benzoic acid has been researched along with Amino Acid Metabolism Disorders, Inborn in 40 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.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Patients with nonketotic hyperglycinemia generally have intractable seizures that are poorly responsive to anticonvulsant medication." | 7.67 | The effectiveness of benzoate in the management of seizures in nonketotic hyperglycinemia. ( Kulovich, S; Nyhan, WL; Qiao, CN; Wolff, JA; Yu, AL, 1986) |
| "These outcomes suggest that benzoate and dextromethorphan are not uniformly effective in nonketotic hyperglycinemia, but for some patients they improve arousal, decrease or eliminate seizures, and allow for some developmental progress." | 3.70 | Long-term use of high-dose benzoate and dextromethorphan for the treatment of nonketotic hyperglycinemia. ( Bellus, GA; Hamosh, A; Johnston, MV; Maher, JF; Rasmussen, SA, 1998) |
| " L-Carnitine was used at doses of 2, 4, 8, or 16 mmol/kg body weight (BW), and levels of ammonia, glutamine, glutamate, and some intermediates of energy metabolism were measured in brain and liver of spf/Y mice." | 3.68 | Effect of L-carnitine on cerebral and hepatic energy metabolites in congenitally hyperammonemic sparse-fur mice and its role during benzoate therapy. ( Butterworth, RF; Qureshi, IA; Ratnakumari, L, 1993) |
| "Patients with nonketotic hyperglycinemia generally have intractable seizures that are poorly responsive to anticonvulsant medication." | 3.67 | The effectiveness of benzoate in the management of seizures in nonketotic hyperglycinemia. ( Kulovich, S; Nyhan, WL; Qiao, CN; Wolff, JA; Yu, AL, 1986) |
| "The concentrations of guanidino compounds were determined in urine, plasma and cerebrospinal fluid of two patients with hyperargininemia during dietary therapy." | 3.67 | Guanidino compounds in plasma, urine and cerebrospinal fluid of hyperargininemic patients during therapy. ( De Deyn, P; Letarte, J; Lowenthal, A; Marescau, B; Qureshi, IA; Ryba, R, 1985) |
| "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) |
| "When patients present in hyperammonemic coma, the urea cycle disorders should be considered, especially if no obvious cause is identified." | 2.38 | Late clinical presentation of partial carbamyl phosphate synthetase I deficiency. ( Klinger, RJ; Lo, WD; Sloan, HR; Sotos, JF, 1993) |
| "Choline treatment did not change brain choline content, and was not associated with clinical or radiological improvement." | 1.30 | One-methyl group metabolism in non-ketotic hyperglycinaemia: mildly elevated cerebrospinal fluid homocysteine levels. ( Bottiglieri, T; Charles, HC; Gray, L; Hyland, K; Jaeken, J; Kahler, SG; Lazeyras, F; Van Hove, JL; Zeisel, SH, 1998) |
| " 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) |
| "Carnitine status was evaluated in 8 patients with partial ornithine transcarbamylase (OTC) deficiency and 19 patients with secondary carnitine deficiency, who were used as positive references." | 1.27 | Hyperammonemia related to carnitine metabolism with particular emphasis on ornithine transcarbamylase deficiency. ( Matsuda, I; Ohtani, Y; Ohyanagi, K; Yamamoto, S, 1987) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 21 (52.50) | 18.7374 |
