phenylacetic acid has been researched along with Deficiency Disease, Ornithine Carbamoyltransferase in 10 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phenylacetic acid : A monocarboxylic acid that is toluene in which one of the hydrogens of the methyl group has been replaced by a carboxy group.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) |
| "Patients with neonatal urea cycle defects (UCDs) typically experience severe hyperammonemia during the first days of life, which results in serious neurological injury or death." | 3.88 | Prenatal treatment of ornithine transcarbamylase deficiency. ( Akula, VP; Alcorn, D; Benitz, WE; Bernstein, JA; Berquist, WE; Blumenfeld, YJ; Castillo, RO; Concepcion, W; Cowan, TM; Cox, KL; Cusmano, K; Enns, GM; Esquivel, CO; Hintz, SR; Homeyer, M; Hudgins, L; Hurwitz, M; Lyell, DJ; Palma, JP; Schelley, S; Summar, ML; Wilnai, Y, 2018) |
| "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) |
| " Ambiguous medical prescriptions and inadequate cross-checking of drug dosage by physicians, nurses and pharmacists were the main causes of these incidents." | 1.31 | Three cases of intravenous sodium benzoate and sodium phenylacetate toxicity occurring in the treatment of acute hyperammonaemia. ( Boyadjiev, SA; Brusilow, SW; Geraghty, MT; Praphanphoj, V; Waber, LJ, 2000) |
| " 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 | 2 (20.00) | 18.7374 |
| 1990's | 2 (20.00) | 18.2507 |
| 2000's | 3 (30.00) | 29.6817 |
| 2010's | 3 (30.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Wilnai, Y | 1 |
| Blumenfeld, YJ | 1 |
| Cusmano, K | 1 |
| Hintz, SR | 1 |
| Alcorn, D | 1 |
| Benitz, WE | 1 |
| Berquist, WE | 1 |
| Bernstein, JA | 1 |
| Castillo, RO | 1 |
| Concepcion, W | 1 |
| Cowan, TM | 1 |
| Cox, KL | 1 |
| Lyell, DJ | 1 |
| Esquivel, CO | 1 |
| Homeyer, M | 1 |
| Hudgins, L | 1 |
| Hurwitz, M | 1 |
| Palma, JP | 1 |
| Schelley, S | 1 |
| Akula, VP | 1 |
| Summar, ML | 1 |
| Enns, GM | 2 |
| Nagasaka, H | 1 |
| Yorifuji, T | 1 |
| Kobayashi, K | 1 |
| Takikawa, H | 1 |
| Komatsu, H | 1 |
| Inui, A | 1 |
| Fujisawa, T | 1 |
| Miida, T | 1 |
| Tsukahara, H | 1 |
| Takatani, T | 1 |
| Hayashi, H | 1 |
| Houston, B | 1 |
| Reiss, KA | 1 |
| Merlo, C | 1 |
| Scaglia, F | 1 |
| Carter, S | 1 |
| O'Brien, WE | 1 |
| Lee, B | 1 |
| Berry, SA | 1 |
| Berry, GT | 1 |
| Rhead, WJ | 1 |
| Brusilow, SW | 4 |
| Hamosh, A | 1 |
| Danney, M | 1 |
| Waber, LJ | 2 |
| Batshaw, M | 1 |
| Burton, B | 1 |
| Levitsky, L | 1 |
| Roth, K | 1 |
| McKeethren, C | 1 |
| Ward, J | 1 |
| Maestri, NE | 1 |
| Clissold, DB | 1 |
| Bassett, SS | 1 |
| Thoene, JG | 1 |
| Praphanphoj, V | 1 |
| Boyadjiev, SA | 1 |
| Geraghty, MT | 1 |
| Batshaw, ML | 1 |
| Hyman, SL | 1 |
| Coyle, JT | 1 |
| Robinson, MB | 1 |
| Qureshi, IA | 1 |
| Mellits, ED | 1 |
| Quaskey, S | 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 |
1 review available for phenylacetic acid and Deficiency Disease, Ornithine Carbamoyltransferase
| Article | Year |
|---|---|
Treatment of urea cycle disorders.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Antimetabolites; Child, Preschool; Drug Combinations; | 1999 |
2 trials available for phenylacetic acid and Deficiency Disease, Ornithine Carbamoyltransferase
| 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 |
7 other studies available for phenylacetic acid and Deficiency Disease, Ornithine Carbamoyltransferase
| Article | Year |
|---|---|
Prenatal treatment of ornithine transcarbamylase deficiency.
Topics: Ammonia; Drug Combinations; Female; Glutamine; Humans; Hyperammonemia; Infant, Newborn; Male; Mutati | 2018 |
Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children.
Topics: Adenosine Triphosphatases; Adolescent; Arginine; ATP Binding Cassette Transporter, Subfamily B, Memb | 2010 |
Healthy, but comatose.
Topics: Adult; Brain Edema; Carbohydrates; Enteral Nutrition; Glucose; Humans; Hyperammonemia; Insulin; Male | 2011 |
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 |
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 |
Three cases of intravenous sodium benzoate and sodium phenylacetate toxicity occurring in the treatment of acute hyperammonaemia.
Topics: Child; Child, Preschool; Drug Overdose; Fatal Outcome; Female; Humans; Injections, Intravenous; Male | 2000 |
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 |