phenylacetic acid has been researched along with Amino Acid Metabolism Disorders, Inborn in 20 studies
phenylacetic acid : A monocarboxylic acid that is toluene in which one of the hydrogens of the methyl group has been replaced by a carboxy group.
| Excerpt | Relevance | Reference |
|---|---|---|
| "We report the results of a 25-year, open-label, uncontrolled study of sodium phenylacetate and sodium benzoate therapy (Ammonul, Ucyclyd Pharma) in 299 patients with urea-cycle disorders in whom there were 1181 episodes of acute hyperammonemia." | 5.12 | Survival after treatment with phenylacetate and benzoate for urea-cycle disorders. ( Berry, GT; Berry, SA; Brusilow, SW; Enns, GM; Hamosh, A; Rhead, WJ, 2007) |
| "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) |
| " This involves the long-term use of oral sodium phenylbutyrate, arginine supplements, or both, depending on the specific enzyme deficiency, and treatment of acute hyperammonemic crises with intravenous sodium benzoate/sodium phenylacetate plus arginine." | 2.41 | Alternative pathway therapy for urea cycle disorders: twenty years later. ( Batshaw, ML; MacArthur, RB; Tuchman, M, 2001) |
| "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) |
| " 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 | 7 (35.00) | 18.7374 |
| 1990's | 6 (30.00) | 18.2507 |
| 2000's | 6 (30.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 1 (5.00) | 2.80 |
| Authors | Studies |
|---|---|
| Armstrong, AJ | 1 |
| Henke, BR | 1 |
| Collado, MS | 1 |
| Taylor, JM | 1 |
| Pourtaheri, TD | 1 |
| Dillberger, JE | 1 |
| Roper, TD | 1 |
| Wamhoff, BR | 1 |
| Olson, MW | 1 |
| Figler, RA | 1 |
| Hoang, SA | 1 |
| Reardon, JE | 1 |
| Johns, BA | 1 |
| Scaglia, F | 1 |
| Carter, S | 1 |
| O'Brien, WE | 1 |
| Lee, B | 1 |
| Enns, GM | 1 |
| Berry, SA | 1 |
| Berry, GT | 1 |
| Rhead, WJ | 1 |
| Brusilow, SW | 4 |
| Hamosh, A | 1 |
| Shih, VE | 1 |
| 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 | 1 |
| van Gelderen, HH | 1 |
| Teijema, HL | 1 |
| Mizutani, N | 1 |
| Maehara, M | 1 |
| Hayakawa, C | 1 |
| Kato, T | 1 |
| Watanabe, K | 1 |
| Suzuki, S | 1 |
| Melnyk, AR | 1 |
| Matalon, R | 1 |
| Henry, BW | 1 |
| Zeller, WP | 1 |
| Lange, C | 1 |
| Lo, WD | 1 |
| Sloan, HR | 1 |
| Sotos, JF | 1 |
| Klinger, RJ | 1 |
| Maestri, NE | 1 |
| Clissold, DB | 1 |
| Bassett, SS | 1 |
| Feillet, F | 1 |
| Leonard, JV | 1 |
| Thoene, JG | 1 |
| Summar, M | 1 |
| Batshaw, ML | 3 |
| MacArthur, RB | 1 |
| Tuchman, M | 3 |
| Yu, X | 1 |
| Thompson, MM | 1 |
| Shi, D | 1 |
| Mauer, SM | 1 |
| Holzknecht, RA | 1 |
| Summar, ML | 1 |
| Vnencak-Jones, CL | 1 |
| Simell, O | 1 |
| Sipilä, I | 1 |
| Rajantie, J | 1 |
| Valle, DL | 1 |
| Monahan, PS | 1 |
| Hyman, SL | 1 |
| Coyle, JT | 1 |
| Robinson, MB | 1 |
| Qureshi, IA | 1 |
| Mellits, ED | 1 |
| Quaskey, S | 1 |
| Petrowski, S | 1 |
| Nyhan, WL | 1 |
| Reznik, V | 1 |
| Sweetman, L | 1 |
| Kulovich, S | 1 |
| Wolff, J | 1 |
| Jones, G | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| A Phase 2, Open-Label, Switch-Over, Dose-Escalation Study of the Safety and Tolerability of HPN-100 Compared to Buphenyl® (Sodium Phenylbutyrate) in Patients With Urea Cycle Disorders[NCT00551200] | Phase 2 | 14 participants (Actual) | Interventional | 2007-10-31 | Completed | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
(NCT00551200)
Timeframe: during the period on 100% Buphenyl (up to 4 weeks) or HPN-100 (up to 10 weeks)
| Intervention | participants (Number) |
|---|---|
| Buphenyl | 7 |
| HPN-100 | 5 |
(NCT00551200)