| 1990's | 18 (45.00) | 18.2507 |
| 2000's | 1 (2.50) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Pueschel, SM | 1 |
| Cha, CJ | 1 |
| Langon, T | 1 |
| Krieger, I | 1 |
| Nigro, M | 1 |
| Brusilow, SW | 3 |
| Danney, M | 1 |
| Waber, LJ | 1 |
| Batshaw, M | 1 |
| Burton, B | 1 |
| Levitsky, L | 1 |
| Roth, K | 1 |
| McKeethren, C | 1 |
| Ward, J | 1 |
| Van de Bor, M | 1 |
| Mooy, P | 1 |
| van Zoeren, D | 1 |
| Berger, R | 2 |
| van Gelderen, HH | 1 |
| Teijema, HL | 1 |
| Berghuis, M | 1 |
| Cats, BP | 1 |
| de Klerk, JB | 1 |
| Duran, M | 1 |
| Mizutani, N | 1 |
| Maehara, M | 1 |
| Hayakawa, C | 1 |
| Kato, T | 1 |
| Watanabe, K | 1 |
| Suzuki, S | 1 |
| Qureshi, IA | 7 |
| Letarte, J | 4 |
| Ouellet, R | 3 |
| Batshaw, ML | 4 |
| Brusilow, S | 2 |
| Takeda, E | 1 |
| Kuroda, Y | 1 |
| Toshima, K | 1 |
| Watanabe, T | 1 |
| Naito, E | 1 |
| Miyao, M | 1 |
| Kodama, H | 1 |
| Kamoshita, S | 1 |
| Motokawa, Y | 1 |
| Waber, L | 1 |
| Blom, W | 2 |
| Brubakk, AM | 2 |
| Burton, BK | 1 |
| Cann, HM | 1 |
| Kerr, D | 1 |
| Mamunes, P | 1 |
| Matalon, R | 2 |
| Myerberg, D | 1 |
| Schafer, IA | 1 |
| Teijema, LL | 1 |
| Imai, M | 1 |
| Kitajima, H | 1 |
| Tokoro, T | 1 |
| Kubo, M | 1 |
| Maekawa, K | 1 |
| Walter, JH | 1 |
| Wraith, JE | 1 |
| Cleary, MA | 1 |
| Zammarchi, E | 2 |
| Donati, MA | 2 |
| Ciani, F | 2 |
| Pasquini, E | 2 |
| Pela, I | 1 |
| Fiorini, P | 1 |
| Ratnakumari, L | 1 |
| Butterworth, RF | 1 |
| Melnyk, AR | 1 |
| Henry, BW | 1 |
| Zeller, WP | 1 |
| Lange, C | 1 |
| Lo, WD | 1 |
| Sloan, HR | 1 |
| Sotos, JF | 1 |
| Klinger, RJ | 1 |
| Van Hove, JL | 2 |
| Kishnani, P | 1 |
| Muenzer, J | 1 |
| Wenstrup, RJ | 1 |
| Summar, ML | 2 |
| Brummond, MR | 1 |
| Lachiewicz, AM | 1 |
| Millington, DS | 2 |
| Kahler, SG | 2 |
| Maestri, NE | 1 |
| Clissold, DB | 1 |
| Bassett, SS | 1 |
| Feoli-Fonseca, JC | 1 |
| Lambert, M | 1 |
| Mitchell, G | 1 |
| Melançon, SB | 1 |
| Dallaire, L | 1 |
| Boneh, A | 1 |
| Degani, Y | 1 |
| Harari, M | 1 |
| Buonocore, G | 1 |
| Shih, VE | 1 |
| Bonocore, G | 1 |
| Hamosh, A | 1 |
| Maher, JF | 1 |
| Bellus, GA | 1 |
| Rasmussen, SA | 1 |
| Johnston, MV | 1 |
| Feillet, F | 1 |
| Leonard, JV | 1 |
| Patejunas, G | 1 |
| Lee, B | 1 |
| Dennis, JA | 1 |
| Healy, PJ | 1 |
| Reeds, PJ | 1 |
| Yu, H | 1 |
| Frazer, M | 1 |
| Mull, B | 1 |
| Warman, AW | 1 |
| Beaudet, AL | 1 |
| O'Brien, WE | 1 |
| Lazeyras, F | 1 |
| Zeisel, SH | 1 |
| Bottiglieri, T | 1 |
| Hyland, K | 1 |
| Charles, HC | 1 |
| Gray, L | 1 |
| Jaeken, J | 1 |
| Yu, X | 1 |
| Thompson, MM | 1 |
| Shi, D | 1 |
| Tuchman, M | 2 |
| Mauer, SM | 1 |
| Holzknecht, RA | 1 |
| Vnencak-Jones, CL | 1 |
| Wada, S | 1 |
| Matsuo, K | 1 |
| Nozawa, K | 1 |
| Yazaki, K | 1 |
| Suzuki, Y | 1 |
| Nishizawa, K | 1 |
| Sakai, H | 1 |
| Huruta, S | 1 |
| Sakuma, T | 1 |
| Barshop, BA | 1 |
| Breuer, J | 1 |
| Holm, J | 1 |
| Leslie, J | 1 |
| Nyhan, WL | 2 |
| Simell, O | 1 |
| Sipilä, I | 1 |
| Rajantie, J | 1 |
| Valle, DL | 1 |
| Matsuo, M | 1 |
| Saiki, K | 1 |
| Tanabe, J | 1 |
| Nakamura, H | 1 |
| Matsuo, T | 1 |
| Monahan, PS | 1 |
| Hyman, SL | 1 |
