Page last updated: 2024-08-26

phenylacetylglutamine and Inborn Urea Cycle Disorder

phenylacetylglutamine has been researched along with Inborn Urea Cycle Disorder in 9 studies

Research

Studies (9)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's9 (100.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Aldámiz-Echevarría, L; Almeida Campos, T; Andrade, F; Barba Romero, MA; Blasco, J; Cañedo, E; Ceberio, L; Correcher, P; de Las Heras, J; Del Toro, M; García Silva, MT; García-Jiménez, MC; Gil, D; Marquez, AM; Martín Hernández, E; Peña-Quintana, L; Pintos-Morell, G; Puig-Piña, R; Quijada-Fraile, P; Rodrigues, E; Ruiz-Pons, M; Villate, O; Vitoria, I; Vives-Piñera, I; Yahyaoui, R1
Almannai, M; Elsea, SH; Jiang, Y; Sun, Q; Sutton, VR1
Berry, SA; Coakley, D; Diaz, GA; Dickinson, K; Lee, B; Lemons, C; Lichter-Konecki, U; Mokhtarani, M; Monteleone, JP; Rhead, W; Scharschmidt, BF1
Bartley, J; Berquist, W; Berry, SA; Brown, RS; Coakley, D; Diaz, GA; Dickinson, K; Feigenbaum, A; Gallagher, R; Ghabril, M; Harding, C; Lee, B; Lemons, C; Lichter-Konecki, U; Longo, N; Mantry, P; McCandless, SE; Milikien, DA; Mokhtarani, M; Moors, T; Nagamani, SC; Norris, C; Rhead, W; Rockey, DC; Scharschmidt, BF; Schulze, A; Smith, W; Vierling, JM1
Beliveau, M; Berry, SA; Diaz, GA; Dickinson, K; Gargosky, S; Lee, B; Marier, JF; Martinez, A; Mauney, J; Mian, A; Mokhtarani, M; Rhead, W; Scharschmidt, BF; Shchelochkov, O1
Dickinson, K; Gargosky, S; Lowe, ME; Martinez, A; McGuire, BM; Mokhtarani, M; Monteleone, J; Scharschmidt, BF; Syplyviy, VA; Xiao, X; Zupanets, IA1
Herebian, D; Laryea, MD; Mayatepek, E; Meissner, T1
Bart, S; Bartholomew, D; Bartley, J; Berquist, W; Berry, SA; Cederbaum, S; Coakley, DF; Diaz, GA; Dickinson, K; Dorrani, N; Feigenbaum, A; Gallagher, R; Harding, CO; Korson, MS; Kronn, D; Lee, B; Lemons, C; Lichter-Konecki, U; Longo, N; McCandless, SE; Merritt, JL; Mokhtarani, M; Moors, TL; Rhead, W; Scharschmidt, BF; Smith, W; Sreenath-Nagamani, S; Summar, M; Vockley, J; Zori, R1
Berry, SA; Coakley, DF; Diaz, GA; Dickinson, K; Harding, CO; Lee, B; LeMons, C; Lichter-Konecki, U; Mauney, J; McCandless, SE; Mokhtarani, M; Moors, T; Scharschmidt, BF; Smith, W1

Trials

4 trial(s) available for phenylacetylglutamine and Inborn Urea Cycle Disorder

ArticleYear
Phase 2 comparison of a novel ammonia scavenging agent with sodium phenylbutyrate in patients with urea cycle disorders: safety, pharmacokinetics and ammonia control.
    Molecular genetics and metabolism, 2010, Volume: 100, Issue:3

    Topics: Adult; Aged; Ammonia; Cross-Over Studies; Female; Glutamine; Glycerol; Humans; Male; Middle Aged; Phenylacetates; Phenylbutyrates; Urea Cycle Disorders, Inborn; Young Adult

2010
Pharmacology and safety of glycerol phenylbutyrate in healthy adults and adults with cirrhosis.
    Hepatology (Baltimore, Md.), 2010, Volume: 51, Issue:6

    Topics: Adult; Cross-Over Studies; Female; Glutamine; Humans; Hydrolysis; Lipase; Liver Cirrhosis; Male; Monte Carlo Method; Phenylbutyrates; Urea Cycle Disorders, Inborn

