glutamine and Inborn Urea Cycle Disorder

glutamine has been researched along with Inborn Urea Cycle Disorder in 25 studies

Research

Studies (25)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's21 (84.00)24.3611
2020's4 (16.00)2.80

Authors

AuthorsStudies
Burczynski, ME; Chen, H; Cheng, X; Halasz, G; Kim, S; Lin, AZ; Mao, X; Murphy, AJ; Na, E; Okamoto, H; Sleeman, MW1
Lichter-Konecki, U; McCarter, R; Sanz, JH1
Daniotti, M; Donati, MA; Pochiero, F; Procopio, E; Sacchini, M; Scaturro, G1
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
Fiumara, A; Iacobacci, R; Meli, C; Messina, M; Raudino, F1
Almannai, M; Elsea, SH; Jiang, Y; Sun, Q; Sutton, VR1
Berry, SA; Diaz, GA; Ficicioglu, C; Harding, CO; Lichter-Konecki, U; Longo, N; McCandless, SE; Robinson, B; Smith, WE; Vockley, J; Zori, R1
Berry, SA; Diaz, GA; Dong, M; Ficicioglu, C; Harding, CO; Holt, RJ; Lichter-Konecki, U; Longo, N; McCandless, SE; Perdok, R; Robinson, B; Smith, WE; Vinks, AA; Vockley, J; Zori, R1
Brunetti-Pierri, N; Soria, LR1
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
Cooper, AJ; Kuhara, T1
Berry, SA; Coakley, DF; Diaz, GA; Lee, B; Lemons, C; Lichter-Konecki, U; McCandless, SE; Mokhtarani, M; Nagamani, SC; Rhead, W; Scharschmidt, BF; Smith, W1
Bartholomew, D; Bartley, JA; Berquist, W; Berry, SA; Cederbaum, S; Coakley, DF; Diaz, GA; Feigenbaum, A; Gallagher, R; Harding, CO; Korson, MS; Kronn, D; Le Mons, C; Lee, B; Lichter-Konecki, U; Longo, N; Marino, M; McCandless, SE; Merritt, JL; Milikien, DA; Mokhtarani, M; Nagamani, SC; Rhead, W; Scharschmidt, BF; Schulze, A; Smith, W; Summar, M; Vockley, G; Vockley, J; Wong, D; Zori, R1
Bartley, J; Berquist, W; Berry, SA; Coakley, DF; Diaz, GA; Feigenbaum, A; Gallagher, RC; Harding, CO; Le Mons, C; Lee, B; Lichter-Konecki, U; Longo, N; Marino, M; McCandless, SE; Mokhtarani, M; Nagamani, SC; Rhead, W; Rowell, R; Scharschmidt, BF; Schulze, A; Smith, W1
Aldámiz-Echevarría, L; De Las Heras, J; Delgado, TC; Martínez-Chantar, ML1
Arranz, JA; Artuch, R; Briones, P; Campistol, J; Fons, C; García-Cazorla, A; Gómez-López, L; Häberle, J; Jara, F; Martins, C; Murgui, E; Pérez-Dueñas, B; Pineda, M; Serrano, M; Vilaseca, MA1
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
Artuch, R; Campistol, J; Garcia-Cazorla, A; Garcia-Romero, R; Lambruschini, N; Meavilla, S; Ormazábal, A; Perez-Dueñas, B; Pineda, M; Serrano, M; Vilaseca, MA1
Walker, V1
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
Bart, SA; Bartholomew, D; Bartley, J; Berquist, W; Berry, SA; C S Nagamani, S; Cederbaum, S; Coakley, DF; Diaz, GA; Dickinson, K; Feigenbaum, A; Gallagher, R; Harding, CO; Korson, MS; Krivitzky, LS; Kronn, D; Lee, B; Lemons, C; Lichter-Konecki, U; Longo, N; Mauney, J; McCandless, SE; Merritt, JL; Mokhtarani, M; Moors, TL; Rhead, W; Scharschmidt, BF; Smith, W; Vockley, G; 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

Reviews

4 review(s) available for glutamine and Inborn Urea Cycle Disorder

ArticleYear
Ammonia and autophagy: An emerging relationship with implications for disorders with hyperammonemia.
    Journal of inherited metabolic disease, 2019, Volume: 42, Issue:6

    Topics: Ammonia; Animals; Autophagy; Glutamate-Ammonia Ligase; Glutamine; Homeostasis; Humans; Hyperammonemia; Liver; Urea; Urea Cycle Disorders, Inborn

2019
α-Ketoglutaramate: an overlooked metabolite of glutamine and a biomarker for hepatic encephalopathy and inborn errors of the urea cycle.
    Metabolic brain disease, 2014, Volume: 29, Issue:4

    Topics: Amidohydrolases; Aminohydrolases; Ammonia; Animals; Biomarkers; Carbon; Glutamine; Hepatic Encephalopathy; Humans; Hyperammonemia; Keto Acids; Ketoglutaric Acids; Kynurenine; Liver Diseases; Mammals; Methionine; Nitrogen; Sulfur; Transaminases; Urea Cycle Disorders, Inborn

2014
An update on the use of benzoate, phenylacetate and phenylbutyrate ammonia scavengers for interrogating and modifying liver nitrogen metabolism and its implications in urea cycle disorders and liver disease.
    Expert opinion on drug metabolism & toxicology, 2017, Volume: 13, Issue:4

    Topics: Ammonia; Animals; Benzoates; Drug Design; Glutamine; Humans; Liver Cirrhosis; Liver Diseases; Liver Failure, Acute; Nitrogen; Phenylacetates; Phenylbutyrates; Urea Cycle Disorders, Inborn

