benzoic acid has been researched along with Ornithine Carbamoyltransferase Deficiency Disease in 16 studies
Benzoic Acid: A fungistatic compound that is widely used as a food preservative. It is conjugated to GLYCINE in the liver and excreted as hippuric acid.
benzoic acid : A compound comprising a benzene ring core carrying a carboxylic acid substituent.
aromatic carboxylic acid : Any carboxylic acid in which the carboxy group is directly bonded to an aromatic ring.
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 |
|---|---|---|
| "Children with inborn errors of urea synthesis accumulate ammonium and other nitrogenous precursors of urea, leading to episodic coma and a high mortality rate." | 3.66 | Treatment of inborn errors of urea synthesis: activation of alternative pathways of waste nitrogen synthesis and excretion. ( Batshaw, ML; Blom, W; Brubakk, AM; Brusilow, S; Burton, BK; Cann, HM; Kerr, D; Mamunes, P; Matalon, R; Myerberg, D; Schafer, IA; Waber, L, 1982) |
| "Girls with symptomatic ornithine transcarbamylase deficiency who are treated with drugs that activate new pathways of waste-nitrogen excretion have fewer hyperammonemic episodes and a reduced risk of further cognitive decline." | 2.68 | Long-term treatment of girls with ornithine transcarbamylase deficiency. ( Bassett, SS; Brusilow, SW; Clissold, DB; Maestri, NE, 1996) |
| " Further prospective studies should be performed to define the optimal dosage of sodium phenylbutyrate and the requirements for protein diet at different ages." | 1.31 | Long-term treatment with sodium phenylbutyrate in ornithine transcarbamylase-deficient patients. ( Burlina, AB; Korall, H; Ogier, H; Trefz, FK, 2001) |
| "B6 status in a patient with OTC deficiency during the therapy with benzoate." | 1.27 | Activity of the glycine cleavage system in hyperammonemia treated with benzoate. ( Fujiwara, K; Kamoshita, S; Kodama, H; Motokawa, Y; Nose, O; Tajiri, H, 1983) |
| " In the child, when the benzoate/phenylacetate dosage was increased from 200 to 375 mg/kg/day each, feeding decreased." | 1.27 | Effect of sodium benzoate and sodium phenylacetate on brain serotonin turnover in the ornithine transcarbamylase-deficient sparse-fur mouse. ( Batshaw, ML; Coyle, JT; Hyman, SL; Mellits, ED; Quaskey, S; Qureshi, IA; Robinson, MB, 1988) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 12 (75.00) | 18.7374 |
| 1990's | 3 (18.75) | 18.2507 |
| 2000's | 1 (6.25) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Brusilow, SW | 2 |
| Danney, M | 1 |
| Waber, LJ | 1 |
| Batshaw, M | 1 |
| Burton, B | 1 |
| Levitsky, L | 1 |
| Roth, K | 1 |
| McKeethren, C | 1 |
| Ward, J | 1 |
| Guibaud, P | 1 |
| Baxter, P | 1 |
| Bourgeois, J | 1 |
| Louis, JJ | 1 |
| Bureau, J | 1 |
| Kodama, H | 1 |
| Fujiwara, K | 1 |
| Motokawa, Y | 1 |
| Tajiri, H | 1 |
| Nose, O | 1 |
| Kamoshita, S | 1 |
| Michels, VV | 1 |
| Beaudet, AL | 1 |
| Takeda, E | 1 |
| Kuroda, Y | 1 |
| Toshima, K | 1 |
| Watanabe, T | 1 |
| Naito, E | 1 |
| Miyao, M | 1 |
| Batshaw, ML | 2 |
| Brusilow, S | 1 |
| Waber, L | 1 |
| Blom, W | 2 |
| Brubakk, AM | 2 |
| Burton, BK | 1 |
| Cann, HM | 1 |
| Kerr, D | 1 |
| Mamunes, P | 1 |
| Matalon, R | 1 |
| Myerberg, D | 1 |
| Schafer, IA | 1 |
| Teijema, LL | 1 |
| Berger, R | 1 |
| Qureshi, IA | 6 |
| Letarte, J | 4 |
| Ouellet, R | 3 |
| Ratnakumari, L | 1 |
| Butterworth, RF | 1 |
| Michalak, A | 1 |
| Maestri, NE | 1 |
| Clissold, DB | 1 |
| Bassett, SS | 1 |
| Burlina, AB | 1 |
| Ogier, H | 1 |
| Korall, H | 1 |
| Trefz, FK | 1 |
| Lebel, S | 1 |
| Qureshi, I | 1 |
| Rouleau, T | 1 |
| Hyman, SL | 1 |
| Coyle, JT | 1 |
| Robinson, MB | 1 |
| Mellits, ED | 1 |
| Quaskey, S | 1 |
| Godard, M | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| The NIH UNI Study: Urea Cycle Disorders, Nutrition and Immunity[NCT01421888] | 4 participants (Actual) | Observational | 2011-08-08 | Terminated | |||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
1 trial available for benzoic acid and Ornithine Carbamoyltransferase Deficiency Disease
| Article | Year |
|---|---|
Long-term treatment of girls with ornithine transcarbamylase deficiency.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Brain Diseases; | 1996 |
15 other studies available for benzoic acid and Ornithine Carbamoyltransferase Deficiency Disease
| Article | Year |
|---|---|
Treatment of episodic hyperammonemia in children with inborn errors of urea synthesis.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Argininosuccinate Synthase; Benzoates; Benz | 1984 |
Severe ornithine transcarbamylase deficiency. Two and a half years' survival with normal development.
