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dichloroacetic acid and Pyruvate Dehydrogenase Complex Deficiency Disease

dichloroacetic acid has been researched along with Pyruvate Dehydrogenase Complex Deficiency Disease in 19 studies

Research

Studies (19)

TimeframeStudies, this research(%)All Research%
pre-19905 (26.32)18.7374
1990's5 (26.32)18.2507
2000's6 (31.58)29.6817
2010's3 (15.79)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Bliksrud, YT; Pedersen, S; Ramm-Pettersen, A; Selmer, KK1
Buchsbaum, R; Lawson, LA; Nixon, SJ; Prather, RA; Shuster, J; Stacpoole, PW; Thompson, JLPS; Zou, B1
Brunetti-Pierri, N; Ferriero, R; Iannuzzi, C; Manco, G1
Benelli, C; Boutron, A; Brivet, M; Fouque, F; Marsac, C; Vequaud, C; Zabot, MT1
Barnes, C; Bunch, ST; Carney, PR; Felitsyn, NM; Fennell, EM; Gilmore, RL; Greer, M; Henderson, GN; Hutson, AD; Kerr, DS; Neiberger, RE; O'Brien, RG; Perkins, LA; Quisling, RG; Shroads, AL; Shuster, JJ; Silverstein, JH; Stacpoole, PW; Theriaque, DW; Valenstein, E1
Berendzen, K; Shuster, J; Stacpoole, PW; Theriaque, DW1
Berendzen, K; Chouthai, N; Han, Z; Maina, N; Srivastava, A; Stacpoole, PW; Surolia, I; Zhao, W; Zhong, L1
Carney, PR; Gilbert, LR; Neiberger, RE; Shuster, JJ; Stacpoole, PW; Theriaque, DW; Valenstein, E1
Hu, CW; Sheu, KF; Utter, MF1
Arai, K; Harada, M; Hashimoto, T; Miyoshi, H; Nishitani, H; Tanouchi, M1
Barnes, CL; Cannon, SL; Hurbanis, MD; Kerr, DS; Stacpoole, PW1
Bachert, P; Langhans, CD; Mayatepek, E; Rating, D; Ruitenbeek, W; Schulze, A1
Beattie, P; Brown, GK; Matthews, PM; Morten, KJ1
Chen, S; Ito, M; Kuroda, Y; Maehara, M; Naito, E; Saijo, T; Yokota, I1
George, JC; Hutson, AD; Neiberger, RE; Perkins, LA; Stacpoole, PW; Theriaque, DW1
DeVivo, DC; Haymond, MW; Nelson, JS; Obert, KA; Pagliara, AS1
Kobashi, H; Kuroda, Y; Miyao, M; Naito, E; Takeda, E; Yokota, I1
Miyabayashi, S; Narisawa, K1
MacMillan, H; Petrova-Benedict, R; Robinson, BH; Sherwood, WG1

Reviews

2 review(s) available for dichloroacetic acid and Pyruvate Dehydrogenase Complex Deficiency Disease

ArticleYear
Pyruvate dehydrogenase deficiency.
    Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke, 2019, 10-22, Volume: 139, Issue:15

    Topics: Diagnosis, Differential; Dichloroacetic Acid; Diet, Ketogenic; Humans; Pyruvate Dehydrogenase Complex; Pyruvate Dehydrogenase Complex Deficiency Disease; Thiamine

2019
Concomitant administration of sodium dichloroacetate and thiamine in west syndrome caused by thiamine-responsive pyruvate dehydrogenase complex deficiency.
    Journal of the neurological sciences, 1999, Dec-01, Volume: 171, Issue:1

    Topics: Amino Acid Substitution; Dichloroacetic Acid; DNA Mutational Analysis; Drug Therapy, Combination; Female; Humans; Infant; Lactic Acid; Pyruvate Dehydrogenase (Lipoamide); Pyruvate Dehydrogenase Complex; Pyruvate Dehydrogenase Complex Deficiency Disease; Sex Factors; Spasms, Infantile; Thiamine

1999

Trials

3 trial(s) available for dichloroacetic acid and Pyruvate Dehydrogenase Complex Deficiency Disease

ArticleYear
Development of a novel observer reported outcome tool as the primary efficacy outcome measure for a rare disease randomized controlled trial.
    Mitochondrion, 2018, Volume: 42

