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valproic acid and Deficiency Disease, Ornithine Carbamoyltransferase

valproic acid has been researched along with Deficiency Disease, Ornithine Carbamoyltransferase in 15 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
"In this report, we describe the diagnosis, investigation and management of a patient presenting with refractory status epilepticus secondary to a previously unrecognised urea cycle defect, ornithine transcarbamylase deficiency, causing a hyperammonaemic encephalopathy."8.02Status epilepticus secondary to hyperammonaemia: a late presentation of an undiagnosed urea cycle defect. ( Beddoes, P; Nerone, G; Tai, C, 2021)
"In this report, we describe the diagnosis, investigation and management of a patient presenting with refractory status epilepticus secondary to a previously unrecognised urea cycle defect, ornithine transcarbamylase deficiency, causing a hyperammonaemic encephalopathy."4.02Status epilepticus secondary to hyperammonaemia: a late presentation of an undiagnosed urea cycle defect. ( Beddoes, P; Nerone, G; Tai, C, 2021)
"The aims of this report are to 1) present a rare case of fatal cerebral edema associated with late-onset ornithine transcarbamylase (OTC) deficiency in a juvenile male patient receiving valproic acid and 2) review the neuropathologic changes associated with the hyperammonemia."3.73Fatal cerebral edema from late-onset ornithine transcarbamylase deficiency in a juvenile male patient receiving valproic acid. ( Fraser, DD; Ramsay, DA; Rupar, CA; Singh, R; Thakur, V, 2006)
"Specific treatment for hyperammonemia must be implemented promptly."1.48Valproate-induced hyperammonemia - uncovering an underlying inherited metabolic disorder: a case report. ( Lachmann, R; Mehta, S; Tayabali, S, 2018)
"Acute hyperammonemia is a medical emergency for which immediate steps must be taken to minimize permanent brain damage."1.32Unusual causes of hyperammonemia in the ED. ( Chen, WJ; Shih, FF; Weng, TI, 2004)
"Ornithine transcarbamylase deficiency is an X linked disorder and the most common inherited cause of hyperammonaemia."1.30Hyperammonaemic encephalopathy after initiation of valproate therapy in unrecognised ornithine transcarbamylase deficiency. ( Kohlschütter, A; Oechsner, M; Steen, C; Stürenburg, HJ, 1998)
"Women with heterozygous ornithine transcarbamylase deficiency may have no symptoms or have episodic, symptomatic hyperammonemia, which can be fatal."1.28Heterozygote ornithine transcarbamylase deficiency presenting as symptomatic hyperammonemia during initiation of valproate therapy. ( Callahan, K; Evans, B; Honeycutt, D; Rutledge, L, 1992)
" It is postulated that the idiosyncratic response in OTC-deficient mice may be caused by an interaction between a metabolic aberration of mitochondria and toxic metabolites of valproate."1.27Hepatotoxicity of sodium valproate in ornithine transcarbamylase-deficient mice. ( Letarte, J; Qureshi, IA; Qureshi, SR; Tuchweber, B; Yousef, I, 1985)

Research

Studies (15)

TimeframeStudies, this research(%)All Research%
pre-19905 (33.33)18.7374
1990's4 (26.67)18.2507
2000's3 (20.00)29.6817
2010's1 (6.67)24.3611
2020's2 (13.33)2.80

Authors

AuthorsStudies
Kazmierski, D1
Sharma, N1
O'Leary, K1
Ochieng, P1
Beddoes, P1
Nerone, G1
Tai, C1
Mehta, S1
Tayabali, S1
Lachmann, R1
Weng, TI1
Shih, FF1
Chen, WJ1
Thakur, V1
Rupar, CA1
Ramsay, DA1
Singh, R1
Fraser, DD1
Tripp, JH1
Hargreaves, T1
Anthony, PP1
Searle, JF1
Miller, P1
Leonard, JV1
Patrick, AD1
Oberholzer, VG1
Jaeken, J1
Corbeel, L1
Leão, M1
Oechsner, M1
Steen, C1
Stürenburg, HJ1
Kohlschütter, A1
Padilla, Mde L1
Miguélez, M1
Riverola, A1
Bueno, J1
Honeycutt, D1
Callahan, K1
Rutledge, L1
Evans, B1
Tokatli, A1
Coşkun, T1
Cataltepe, S1
Ozalp, I1
Kennedy, CR1
Cogswell, JJ1
Kay, JD1
Hilton-Jones, D1
Hyman, N1
Qureshi, IA1
Letarte, J1
Tuchweber, B1
Yousef, I1
Qureshi, SR1

Other Studies

15 other studies available for valproic acid and Deficiency Disease, Ornithine Carbamoyltransferase