Topics: Amino Acids; Ammonia; Benzoates; Benzoic Acid; Drug Therapy, Combination; Enteral Nutrition; Humans; | 1984 |
Activity of the glycine cleavage system in hyperammonemia treated with benzoate.
Topics: Ammonia; Animals; Benzoates; Benzoic Acid; Female; Folic Acid; Glycine; Humans; Liver; Male; Mice; M | 1983 |
Treatment of OTC deficiency.
Topics: Benzoates; Benzoic Acid; Child; Humans; Keto Acids; Male; Ornithine Carbamoyltransferase Deficiency | 1983 |
Effect of long-term administration of sodium benzoate to a patient with partial ornithine carbamoyl transferase deficiency.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Child; Female; Humans; Male; | 1983 |
Treatment of inborn errors of urea synthesis: activation of alternative pathways of waste nitrogen synthesis and excretion.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Essential; Ammonia; Arginine; Argininosuccinate S | 1982 |
Successful treatment of severe OTC deficiency.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Benzoates; Benzoic Acid; Citrulline; Humans | 1982 |
Spontaneous animal models of ornithine transcarbamylase deficiency: studies on serum and urinary nitrogenous metabolites.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Benzoates; Benzoic Acid; Diet; Female; Gluta | 1982 |
Effect of sodium benzoate on cerebral and hepatic energy metabolites in spf mice with congenital hyperammonemia.
Topics: Acetyl Coenzyme A; Adenosine Triphosphate; Ammonia; Animals; Benzoates; Benzoic Acid; Brain; Coenzym | 1993 |
Free and esterified coenzyme A in the liver and muscles of chronically hyperammonemic mice treated with sodium benzoate.
Topics: Acetyl Coenzyme A; Ammonia; Animals; Benzoates; Benzoic Acid; Chronic Disease; Coenzyme A; Disease M | 1995 |
Long-term treatment with sodium phenylbutyrate in ornithine transcarbamylase-deficient patients.
Topics: Adolescent; Ammonia; Benzoates; Benzoic Acid; Child; Child, Preschool; DNA Mutational Analysis; Drug | 2001 |
Development and inducibility of the hepatic and renal hippurate-synthesizing system in sparse-fur (spf) mutant mice with ornithine transcarbamylase deficiency.
Topics: Acyltransferases; Animals; Benzoates; Benzoic Acid; Enzyme Induction; Hippurates; Kidney Cortex; Liv | 1989 |
Significance of transported glycine in the conjugation of sodium benzoate in spf mutant mice with ornithine transcarbamylase deficiency.
Topics: Animals; Benzoates; Benzoic Acid; Carbon Radioisotopes; Glycine; Kinetics; Liver; Mice; Mice, Mutant | 1986 |
Effect of sodium benzoate and sodium phenylacetate on brain serotonin turnover in the ornithine transcarbamylase-deficient sparse-fur mouse.
Topics: Administration, Oral; Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Benzoates; Benzoic Aci | 1988 |
Chronic benzoate therapy in a boy with partial ornithine transcarbamylase deficiency.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Child; Humans; Male; Ornithi | 1985 |