation, metabolism) of sugar. The concentrated form is used internally to prevent gastrointestinal fermentation. (From Stedman, 26th ed)
2-hydroxypropanoic acid : A 2-hydroxy monocarboxylic acid that is propanoic acid in which one of the alpha-hydrogens is replaced by a hydroxy group.

Research Excerpts

ExcerptRelevanceReference
"Five patients became seizure-free with the onset of ketosis, and anticonvulsants were discontinued."1.42The Effects of Ketogenic Diet on Seizures, Cognitive Functions, and Other Neurological Disorders in Classical Phenotype of Glucose Transporter 1 Deficiency Syndrome. ( Bayram, AK; Çağlayan, AO; Canpolat, M; Gumus, H; Kardas, F; Kendirci, M; Kumandas, S; Per, H, 2015)
"9 mmol/l, respectively, developed in three patients with hyperosmolar syndromes (two hypernatremic, 417 and 415 mosmol/kg H2O; one hyperglycemic 437 mosmol/kg H2O) during rehydration treatment with 5% fructose in water (fructose dosage 0."1.27Fructose-induced hyperlactemia in hyperosmolar syndromes. ( Druml, W; Kleinberger, G; Laggner, A; Lenz, K; Schneeweiss, B, 1986)

Research

Studies (9)

TimeframeStudies, this research(%)All Research%
pre-19904 (44.44)18.7374
1990's0 (0.00)18.2507
2000's4 (44.44)29.6817
2010's1 (11.11)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Gumus, H1
Bayram, AK1
Kardas, F1
Canpolat, M1
Çağlayan, AO1
Kumandas, S1
Kendirci, M1
Per, H1
van Baalen, A1
Stephani, U1
Rohr, A1
ISRAELS, S1
HAWORTH, JC1
GOURLEY, B1
FORD, JD1
Overweg-Plandsoen, WC1
Groener, JE1
Wang, D2
Onkenhout, W1
Brouwer, OF1
Bakker, HD1
De Vivo, DC2
Pascual, JM1
Yang, H1
Engelstad, K1
Jhung, S1
Sun, RP1
Gudmand-Høyer, E1
Krasilnikoff, PA1
Skovbjerg, H1
Merezhinskaya, N1
Fishbein, WN1
Davis, JI1
Foellmer, JW1
Lemonnier, F1
Delhotal-Landes, B1
Couturier, M1
Decimo, D1
Odiévre, M1
Gautier, M1
Lemonnier, A1
Druml, W1
Kleinberger, G1
Lenz, K1
Laggner, A1
Schneeweiss, B1

Clinical Trials (4)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Post Study Continuation of C7 for G1D[NCT02018302]0 participants Expanded AccessNo longer available
Treatment Development of Triheptanoin for Glucose Transporter Type I Deficiency[NCT02021526]Phase 1/Phase 20 participants (Actual)Interventional2015-12-31Withdrawn (stopped due to NIH funding resulted in new clinical trial)
The Glucose Transporter Type I Deficiency (G1D) Registry[NCT02013583]750 participants (Anticipated)Observational [Patient Registry]2013-12-31Recruiting
Clinical Trial of Citric Acid Cycle Stimulation in Energy-deficiency States: Treatment Development for Glucose Transporter Type I Deficiency Syndrome (G1D) (NMTUT 2010B)[NCT02018315]Phase 114 participants (Actual)Interventional2012-01-31Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Number of Participants With Change in Brain Metabolic Rate After 3 Months

Magnetic Resonance Imaging (MRI) used to calculate brain metabolic rate. Brain metabolic rate compared before oil ingestion (Baseline), 90 minutes after oil ingestion, and after 3 months of daily oil ingestion in each participant. Triheptanoin metabolism may lead to increased oxygen consumption only while the brain undergoes a reduction of ictogenesis. We hypothesize that when ictogenesis is abolished by triheptanoin or absent at baseline, triheptanoin exerts little or no effect on CMR02. (NCT02018315)
Timeframe: 3 months

InterventionParticipants (Count of Participants)
Experimental: Triheptanoin5

Number of Participants With Reduction in Spike-wave Fraction of the EEG Recording Time

Visual analysis of EEG recording to determine the fraction of spike-range within the area of recording. (NCT02018315)
Timeframe: 1 day

InterventionParticipants (Count of Participants)
Experimental: Triheptanoin13

Reviews

1 review available for lactic acid and Carbohydrate Metabolism, Inborn Error

ArticleYear
Sucrose-isomaltose malabsorption.
    Advances in nutritional research, 1984, Volume: 6

    Topics: Animals; Barium; Breath Tests; Carbohydrate Metabolism, Inborn Errors; Disaccharidases; Disaccharide

1984

Trials

2 trials available for lactic acid and Carbohydrate Metabolism, Inborn Error

ArticleYear
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects.
    Annals of neurology, 2005, Volume: 57, Issue:1

    Topics: 3-O-Methylglucose; Adolescent; Blood Glucose; Brain; Carbohydrate Metabolism, Inborn Errors; Child;

2005
Mutations in MCT1 cDNA in patients with symptomatic deficiency in lactate transport.
    Muscle & nerve, 2000, Volume: 23, Issue:1

    Topics: Adult; Arm; Biological Transport, Active; Carbohydrate Metabolism, Inborn Errors; Carrier Proteins;

2000

Other Studies

6 other studies available for lactic acid and Carbohydrate Metabolism, Inborn Error

ArticleYear
The Effects of Ketogenic Diet on Seizures, Cognitive Functions, and Other Neurological Disorders in Classical Phenotype of Glucose Transporter 1 Deficiency Syndrome.
    Neuropediatrics, 2015, Volume: 46, Issue:5

    Topics: Adolescent; Biomarkers; Blood Glucose; Carbohydrate Metabolism, Inborn Errors; Child; Child, Prescho

2015
Increased brain lactate during stroke-like episode in a patient with congenital disorder of glycosylation type Ia.
    Brain & development, 2009, Volume: 31, Issue:2

    Topics: Acidosis, Lactic; Aspartic Acid; Brain; Carbohydrate Metabolism, Inborn Errors; Child; Female; Fever

2009
CHRONIC ACIDOSIS DUE TO AN ERROR IN LACTATE AND PYRUVATE METABOLISM. REPORT OF TWO CASES.
    Pediatrics, 1964, Volume: 34

    Topics: Acetoacetates; Acidosis; Blood; Blood Gas Analysis; Carbohydrate Metabolism; Carbohydrate Metabolism

1964
GLUT-1 deficiency without epilepsy--an exceptional case.
    Journal of inherited metabolic disease, 2003, Volume: 26, Issue:6

    Topics: Ataxia; Blood Glucose; Carbohydrate Metabolism, Inborn Errors; Child; DNA; DNA Mutational Analysis;

2003
Comparative use of glucose and fructose in cultured fibroblasts from patients with hereditary fructose intolerance.
    Journal of inherited metabolic disease, 1987, Volume: 10, Issue:1

    Topics: Carbohydrate Metabolism, Inborn Errors; Cells, Cultured; Child; Child, Preschool; Female; Fibroblast

1987
Fructose-induced hyperlactemia in hyperosmolar syndromes.
    Klinische Wochenschrift, 1986, Jul-01, Volume: 64, Issue:13

    Topics: Acidosis; Blood Glucose; Carbohydrate Metabolism, Inborn Errors; Diabetic Coma; Electrolytes; Female

1986