valproic acid has been researched along with Metabolism, Inborn Errors in 12 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.
Metabolism, Inborn Errors: Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.
| Excerpt | Relevance | Reference |
|---|---|---|
| " Valproic acid appears to be use cautionally in obese females with epilepsy." | 5.24 | Evaluate the effects of long-term valproic acid treatment on metabolic profiles in newly diagnosed or untreated female epileptic patients: A prospective study. ( Sadhotra, A; Sidhu, HS; Srinivas, R, 2017) |
| " The pathogenesis of VPA hepatotoxicity is unclear but may relate to the accumulation of a toxic metabolite of VPA which impairs fatty-acid oxidation." | 1.28 | The high incidence of valproate hepatotoxicity in infants may relate to familial metabolic defects. ( Applegarth, DA; Appleton, RE; Davidson, AG; Dimmick, JE; Farrell, K; Wong, LT, 1990) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 3 (25.00) | 18.7374 |
| 1990's | 4 (33.33) | 18.2507 |
| 2000's | 3 (25.00) | 29.6817 |
| 2010's | 2 (16.67) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Sidhu, HS | 1 |
| Srinivas, R | 1 |
| Sadhotra, A | 1 |
| LaBuzetta, JN | 1 |
| Yao, JZ | 1 |
| Bourque, DL | 1 |
| Zivin, J | 1 |
| Shinka, T | 1 |
| Ohfu, M | 1 |
| Hirose, S | 1 |
| Kuhara, T | 1 |
| Meshitsuka, S | 1 |
| Koeda, T | 1 |
| Muro, H | 1 |
| Silva, MF | 1 |
| Aires, CC | 1 |
| Luis, PB | 1 |
| Ruiter, JP | 1 |
| IJlst, L | 1 |
| Duran, M | 1 |
| Wanders, RJ | 1 |
| Tavares de Almeida, I | 1 |
| Mortensen, PB | 1 |
| Sherratt, HS | 1 |
| Veitch, RK | 1 |
| Treem, WR | 1 |
| Tein, I | 1 |
| DiMauro, S | 1 |
| Xie, ZW | 1 |
| De Vivo, DC | 1 |
| Kelley, RI | 1 |
| Appleton, RE | 1 |
| Farrell, K | 1 |
| Applegarth, DA | 1 |
| Dimmick, JE | 1 |
| Wong, LT | 1 |
| Davidson, AG | 1 |
| Hjelm, M | 1 |
| de Silva, LV | 1 |
| Seakins, JW | 1 |
| Oberholzer, VG | 1 |
| Rolles, CJ | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| 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 valproic acid and Metabolism, Inborn Errors
| Article | Year |
|---|---|
Adult nonhepatic hyperammonemia: a case report and differential diagnosis.
Topics: Ammonia; Diagnosis, Differential; Humans; Hyperammonemia; Liver Diseases; Male; Metabolism, Inborn E | 2010 |
Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation: a review.
Topics: Animals; Anticonvulsants; Biotransformation; Chemical and Drug Induced Liver Injury; Fatty Acids; Fa | 2008 |
Inherited and acquired syndromes of hyperammonemia and encephalopathy in children.
Topics: Ammonia; Child; Hepatic Encephalopathy; Humans; Liver Diseases; Metabolism, Inborn Errors; Reye Synd | 1994 |
2 trials available for valproic acid and Metabolism, Inborn Errors
| Article | Year |
|---|---|
Evaluate the effects of long-term valproic acid treatment on metabolic profiles in newly diagnosed or untreated female epileptic patients: A prospective study.
Topics: Adiponectin; Adolescent; Adult; Anticonvulsants; Blood Glucose; Body Mass Index; Cholesterol; Choles | 2017 |
The role of carnitine supplementation in valproic acid therapy.
Topics: Carnitine; Child; Double-Blind Method; Fatty Acid Desaturases; Humans; Metabolism, Inborn Errors; Va | 1994 |
7 other studies available for valproic acid and Metabolism, Inborn Errors
| Article | Year |
|---|---|
Effect of valproic acid on the urinary metabolic profile of a patient with succinic semialdehyde dehydrogenase deficiency.
