valproic acid has been researched along with Ornithine Carbamoyltransferase Deficiency Disease 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.
Ornithine Carbamoyltransferase Deficiency Disease: An inherited urea cycle disorder associated with deficiency of the enzyme ORNITHINE CARBAMOYLTRANSFERASE, transmitted as an X-linked trait and featuring elevations of amino acids and ammonia in the serum. Clinical features, which are more prominent in males, include seizures, behavioral alterations, episodic vomiting, lethargy, and coma. (Menkes, Textbook of Child Neurology, 5th ed, pp49-50)
| Excerpt | Relevance | Reference |
|---|---|---|
| "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." | 8.02 | Status 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." | 4.02 | Status 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." | 3.73 | Fatal 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." | 1.48 | Valproate-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." | 1.32 | Unusual 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." | 1.30 | Hyperammonaemic 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." | 1.28 | Heterozygote 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." | 1.27 | Hepatotoxicity of sodium valproate in ornithine transcarbamylase-deficient mice. ( Letarte, J; Qureshi, IA; Qureshi, SR; Tuchweber, B; Yousef, I, 1985) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 5 (33.33) | 18.7374 |
| 1990's | 4 (26.67) | 18.2507 |
| 2000's | 3 (20.00) | 29.6817 |
| 2010's | 1 (6.67) | 24.3611 |
| 2020's | 2 (13.33) | 2.80 |
| Authors | Studies |
|---|---|
| Kazmierski, D | 1 |
| Sharma, N | 1 |
| O'Leary, K | 1 |
| Ochieng, P | 1 |
| Beddoes, P | 1 |
| Nerone, G | 1 |
| Tai, C | 1 |
| Mehta, S | 1 |
| Tayabali, S | 1 |
| Lachmann, R | 1 |
| Weng, TI | 1 |
| Shih, FF | 1 |
| Chen, WJ | 1 |
| Thakur, V | 1 |
| Rupar, CA | 1 |
| Ramsay, DA | 1 |
| Singh, R | 1 |
| Fraser, DD | 1 |
| Tripp, JH | 1 |
| Hargreaves, T | 1 |
| Anthony, PP | 1 |
| Searle, JF | 1 |
| Miller, P | 1 |
| Leonard, JV | 1 |
| Patrick, AD | 1 |
| Oberholzer, VG | 1 |
| Jaeken, J | 1 |
| Corbeel, L | 1 |
| Leão, M | 1 |
| Oechsner, M | 1 |
| Steen, C | 1 |
| Stürenburg, HJ | 1 |
| Kohlschütter, A | 1 |
| Padilla, Mde L | 1 |
| Miguélez, M | 1 |
| Riverola, A | 1 |
| Bueno, J | 1 |
| Honeycutt, D | 1 |
| Callahan, K | 1 |
| Rutledge, L | 1 |
| Evans, B | 1 |
| Tokatli, A | 1 |
| Coşkun, T | 1 |
| Cataltepe, S | 1 |
| Ozalp, I | 1 |
| Kennedy, CR | 1 |
| Cogswell, JJ | 1 |
| Kay, JD | 1 |
| Hilton-Jones, D | 1 |
| Hyman, N | 1 |
| Qureshi, IA | 1 |
| Letarte, J | 1 |
| Tuchweber, B | 1 |
| Yousef, I | 1 |
| Qureshi, SR | 1 |
15 other studies available for valproic acid and Ornithine Carbamoyltransferase Deficiency Disease
| Article | Year |
|---|---|
Valproate-induced fatal acute hyperammonaemia-related encephalopathy in late-onset ornithine transcarbamylase deficiency.
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.
Topics: Adult; Child; Humans; Hyperammonemia; Ornithine Carbamoyltransferase Deficiency Disease; Status Epil | 2021 |
Valproate-induced hyperammonemia - uncovering an underlying inherited metabolic disorder: a case report.
Topics: Anticonvulsants; Critical Illness; Dietary Proteins; Humans; Hyperammonemia; Male; Ornithine Carbamo | 2018 |
Unusual causes of hyperammonemia in the ED.
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.
Topics: Adolescent; Brain Edema; Fatal Outcome; GABA Agents; Humans; Hyperammonemia; Male; Ornithine Carbamo | 2006 |
Sodium valproate and ornithine carbamyl transferase deficiency.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Humans; Infant; Liver; Male; Mitochondria, Liver; Orn | 1981 |
Sodium valproate and the development of acute hepatic failure.
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.
Topics: Ammonia; Child; Epilepsy, Tonic-Clonic; Female; Heterozygote; Humans; Ornithine Carbamoyltransferase | 1995 |
Hyperammonaemic encephalopathy after initiation of valproate therapy in unrecognised ornithine transcarbamylase deficiency.
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].
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.
Topics: Adult; Ammonia; Female; Heterozygote; Humans; Metabolic Diseases; Ornithine Carbamoyltransferase Def | 1992 |
Valproate-induced lethal hyperammonaemic coma in a carrier of ornithine carbamoyltransferase deficiency.
Topics: Ammonia; Child, Preschool; Coma; Epilepsy; Female; Heterozygote; Humans; Ornithine Carbamoyltransfer | 1991 |
Late onset ornithine carbamoyl transferase deficiency in males.
Topics: Child; Humans; Male; Ornithine Carbamoyltransferase Deficiency Disease; Photosensitivity Disorders; | 1989 |
Valproate toxicity and ornithine carbamoyltransferase deficiency.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Female; Humans; Ornithine Carbamoyltransferase Deficien | 1986 |
Hepatotoxicity of sodium valproate in ornithine transcarbamylase-deficient mice.
Topics: Animals; Dose-Response Relationship, Drug; Liver; Male; Mice; Mice, Mutant Strains; Ornithine Carbam | 1985 |