2010
Urinary phenylacetylglutamine as dosing biomarker for patients with urea cycle disorders.
    Molecular genetics and metabolism, 2012, Volume: 107, Issue:3

    Topics: Adolescent; Adult; Ammonia; Biomarkers, Pharmacological; Child; Cross-Over Studies; Drug Administration Schedule; Female; Glutamine; Glycerol; Humans; Male; Phenylacetates; Phenylbutyrates; Urea Cycle Disorders, Inborn

2012
Ammonia control in children ages 2 months through 5 years with urea cycle disorders: comparison of sodium phenylbutyrate and glycerol phenylbutyrate.
    The Journal of pediatrics, 2013, Volume: 162, Issue:6

    Topics: Ammonia; Child; Child, Preschool; Drug Substitution; Female; Glutamine; Glycerol; Humans; Infant; Liver; Male; Phenylbutyrates; Surveys and Questionnaires; Urea Cycle Disorders, Inborn

2013

Other Studies

5 other study(ies) available for phenylacetylglutamine and Inborn Urea Cycle Disorder

ArticleYear
Quantification of urinary derivatives of Phenylbutyric and Benzoic acids by LC-MS/MS as treatment compliance biomarkers in Urea Cycle disorders.
    Journal of pharmaceutical and biomedical analysis, 2019, Nov-30, Volume: 176

    Topics: Adolescent; Adult; Benzoates; Biomarkers; Child; Child, Preschool; Chromatography, High Pressure Liquid; Drug Monitoring; Female; Glutamine; Humans; Infant; Infant, Newborn; Male; Medication Adherence; Phenylbutyrates; Tandem Mass Spectrometry; Urea Cycle Disorders, Inborn; Young Adult

2019
Quantitation of phenylbutyrate metabolites by UPLC-MS/MS demonstrates inverse correlation of phenylacetate:phenylacetylglutamine ratio with plasma glutamine levels.
    Molecular genetics and metabolism, 2017, Volume: 122, Issue:3

    Topics: Ammonia; Argininosuccinic Aciduria; Chromatography, Liquid; Female; Glutamine; Glycerol; Humans; Limit of Detection; Male; Middle Aged; Ornithine Carbamoyltransferase Deficiency Disease; Phenylacetates; Phenylbutyrates; Tandem Mass Spectrometry; Urea; Urea Cycle Disorders, Inborn

2017
Population pharmacokinetic modeling and dosing simulations of nitrogen-scavenging compounds: disposition of glycerol phenylbutyrate and sodium phenylbutyrate in adult and pediatric patients with urea cycle disorders.
    Journal of clinical pharmacology, 2013, Volume: 53, Issue:7

    Topics: Adult; Child; Child, Preschool; Clinical Trials, Phase II as Topic; Clinical Trials, Phase III as Topic; Computer Simulation; Female; Glutamine; Glycerol; Humans; Male; Models, Biological; Nitrogen; Phenylbutyrates; Randomized Controlled Trials as Topic; Rare Diseases; Urea Cycle Disorders, Inborn

2013
Elevated phenylacetic acid levels do not correlate with adverse events in patients with urea cycle disorders or hepatic encephalopathy and can be predicted based on the plasma PAA to PAGN ratio.
    Molecular genetics and metabolism, 2013, Volume: 110, Issue:4

    Topics: Biomarkers; Drug-Related Side Effects and Adverse Reactions; Glutamine; Glycerol; Hepatic Encephalopathy; Humans; Liver; Neoplasms; Phenylacetates; Phenylbutyrates; Randomized Controlled Trials as Topic; Urea Cycle Disorders, Inborn

2013
Simultaneous LC-MS/MS determination of phenylbutyrate, phenylacetate benzoate and their corresponding metabolites phenylacetylglutamine and hippurate in blood and urine.
    Journal of inherited metabolic disease, 2010, Volume: 33 Suppl 3

    Topics: Benzoates; Biomarkers; Biotransformation; Calibration; Chromatography, Reverse-Phase; Glutamine; Hippurates; Humans; Hyperammonemia; Limit of Detection; Linear Models; Phenylacetates; Phenylbutyrates; Predictive Value of Tests; Reference Standards; Reproducibility of Results; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Urea Cycle Disorders, Inborn

2010