ArticleYear
Valproate-induced fatal acute hyperammonaemia-related encephalopathy in late-onset ornithine transcarbamylase deficiency.
    BMJ case reports, 2021, May-25, Volume: 14, Issue:5

    Topics: Adult; Brain Diseases; Humans; Hyperammonemia; Male; Ornithine Carbamoyltransferase Deficiency Disea

2021
Status epilepticus secondary to hyperammonaemia: a late presentation of an undiagnosed urea cycle defect.
    BMJ case reports, 2021, May-31, Volume: 14, Issue:5

    Topics: Adult; Child; Humans; Hyperammonemia; Ornithine Carbamoyltransferase Deficiency Disease; Status Epil

2021
Valproate-induced hyperammonemia - uncovering an underlying inherited metabolic disorder: a case report.
    Journal of medical case reports, 2018, May-17, Volume: 12, Issue:1

    Topics: Anticonvulsants; Critical Illness; Dietary Proteins; Humans; Hyperammonemia; Male; Ornithine Carbamo

2018
Unusual causes of hyperammonemia in the ED.
    The American journal of emergency medicine, 2004, Volume: 22, Issue:2

    Topics: Adult; Aged; Anticonvulsants; Antimetabolites, Antineoplastic; Emergency Service, Hospital; Female;

2004
Fatal cerebral edema from late-onset ornithine transcarbamylase deficiency in a juvenile male patient receiving valproic acid.
    Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies, 2006, Volume: 7, Issue:3

    Topics: Adolescent; Brain Edema; Fatal Outcome; GABA Agents; Humans; Hyperammonemia; Male; Ornithine Carbamo

2006
Sodium valproate and ornithine carbamyl transferase deficiency.
    Lancet (London, England), 1981, May-23, Volume: 1, Issue:8230

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Humans; Infant; Liver; Male; Mitochondria, Liver; Orn

1981
Sodium valproate and the development of acute hepatic failure.
    Pediatric research, 1984, Volume: 18, Issue:4

    Topics: Chemical and Drug Induced Liver Injury; Humans; Infant; Male; Ornithine Carbamoyltransferase Deficie

1984
Valproate as a cause of hyperammonemia in heterozygotes with ornithine-transcarbamylase deficiency.
    Neurology, 1995, Volume: 45, Issue:3 Pt 1

    Topics: Ammonia; Child; Epilepsy, Tonic-Clonic; Female; Heterozygote; Humans; Ornithine Carbamoyltransferase

1995
Hyperammonaemic encephalopathy after initiation of valproate therapy in unrecognised ornithine transcarbamylase deficiency.
    Journal of neurology, neurosurgery, and psychiatry, 1998, Volume: 64, Issue:5

    Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Ammonia; Anticonvulsants; Brain Diseases, Metaboli

1998
[Hyperammonaemic coma after valproate therapy as adult onset of ornithine transcarbamylase deficiency].
    Medicina clinica, 2002, Feb-16, Volume: 118, Issue:5

    Topics: Coma; Female; Humans; Hyperammonemia; Middle Aged; Ornithine Carbamoyltransferase Deficiency Disease

2002
Heterozygote ornithine transcarbamylase deficiency presenting as symptomatic hyperammonemia during initiation of valproate therapy.
    Neurology, 1992, Volume: 42, Issue:3 Pt 1

    Topics: Adult; Ammonia; Female; Heterozygote; Humans; Metabolic Diseases; Ornithine Carbamoyltransferase Def

1992
Valproate-induced lethal hyperammonaemic coma in a carrier of ornithine carbamoyltransferase deficiency.
    Journal of inherited metabolic disease, 1991, Volume: 14, Issue:5

    Topics: Ammonia; Child, Preschool; Coma; Epilepsy; Female; Heterozygote; Humans; Ornithine Carbamoyltransfer

1991
Late onset ornithine carbamoyl transferase deficiency in males.
    Archives of disease in childhood, 1989, Volume: 64, Issue:4

    Topics: Child; Humans; Male; Ornithine Carbamoyltransferase Deficiency Disease; Photosensitivity Disorders;

1989
Valproate toxicity and ornithine carbamoyltransferase deficiency.
    Lancet (London, England), 1986, Nov-29, Volume: 2, Issue:8518

    Topics: Adult; Amino Acid Metabolism, Inborn Errors; Female; Humans; Ornithine Carbamoyltransferase Deficien

1986
Hepatotoxicity of sodium valproate in ornithine transcarbamylase-deficient mice.
    Toxicology letters, 1985, Volume: 25, Issue:3

    Topics: Animals; Dose-Response Relationship, Drug; Liver; Male; Mice; Mice, Mutant Strains; Ornithine Carbam

1985