Page last updated: 2024-10-26

valproic acid and Fatty Liver with Encephalopathy

valproic acid has been researched along with Fatty Liver with Encephalopathy in 23 studies

Valproic Acid: A fatty acid with anticonvulsant and anti-manic properties that is used in the treatment of EPILEPSY and BIPOLAR DISORDER. The mechanisms of its therapeutic actions are not well understood. It may act by increasing GAMMA-AMINOBUTYRIC ACID levels in the brain or by altering the properties of VOLTAGE-GATED SODIUM CHANNELS.
valproic acid : A branched-chain saturated fatty acid that comprises of a propyl substituent on a pentanoic acid stem.

Research Excerpts

ExcerptRelevanceReference
"5 of these patients developed toxic encephalopathy with hyperammonemia induced by VPA."1.27Treatment of epileptic patients with valproic acid does not modify plasma and urine short-chain-fatty acids. ( Arnetoli, G; Boncinelli, L; Campostrini, R; Paganini, M; Zaccara, G; Zappoli, R, 1983)
"This case study shows that Reye's syndrome causes altered valproate metabolism, consistent with the defective mitochondrial beta-oxidation of medium chain fatty acids, and suggests that valproic acid should not be used in the treatment of seizures in patients with this syndrome."1.27Altered metabolic profiles of valproic acid in a patient with Reye's syndrome. ( Fujii, H; Inoue, Y; Kitamura, K; Kuhara, T; Matsumoto, I; Matsumoto, M; Sakura, N; Shinka, T, 1985)
"She developed the typical features of Reye syndrome."1.26Decreased serum carnitine in valproate induced Reye syndrome. ( Böhles, H; Richter, K; Schäfer, H; Wagner-Thiessen, E, 1982)

Research

Studies (23)

TimeframeStudies, this research(%)All Research%
pre-199018 (78.26)18.7374
1990's4 (17.39)18.2507
2000's1 (4.35)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Coulter, DL1
Kim, CS1
O'Tuama, LA1
Mann, JD1
Roe, CR1
Sugimoto, T1
Nishida, N1
Yasuhara, A1
Ono, A1
Sakane, Y1
Matsumura, T1
Zaccara, G1
Boncinelli, L1
Paganini, M1
Campostrini, R1
Arnetoli, G1
Zappoli, R1
Berlin, CM1
Powell-Jackson, PR1
Tredger, JM1
Williams, R1
Young, RS1
Bergman, I1
Gang, DL1
Richardson, EP1
Keene, DL1
Humphreys, P1
Carpenter, B1
Fletcher, JP1
Böhles, H1
Richter, K1
Wagner-Thiessen, E1
Schäfer, H1
Seghieri, G1
De Giorgio, LA1
Innocenti, F1
Pettiná, G1
Zolfanelli, F1
Apicella, P1
Treem, WR1
Trost, LC1
Lemasters, JJ1
Forget, PP1
van Oosterhout, M1
Bakker, JA1
Wermuth, B1
Vles, JS1
Spaapen, LJ1
Yamanouchi, H1
Imataka, G1
Gerber, N1
Dickinson, RG1
Harland, RC1
Lynn, RK1
Houghton, LD1
Antonias, JI1
Schimschock, JC1
Ozawa, Y1
Inagaki, Y1
Yonei, Y1
Tsukada, N1
Okawa, H1
Kiryu, Y1
Oda, M1
Lorz, U1
Glötzner, FL1
Murphy, JV1
Marquardt, KM1
Shug, AL1
Coraggio, F1
Angiulli, B1
Carbone, M1
Catalano, A1
Costa, MR1
Gargiulo, L1
Gentile, B1
Masetto, O1
Pomponio, N1
Scarpato, P1
Matsuda, I1
Ohtani, Y1
Ninomiya, N1
Klein, M1
Wendt, U1
Kuhara, T1
Inoue, Y1
Matsumoto, M1
Shinka, T1
Matsumoto, I1
Kitamura, K1
Fujii, H1
Sakura, N1
Izumi, T1
Utsumi, H1
Osawa, M1
Fukuyama, Y1