| Coyle, JT | 1 |
| Robinson, MB | 1 |
| Mellits, ED | 1 |
| Quaskey, S | 1 |
| Matsuda, I | 1 |
| Ohtani, Y | 1 |
| Ohyanagi, K | 1 |
| Yamamoto, S | 1 |
| Wolff, JA | 1 |
| Kulovich, S | 1 |
| Yu, AL | 1 |
| Qiao, CN | 1 |
| Marescau, B | 1 |
| De Deyn, P | 1 |
| Ryba, R | 1 |
| Lowenthal, A | 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] | |||||||
3 reviews available for benzoic acid and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
Late clinical presentation of partial carbamyl phosphate synthetase I deficiency.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Biopsy; Body Hei | 1993 |
Alternative pathway therapy for urea cycle disorders.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Arginine; Benzoates; Benzoic Acid; Child; Citrulline; | 1998 |
Treatment of urea cycle disorders.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Benzoates; Benzoic Acid; Dietary Proteins; H | 1987 |
2 trials available for benzoic acid and Amino Acid Metabolism Disorders, Inborn
| 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 |
Chronic sodium benzoate therapy in children with inborn errors of urea synthesis: effect on carnitine metabolism and ammonia nitrogen removal.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carnitine | 1996 |
35 other studies available for benzoic acid and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
Therapeutic attempts in infants with nonketotic hyperglycinaemia.
Topics: Adrenocorticotropic Hormone; Amino Acid Metabolism, Inborn Errors; Benzoates; Benzoic Acid; Clonazep | 1981 |
Evidence of defective threonine metabolism in non-ketotic hyperglycinaemia.
Topics: Amino Acid Metabolism, Inborn Errors; Benzoates; Benzoic Acid; Glycine; Humans; Infant; Infant, Newb | 1983 |
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 |
Successful treatment of severe carbamyl phosphate synthetase I deficiency.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carbamoyl-Phosphate Synthase | 1984 |
[A patient with neonatal citrullinemia].
Topics: Amino Acid Metabolism, Inborn Errors; Arginine; Benzoates; Benzoic Acid; Citrulline; Exchange Transf | 1983 |
Hyperargininemia: clinical course and treatment with sodium benzoate and phenylacetic acid.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Arginine; Benzoates; Benzoic Acid; Child | 1983 |
Treatment of hyperargininemia with sodium benzoate and arginine-restricted diet.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Benzoates; Benzoic Acid; Combin | 1984 |
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 hyperammonemia with sodium benzoate.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Child; Female; Humans | 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 |
[Therapeutic trial of NMDA antagonist for a nonketotic hyperglycinemic infant].
Topics: Amino Acid Metabolism, Inborn Errors; Benzoates; Benzoic Acid; Dextromethorphan; Glycine; Humans; In | 1995 |
Absence of acidosis in the initial presentation of propionic acidaemia.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carnitine; Food Pr | 1995 |
Failure of early dextromethorphan and sodium benzoate therapy in an infant with nonketotic hyperglycinemia.