Timeframe: during the period subjects on 100% Buphenyl (up to 4 weeks) or HPN-100 (up to 10 weeks)
| Intervention | participants (Number) |
|---|---|
| Buphenyl | 1 |
| HPN-100 | 0 |
(NCT00551200)
Timeframe: End of Study
| Intervention | participants (Number) | |
|---|---|---|
| prefer Buphenyl | prefer HPN-100 | |
| Buphenyl to HPN-100 | 1 | 9 |
measured AUC0-24 (Area under the curve from time 0 (pre-dose) to 24 hours) for each metabolite in plasma. Data were collected at 30 minutes and 1, 2, 4, 5, 6, 8, 10, 12, and 24 hours post-first dose. (NCT00551200)
Timeframe: At steady state (1 week) on each medication (Buphenyl® alone, HPN-100 alone)
| Intervention | μg*h/mL (Mean) | ||
|---|---|---|---|
| AUC0-24 PBA (phenylbutyrate) in plasma | AUC0-24 PAA (phenylacetate) in plasma | AUC0-24 PAGN (phenylacetylglutamine) in plasma | |
| HPN-100 Steady State | 540 | 575 | 1098 |
| NaPBA Steady State | 740 | 596 | 1133 |
Data were collected at pre-first dose and at 30 minutes and 1, 2, 4, 5, 6, 8, 10, 12, and 24 hours post first dose. (NCT00551200)
Timeframe: At steady state (1 week) on each medication (Buphenyl® alone, HPN-100 alone), and at steady state (1 week) after each dose escalation
| Intervention | μmol/L (Mean) | |
|---|---|---|
| in peak | in TNAUC (time-normalized area under the curve) | |
| HPN-100 Steady State | 56.3 | 26.5 |
| NaPBA Steady State | 79.1 | 38.4 |
5 reviews available for phenylacetic 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; Antimetabolites; Child, Preschool; Drug Combinations; | 1999 |
Alternative pathway therapy for urea cycle disorders: twenty years later.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Arginine; Humans; Hyperammonemia; Phenylacetates; Phe | 2001 |
Treatment of urea cycle disorders.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Benzoates; Benzoic Acid; Dietary Proteins; H | 1987 |
2 trials available for phenylacetic acid and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
Survival after treatment with phenylacetate and benzoate for urea-cycle disorders.
Topics: Adolescent; Adult; Age Factors; Age of Onset; Amino Acid Metabolism, Inborn Errors; Ammonia; Carbamo | 2007 |
Long-term treatment of girls with ornithine transcarbamylase deficiency.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Brain Diseases; | 1996 |
13 other studies available for phenylacetic acid and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia.
Topics: Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Animals; Area Under Curve; Butyrates; Cells, | 2021 |
Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Citrullinemia; | 2004 |
Alternative-pathway therapy for hyperammonemia.
Topics: Amino Acid Metabolism, Inborn Errors; Humans; Hyperammonemia; Phenylacetates; Sodium Benzoate; Urea | 2007 |
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 |
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 |
Prospective management of a child with neonatal citrullinemia.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Argininosuccinate Synthase; Benzoates; Benz | 1993 |
Current strategies for the management of neonatal urea cycle disorders.
Topics: Algorithms; Amino Acid Metabolism, Inborn Errors; Antimetabolites, Antineoplastic; Arginine; Dialysi | 2001 |
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 |
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 |
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 |
Pharmacologic amino acid acylation in the acute hyperammonemia of propionic acidemia.
Topics: Acylation; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Humans; Infant; Male; Phenyla | 1987 |