Clinical Trials (3)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
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

5 reviews available for valproic acid and Fatty Liver with Encephalopathy

ArticleYear
Advances in pediatric pharmacology and toxicology.
    Advances in pediatrics, 1983, Volume: 30

    Topics: Animals; Anti-Bacterial Agents; Aspirin; Attention Deficit Disorder with Hyperactivity; Biological A

1983
Hepatotoxicity to sodium valproate: a review.
    Gut, 1984, Volume: 25, Issue:6

    Topics: Adolescent; Adult; Ammonia; Animals; Chemical and Drug Induced Liver Injury; Child; Child, Preschool

1984
Inherited and acquired syndromes of hyperammonemia and encephalopathy in children.
    Seminars in liver disease, 1994, Volume: 14, Issue:3

    Topics: Ammonia; Child; Hepatic Encephalopathy; Humans; Liver Diseases; Metabolism, Inborn Errors; Reye Synd

1994
[Mitochondrial diseases due to drug toxicity].
    Nihon rinsho. Japanese journal of clinical medicine, 2002, Volume: 60 Suppl 4

    Topics: Animals; Antibiotics, Antineoplastic; Aspirin; Humans; Mitochondria; Mitochondrial Diseases; Reye Sy

2002
[Acute hepatic steatosis. Description of a clinical case of sodium valproate-induced acute hepatic steatosis].
    La Clinica terapeutica, 1988, Mar-31, Volume: 124, Issue:6

    Topics: Acute Disease; Adult; Biopsy; Child; Child, Preschool; Diagnosis, Differential; Fatty Liver; Fatty L

1988

Other Studies

18 other studies available for valproic acid and Fatty Liver with Encephalopathy

ArticleYear
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
    Lancet (London, England), 1984, Mar-24, Volume: 1, Issue:8378

    Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid

1984
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
    Lancet (London, England), 1984, Mar-24, Volume: 1, Issue:8378

    Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid

1984
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
    Lancet (London, England), 1984, Mar-24, Volume: 1, Issue:8378

    Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid

1984
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
    Lancet (London, England), 1984, Mar-24, Volume: 1, Issue:8378

    Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid

1984
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
    Lancet (London, England), 1984, Mar-24, Volume: 1, Issue:8378

    Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid

1984
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
    Lancet (London, England), 1984, Mar-24, Volume: 1, Issue:8378

    Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid

1984
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
    Lancet (London, England), 1984, Mar-24, Volume: 1, Issue:8378

    Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid

1984
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
    Lancet (London, England), 1984, Mar-24, Volume: 1, Issue:8378

    Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid

1984
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
    Lancet (London, England), 1984, Mar-24, Volume: 1, Issue:8378

    Topics: Carnitine; Child; Humans; Liver; Reye Syndrome; Valproic Acid

1984
Effect of increasing carbon chain length on organic acid transport by the choroid plexus: a potential factor in Reye's syndrome.
    Brain research, 1983, Jan-24, Volume: 259, Issue:2

    Topics: 2,4-Dichlorophenoxyacetic Acid; 3-Hydroxybutyric Acid; Acids; Animals; Blood-Brain Barrier; Caproate

1983
Reye-like syndrome associated with valproic acid.
    Brain & development, 1983, Volume: 5, Issue:3

    Topics: Adolescent; Ammonia; Biopsy; Female; Humans; Liver; Liver Function Tests; Reye Syndrome; Status Epil

1983
Treatment of epileptic patients with valproic acid does not modify plasma and urine short-chain-fatty acids.
    Acta neurologica Scandinavica, 1983, Volume: 68, Issue:4

    Topics: Adolescent; Adult; Ammonia; Brain Diseases; Butyrates; Epilepsy; Fatty Acids; Female; Hemiterpenes;

1983
Fatal Reye-like syndrome associated with valproic acid.
    Annals of neurology, 1980, Volume: 7, Issue:4

    Topics: Child; Humans; Male; Reye Syndrome; Valproic Acid

1980
Valproic acid producing a Reye-like syndrome.
    The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques, 1982, Volume: 9, Issue:4