    Topics: Child; Child, Preschool; Cross-Over Studies; Dichloroacetic Acid; Female; Humans; Male; Placebos; Pyruvate Dehydrogenase Complex Deficiency Disease; Rare Diseases; Symptom Assessment; Treatment Outcome

2018
Controlled clinical trial of dichloroacetate for treatment of congenital lactic acidosis in children.
    Pediatrics, 2006, Volume: 117, Issue:5

    Topics: Acidosis, Lactic; Adolescent; Adult; Child; Child, Preschool; Dichloroacetic Acid; Female; Humans; Infant; Lactates; Male; Mitochondrial Diseases; Neurologic Examination; Neuropsychological Tests; Pyruvate Dehydrogenase Complex Deficiency Disease; Quality of Life

2006
Evaluation of long-term treatment of children with congenital lactic acidosis with dichloroacetate.
    Pediatrics, 2008, Volume: 121, Issue:5

    Topics: Acidosis, Lactic; Administration, Oral; Adolescent; Adult; Child; Child, Preschool; Dichloroacetic Acid; Double-Blind Method; Female; Humans; Infant; Kaplan-Meier Estimate; Lactic Acid; Male; Mitochondrial Encephalomyopathies; Neural Conduction; Pyruvate Dehydrogenase Complex Deficiency Disease

2008

Other Studies

14 other study(ies) available for dichloroacetic acid and Pyruvate Dehydrogenase Complex Deficiency Disease

ArticleYear
Differential inhibition of PDKs by phenylbutyrate and enhancement of pyruvate dehydrogenase complex activity by combination with dichloroacetate.
    Journal of inherited metabolic disease, 2015, Volume: 38, Issue:5

    Topics: Animals; Binding Sites; Cells, Cultured; Dichloroacetic Acid; Enzyme Activation; Humans; Male; Mice; Mice, Inbred C57BL; Phenylbutyrates; Protein Serine-Threonine Kinases; Pyruvate Dehydrogenase Acetyl-Transferring Kinase; Pyruvate Dehydrogenase Complex; Pyruvate Dehydrogenase Complex Deficiency Disease

2015
Differential effect of DCA treatment on the pyruvate dehydrogenase complex in patients with severe PDHC deficiency.
    Pediatric research, 2003, Volume: 53, Issue:5

    Topics: Cells, Cultured; Dichloroacetic Acid; Dihydrolipoyllysine-Residue Acetyltransferase; Enzyme Activation; Female; Fibroblasts; Humans; In Vitro Techniques; Infant; Lymphocytes; Male; Pyruvate Dehydrogenase (Lipoamide); Pyruvate Dehydrogenase Complex; Pyruvate Dehydrogenase Complex Deficiency Disease; Severity of Illness Index

2003
Therapeutic potential of dichloroacetate for pyruvate dehydrogenase complex deficiency.
    Mitochondrion, 2006, Volume: 6, Issue:3

    Topics: Acidosis, Lactic; Adolescent; Adult; Child; Child, Preschool; Dichloroacetic Acid; Female; Humans; Infant; Infant, Newborn; Male; Middle Aged; Mitochondria; Models, Biological; Mutation; Pyruvate Dehydrogenase Complex; Pyruvate Dehydrogenase Complex Deficiency Disease

2006
A combined therapeutic approach for pyruvate dehydrogenase deficiency using self-complementary adeno-associated virus serotype-specific vectors and dichloroacetate.
    Molecular genetics and metabolism, 2008, Volume: 93, Issue:4

    Topics: Cells, Cultured; Dependovirus; Dichloroacetic Acid; Fibroblasts; Genetic Therapy; Genetic Vectors; Humans; Pyruvate Dehydrogenase (Lipoamide); Pyruvate Dehydrogenase Complex Deficiency Disease; Transduction, Genetic

2008
Pyruvate dehydrogenase complex activity in normal and deficient fibroblasts.
    The Journal of clinical investigation, 1981, Volume: 67, Issue:5

    Topics: Acetyl Coenzyme A; Carbon Dioxide; Cell-Free System; Cells, Cultured; Dichloroacetic Acid; Enzyme Activation; Fluorides; Humans; Phosphoprotein Phosphatases; Phosphorylation; Pyruvate Dehydrogenase Complex; Pyruvate Dehydrogenase Complex Deficiency Disease; Skin