2017
Severe hyperammonaemia in adults not explained by liver disease.
    Annals of clinical biochemistry, 2012, Volume: 49, Issue:Pt 3

    Topics: Adult; Amino Acids; Ammonia; Biological Transport; Blood-Brain Barrier; Brain; Glutamine; Humans; Hyperammonemia; Liver; Liver Diseases; Nitrogen; Urea; Urea Cycle Disorders, Inborn

2012

Trials

7 trial(s) available for glutamine and Inborn Urea Cycle Disorder

ArticleYear
Glycerol phenylbutyrate treatment in children with urea cycle disorders: pooled analysis of short and long-term ammonia control and outcomes.
    Molecular genetics and metabolism, 2014, Volume: 112, Issue:1

    Topics: Adolescent; Ammonia; Child; Child, Preschool; Cross-Over Studies; Female; Glutamine; Humans; Infant; Infant, Newborn; Male; Phenylbutyrates; Urea Cycle Disorders, Inborn

2014
Glutamine and hyperammonemic crises in patients with urea cycle disorders.
    Molecular genetics and metabolism, 2016, Volume: 117, Issue:1

    Topics: Adolescent; Adult; Ammonia; Biomarkers; Child; Child, Preschool; Fasting; Female; Glutamine; Glycerol; Humans; Hyperammonemia; Male; Phenylbutyrates; Predictive Value of Tests; Urea Cycle Disorders, Inborn; Young Adult

2016
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 and neurocognitive outcome among urea cycle disorder patients treated with glycerol phenylbutyrate.
    Hepatology (Baltimore, Md.), 2013, Volume: 57, Issue:6

    Topics: Adolescent; Adult; Ammonia; Child; Cross-Over Studies; Double-Blind Method; Female; Glutamine; Glycerol; Humans; Male; Middle Aged; Neuropsychological Tests; Phenylbutyrates; Urea Cycle Disorders, Inborn; Young Adult

2013
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

14 other study(ies) available for glutamine and Inborn Urea Cycle Disorder

ArticleYear
Glutaminase 2 knockdown reduces hyperammonemia and associated lethality of urea cycle disorder mouse model.
    Journal of inherited metabolic disease, 2022, Volume: 45, Issue:3

    Topics: Ammonia; Animals; Disease Models, Animal; Glutaminase; Glutamine; Humans; Hyperammonemia; Liver; Mice; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Urea; Urea Cycle Disorders, Inborn

2022
Relationship between longitudinal changes in neuropsychological outcome and disease biomarkers in urea cycle disorders.
    Pediatric research, 2023, Volume: 94, Issue:6

    Topics: Ammonia; Biomarkers; Glutamine; Humans; Hyperammonemia; Longitudinal Studies; Neuropsychological Tests; Urea Cycle Disorders, Inborn

2023
Transition to glycerol phenylbutyrate for the management of urea cycle disorders: clinical experiences.
    European review for medical and pharmacological sciences, 2023, Volume: 27, Issue:22

    Topics: Ammonia; Glutamine; Humans; Quality of Life; Urea; Urea Cycle Disorders, Inborn

2023
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
New ratio as a useful marker for early diagnosis of proximal urea cycle disorders.
    Clinica chimica acta; international journal of clinical chemistry, 2021, Volume: 520

    Topics: Biomarkers; Citrulline; Early Diagnosis; Glutamine; Humans; Infant, Newborn; Urea Cycle Disorders, Inborn

2021
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
Safety and efficacy of glycerol phenylbutyrate for management of urea cycle disorders in patients aged 2months to 2years.
    Molecular genetics and metabolism, 2017, Volume: 122, Issue:3

    Topics: Ammonia; Child, Preschool; Cough; Disease Management; Drug-Related Side Effects and Adverse Reactions; Female; Fever; Glutamine; Glycerol; Humans; Infant; Male; Neutropenia; Phenylbutyrates; Prospective Studies; Retrospective Studies; Urea Cycle Disorders, Inborn

2017
Pharmacokinetics of glycerol phenylbutyrate in pediatric patients 2 months to 2 years of age with urea cycle disorders.
    Molecular genetics and metabolism, 2018, Volume: 125, Issue:3

    Topics: Child; Child, Preschool; Female; Glutamine; Glycerol; Humans; Infant; Lipase; Male; Nitrogen; Phenylacetates; Phenylbutyrates; Prodrugs; Urea Cycle Disorders, Inborn

2018
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
Blood ammonia and glutamine as predictors of hyperammonemic crises in patients with urea cycle disorder.
    Genetics in medicine : official journal of the American College of Medical Genetics, 2015, Volume: 17, Issue:7

    Topics: Adolescent; Adult; Ammonia; Child; Child, Preschool; Female; Glutamine; Humans; Hyperammonemia; Infant; Male; Middle Aged; Predictive Value of Tests; Urea Cycle Disorders, Inborn; Young Adult

2015
Neuropsychiatric manifestations in late-onset urea cycle disorder patients.
    Journal of child neurology, 2010, Volume: 25, Issue:3

    Topics: Adolescent; Adult; Age of Onset; Carbamoyl-Phosphate Synthase I Deficiency Disease; Child; Child, Preschool; Citrullinemia; Female; Follow-Up Studies; Glutamine; Humans; Male; Mental Disorders; Ornithine Carbamoyltransferase Deficiency Disease; Quaternary Ammonium Compounds; Retrospective Studies; Treatment Outcome; Urea Cycle Disorders, Inborn

2010
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
Assessment of plasma ammonia and glutamine concentrations in urea cycle disorders.
    Clinical biochemistry, 2011, Volume: 44, Issue:8-9

    Topics: Ammonia; Glutamine; Humans; Infant, Newborn; Urea Cycle Disorders, Inborn

2011