    Topics: Aspartate Aminotransferases; Chemical and Drug Induced Liver Injury; Chemical Phenomena; Chemistry;

1982
Decreased serum carnitine in valproate induced Reye syndrome.
    European journal of pediatrics, 1982, Volume: 139, Issue:3

    Topics: Carnitine; Child, Preschool; Female; Humans; Reye Syndrome; Valproic Acid

1982
[Description of a case of a syndrome similar to Reye's syndrome in a male adult undergoing treatment with valproic acid and phenobarbital].
    Giornale di clinica medica, 1982, Volume: 63, Issue:9

    Topics: Adult; Brain Diseases; Coma; Epilepsy, Tonic-Clonic; Fever; Humans; Male; Muscle Rigidity; Phenobarb

1982
The mitochondrial permeability transition: a new pathophysiological mechanism for Reye's syndrome and toxic liver injury.
    The Journal of pharmacology and experimental therapeutics, 1996, Volume: 278, Issue:3

    Topics: Animals; Aspirin; Carboxylic Acids; Chemical and Drug Induced Liver Injury; Cyclosporine; Male; Memb

1996
Partial N-acetyl-glutamate synthetase deficiency masquerading as a valproic acid-induced Reye-like syndrome.
    Acta paediatrica (Oslo, Norway : 1992), 1999, Volume: 88, Issue:12

    Topics: Ammonia; Child, Preschool; Glutamate Synthase; Humans; Liver Function Tests; Male; Reye Syndrome; Va

1999
Reye-like syndrome associated with valproic acid therapy.
    The Journal of pediatrics, 1979, Volume: 95, Issue:1

    Topics: Chemical and Drug Induced Liver Injury; Child; Epilepsy; Female; Humans; Reye Syndrome; Valproic Aci

1979
[An adult case of Reye like syndrome and acute pancreatitis associated with sodium valproate].
    Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology, 1992, Volume: 89, Issue:7

    Topics: Acute Disease; Biopsy; Humans; Liver; Male; Middle Aged; Pancreatitis; Reye Syndrome; Valproic Acid

1992
[Reye syndrome treated with valproic acid in association with varicella infection].
    Der Nervenarzt, 1989, Volume: 60, Issue:5

    Topics: Adult; Aspirin; Carbamazepine; Chickenpox; Drug Therapy, Combination; Epilepsy; Humans; Male; Reye S

1989
Valproic acid associated abnormalities of carnitine metabolism.
    Lancet (London, England), 1985, Apr-06, Volume: 1, Issue:8432

    Topics: Carnitine; Child; Child, Preschool; Humans; Infant; Reye Syndrome; Valproic Acid

1985
Renal handling of carnitine in children with carnitine deficiency and hyperammonemia associated with valproate therapy.
    The Journal of pediatrics, 1986, Volume: 109, Issue:1

    Topics: Ammonia; Carnitine; Epilepsy; Humans; Infant; Intellectual Disability; Kidney; Metabolic Clearance R

1986
[Reye-like syndrome following valproate therapy in an adult].
    Psychiatrie, Neurologie, und medizinische Psychologie, 1988, Volume: 40, Issue:6

    Topics: Chemical and Drug Induced Liver Injury; Drug Therapy, Combination; Epilepsies, Partial; Humans; Male

1988
Altered metabolic profiles of valproic acid in a patient with Reye's syndrome.
    Clinica chimica acta; international journal of clinical chemistry, 1985, Jan-30, Volume: 145, Issue:2

    Topics: Biotransformation; Child; Chromatography, Gas; Female; Gas Chromatography-Mass Spectrometry; Glucuro

1985
[A clinico-biochemical study of acute encephalopathy in patients treated with Ca-hopantenate].
    No to hattatsu = Brain and development, 1985, Volume: 17, Issue:5

    Topics: Acute Disease; Adolescent; Adult; Age Factors; Blood Chemical Analysis; Brain Diseases; Child; Child

1985