Topics: Amino Acid Metabolism, Inborn Errors; Benzoates; Benzoic Acid; Dextromethorphan; Drug Therapy, Combi | 1994 |
Effect of L-carnitine on cerebral and hepatic energy metabolites in congenitally hyperammonemic sparse-fur mice and its role during benzoate therapy.
Topics: Adenosine Triphosphate; Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Benzoates; Benzoic A | 1993 |
Prospective management of a child with neonatal citrullinemia.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Argininosuccinate Synthase; Benzoates; Benz | 1993 |
Benzoate therapy and carnitine deficiency in non-ketotic hyperglycinemia.
Topics: Amino Acid Metabolism, Inborn Errors; Benzoates; Benzoic Acid; Carnitine; Drug Interactions; Female; | 1995 |
Prognostic clues and outcome of early treatment of nonketotic hyperglycinemia.
Topics: Amino Acid Metabolism, Inborn Errors; Benzoates; Benzoic Acid; Brain Diseases, Metabolic; Child, Pre | 1996 |
Neonatal onset of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome with favorable outcome.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carnitine; Citrulline; Diet, | 1997 |
Long-term use of high-dose benzoate and dextromethorphan for the treatment of nonketotic hyperglycinemia.
Topics: Amino Acid Metabolism, Inborn Errors; Benzoates; Benzoic Acid; Child; Child, Preschool; Dextromethor | 1998 |
Evaluation of gene therapy for citrullinaemia using murine and bovine models.
Topics: Adenoviruses, Human; Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Arginine; Argininosucci | 1998 |
One-methyl group metabolism in non-ketotic hyperglycinaemia: mildly elevated cerebrospinal fluid homocysteine levels.
Topics: Amino Acid Metabolism, Inborn Errors; Benzoic Acid; Brain Diseases; Choline; Coma; Female; Glycine; | 1998 |
Quantification of benzoic, phenylacetic, and phenylbutyric acids from filter-paper blood spots by gas chromatography--mass spectrometry with stable isotope dilution.
Topics: Amino Acid Metabolism, Inborn Errors; Benzoic Acid; Blood Specimen Collection; Deuterium; Gas Chroma | 2001 |
Prospective versus clinical diagnosis and therapy of acute neonatal hyperammonaemia in two sisters with carbamyl phosphate synthetase deficiency.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carbamoyl-Phosphate Synthase | 1992 |
[A case of citrullinemia with fair response to oral administration of sodium benzoate for consciousness disturbance].
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Argininosuccinate Synthase; Benzoates; Benzoic Acid; Ci | 1991 |
Alteration of urinary carnitine profile induced by benzoate administration.
Topics: Acetylcarnitine; Administration, Oral; Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Ben | 1991 |
Excretion of hippuric acid during sodium benzoate therapy in patients with hyperglycinaemia or hyperammonaemia.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Child, Preschool; Female; Gl | 1989 |
Waste nitrogen excretion via amino acid acylation: benzoate and phenylacetate in lysinuric protein intolerance.
Topics: Acylation; Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Benzoates; Benzoic A | 1986 |
Citrullinaemia: an infantile form with p-hydroxyphenylpyruvic and p-hydroxyphenyllactic acidurias.
Topics: Amino Acid Metabolism, Inborn Errors; Benzoates; Benzoic Acid; Citrulline; Female; Humans; Infant; P | 1987 |
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 |
Hyperammonemia related to carnitine metabolism with particular emphasis on ornithine transcarbamylase deficiency.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carnitine; Child; Dietary Pr | 1987 |
The effectiveness of benzoate in the management of seizures in nonketotic hyperglycinemia.
Topics: Amino Acid Metabolism, Inborn Errors; Benzoates; Benzoic Acid; Female; Glycine; Humans; Infant, Newb | 1986 |
Guanidino compounds in plasma, urine and cerebrospinal fluid of hyperargininemic patients during therapy.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Anorexia Nervosa; Arginine; Benzoates; Benzoic Aci | 1985 |
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 |