1981
Therapeutic efficacy of a case of pyruvate dehydrogenase complex deficiency monitored by localized proton magnetic resonance spectroscopy.
    Magnetic resonance imaging, 1996, Volume: 14, Issue:1

    Topics: Brain; Brain Chemistry; Dichloroacetic Acid; Humans; Infant; Lactates; Lactic Acid; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Pyruvate Dehydrogenase Complex Deficiency Disease; Pyruvates; Pyruvic Acid

1996
Treatment of congenital lactic acidosis with dichloroacetate.
    Archives of disease in childhood, 1997, Volume: 77, Issue:6

    Topics: Acidosis, Lactic; Adolescent; Child; Dichloroacetic Acid; Female; Humans; Infant; Infant, Newborn; Male; Mutation; Pyruvate Dehydrogenase Complex Deficiency Disease

1997
In vivo methods useful for therapy monitoring in lactic acidosis.
    Journal of inherited metabolic disease, 1998, Volume: 21, Issue:6

    Topics: Acidosis, Lactic; Carbon Isotopes; Carbon Radioisotopes; Dichloroacetic Acid; Female; Humans; Infant; Magnetic Resonance Imaging; Monitoring, Physiologic; Pyruvate Dehydrogenase Complex Deficiency Disease

1998
Dichloroacetate stabilizes the mutant E1alpha subunit in pyruvate dehydrogenase deficiency.
    Neurology, 1999, Aug-11, Volume: 53, Issue:3

    Topics: Cell Line; Cells, Cultured; Dichloroacetic Acid; Humans; Immunoblotting; Pyruvate Dehydrogenase Complex Deficiency Disease

1999
Renal manifestations of congenital lactic acidosis.
    American journal of kidney diseases : the official journal of the National Kidney Foundation, 2002, Volume: 39, Issue:1

    Topics: Acidosis, Lactic; Adolescent; Adult; Bicarbonates; Blood Urea Nitrogen; Calcium; Child; Child, Preschool; Creatinine; Dichloroacetic Acid; Female; Humans; Infant; Kidney Diseases; Kidney Tubules; Lactic Acid; Male; Parathyroid Hormone; Phosphates; Pyruvate Dehydrogenase Complex Deficiency Disease; Treatment Outcome; Vitamin D

2002
Defective activation of the pyruvate dehydrogenase complex in subacute necrotizing encephalomyelopathy (Leigh disease).
    Annals of neurology, 1979, Volume: 6, Issue:6

    Topics: Alanine; Blood Glucose; Brain; Brain Diseases; Decarboxylation; Dichloroacetic Acid; Enzyme Activation; Fibroblasts; Humans; In Vitro Techniques; Infant; Liver; Necrosis; Phosphorylation; Pyruvate Dehydrogenase Complex Deficiency Disease; Syndrome

1979
Detection of pyruvate metabolism disorders by culture of skin fibroblasts with dichloroacetate.
    Pediatric research, 1988, Volume: 23, Issue:6

    Topics: Acetates; Acidosis, Lactic; Cells, Cultured; Child; Dichloroacetic Acid; Enzyme Activation; Female; Fibroblasts; Humans; Infant; Male; Mitochondria; Phosphoprotein Phosphatases; Pyruvate Dehydrogenase Complex Deficiency Disease; Pyruvates; Pyruvic Acid

1988
[Clinical and biochemical approach to mitochondrial cytopathy--pyruvate dehydrogenase complex deficiency].
    No to hattatsu = Brain and development, 1987, Volume: 19, Issue:2

    Topics: Child, Preschool; Dichloroacetic Acid; Humans; Infant; Infant, Newborn; Leigh Disease; Microscopy, Electron; Mitochondria, Muscle; Muscles; Prenatal Diagnosis; Pyruvate Dehydrogenase Complex Deficiency Disease

1987
Variable clinical presentation in patients with defective E1 component of pyruvate dehydrogenase complex.
    The Journal of pediatrics, 1987, Volume: 111, Issue:4

    Topics: Acidosis, Lactic; Adolescent; Brain Diseases; Cells, Cultured; Child; Child, Preschool; Dichloroacetic Acid; Fibroblasts; Humans; Infant; Infant, Newborn; Intellectual Disability; Muscle Hypotonia; Pyruvate Dehydrogenase Complex Deficiency Disease; Skin

1987