Topics: Aldehyde Oxidoreductases; Anticonvulsants; Gas Chromatography-Mass Spectrometry; Humans; Hydroxybuty | 2003 |
Direct observation of 3-keto-valproate in urine by 2D-NMR spectroscopy.
Topics: Adolescent; Anticonvulsants; Consanguinity; Epilepsy; Humans; Indicators and Reagents; Ketones; Magn | 2003 |
Inhibition of fatty acid oxidation by valproate.
Topics: Fatty Acids; Humans; Metabolism, Inborn Errors; Oxidation-Reduction; Valproic Acid | 1980 |
Animal models for dicarboxylic aciduria.
Topics: Animals; Dicarboxylic Acids; Disease Models, Animal; Epoxy Compounds; Fatty Acids, Monounsaturated; | 1984 |
Valproic acid impairs carnitine uptake in cultured human skin fibroblasts. An in vitro model for the pathogenesis of valproic acid-associated carnitine deficiency.
Topics: Biological Transport; Carnitine; Cells, Cultured; Child; Dose-Response Relationship, Drug; Fibroblas | 1993 |
Valproic acid impairs carnitine uptake in cultured human skin fibroblasts. An in vitro model for the pathogenesis of valproic acid-associated carnitine deficiency.
Topics: Biological Transport; Carnitine; Cells, Cultured; Child; Dose-Response Relationship, Drug; Fibroblas | 1993 |
Valproic acid impairs carnitine uptake in cultured human skin fibroblasts. An in vitro model for the pathogenesis of valproic acid-associated carnitine deficiency.
Topics: Biological Transport; Carnitine; Cells, Cultured; Child; Dose-Response Relationship, Drug; Fibroblas | 1993 |
Valproic acid impairs carnitine uptake in cultured human skin fibroblasts. An in vitro model for the pathogenesis of valproic acid-associated carnitine deficiency.
Topics: Biological Transport; Carnitine; Cells, Cultured; Child; Dose-Response Relationship, Drug; Fibroblas | 1993 |
Valproic acid impairs carnitine uptake in cultured human skin fibroblasts. An in vitro model for the pathogenesis of valproic acid-associated carnitine deficiency.
Topics: Biological Transport; Carnitine; Cells, Cultured; Child; Dose-Response Relationship, Drug; Fibroblas | 1993 |
Valproic acid impairs carnitine uptake in cultured human skin fibroblasts. An in vitro model for the pathogenesis of valproic acid-associated carnitine deficiency.
Topics: Biological Transport; Carnitine; Cells, Cultured; Child; Dose-Response Relationship, Drug; Fibroblas | 1993 |
Valproic acid impairs carnitine uptake in cultured human skin fibroblasts. An in vitro model for the pathogenesis of valproic acid-associated carnitine deficiency.
Topics: Biological Transport; Carnitine; Cells, Cultured; Child; Dose-Response Relationship, Drug; Fibroblas | 1993 |
Valproic acid impairs carnitine uptake in cultured human skin fibroblasts. An in vitro model for the pathogenesis of valproic acid-associated carnitine deficiency.
Topics: Biological Transport; Carnitine; Cells, Cultured; Child; Dose-Response Relationship, Drug; Fibroblas | 1993 |
Valproic acid impairs carnitine uptake in cultured human skin fibroblasts. An in vitro model for the pathogenesis of valproic acid-associated carnitine deficiency.
Topics: Biological Transport; Carnitine; Cells, Cultured; Child; Dose-Response Relationship, Drug; Fibroblas | 1993 |
The high incidence of valproate hepatotoxicity in infants may relate to familial metabolic defects.
Topics: Chemical and Drug Induced Liver Injury; Humans; Infant; Liver Diseases; Male; Metabolism, Inborn Err | 1990 |
Evidence of inherited urea cycle defect in a case of fatal valproate toxicity.
Topics: Adult; Ammonia; Child; Child, Preschool; Female; Humans; Male; Metabolism, Inborn Errors; Middle Age | 1986 |