urea has been researched along with Inborn Urea Cycle Disorder in 36 studies
pseudourea: clinical use; structure
isourea : A carboximidic acid that is the imidic acid tautomer of urea, H2NC(=NH)OH, and its hydrocarbyl derivatives.
| Excerpt | Relevance | Reference |
|---|---|---|
| " Urea cycle disorders with hyperammonemia remain difficult to treat and eventually necessitate liver transplantation." | 8.12 | Glutaminase 2 knockdown reduces hyperammonemia and associated lethality of urea cycle disorder mouse model. ( Burczynski, ME; Chen, H; Cheng, X; Halasz, G; Kim, S; Lin, AZ; Mao, X; Murphy, AJ; Na, E; Okamoto, H; Sleeman, MW, 2022) |
| "Children with urea cycle disorders (UCDs) or organic acidemias (OAs) and acute hyperammonemia and encephalopathy are at great risk for neurological injury, developmental delay, intellectual disability, and death." | 7.79 | Feasibility of adjunct therapeutic hypothermia treatment for hyperammonemia and encephalopathy due to urea cycle disorders and organic acidemias. ( Baumgart, S; Cook, N; Dimmock, D; Lichter-Konecki, U; Meyer, MT; Moudgil, A; Nadkarni, V; Poeschl, J, 2013) |
| "The urea cycle disorders are a group of inherited biochemical diseases caused by a complete or partial deficiency of any one of the enzymes or transport proteins required to convert toxic ammonia into urea and to produce arginine and citrulline." | 6.58 | Inborn Errors of Metabolism with Hyperammonemia: Urea Cycle Defects and Related Disorders. ( Mew, NA; Summar, ML, 2018) |
| "Chronic inflammation is linked to diverse disease processes, but the intrinsic mechanisms that determine cellular sensitivity to inflammation are incompletely understood." | 5.56 | Glucose-dependent partitioning of arginine to the urea cycle protects β-cells from inflammation. ( Alvarez-Perez, JC; Avizonis, D; Badur, MG; Bird, GH; Bridon, G; Choi, DW; Danial, NN; Dhe-Paganon, S; Evans, L; Ficarro, SB; Fu, A; Garcia-Ocana, A; James Shapiro, AM; Jones, RG; Kamphorst, JJ; Karakose, E; Kin, T; Marto, JA; Rosselot, C; Seo, HS; Stewart, AF; Walensky, LD, 2020) |
| " Urea cycle disorders with hyperammonemia remain difficult to treat and eventually necessitate liver transplantation." | 4.12 | Glutaminase 2 knockdown reduces hyperammonemia and associated lethality of urea cycle disorder mouse model. ( Burczynski, ME; Chen, H; Cheng, X; Halasz, G; Kim, S; Lin, AZ; Mao, X; Murphy, AJ; Na, E; Okamoto, H; Sleeman, MW, 2022) |
| "Children with urea cycle disorders (UCDs) or organic acidemias (OAs) and acute hyperammonemia and encephalopathy are at great risk for neurological injury, developmental delay, intellectual disability, and death." | 3.79 | Feasibility of adjunct therapeutic hypothermia treatment for hyperammonemia and encephalopathy due to urea cycle disorders and organic acidemias. ( Baumgart, S; Cook, N; Dimmock, D; Lichter-Konecki, U; Meyer, MT; Moudgil, A; Nadkarni, V; Poeschl, J, 2013) |
| " No statistically significant differences were observed in plasma phenylacetic acid and PAGN exposure during dosing with GPB vs." | 2.76 | Ammonia control in children with urea cycle disorders (UCDs); phase 2 comparison of sodium phenylbutyrate and glycerol phenylbutyrate. ( Beliveau, M; Diaz, GA; Dickinson, K; Feigenbaum, A; Jomphe, C; Lichter-Konecki, U; Marier, JF; Martinez, A; Mauney, J; Merritt, JL; Mokhtarani, M; Rhead, W; Scharschmidt, B, 2011) |
| "The urea cycle disorders are a group of inherited biochemical diseases caused by a complete or partial deficiency of any one of the enzymes or transport proteins required to convert toxic ammonia into urea and to produce arginine and citrulline." | 2.58 | Inborn Errors of Metabolism with Hyperammonemia: Urea Cycle Defects and Related Disorders. ( Mew, NA; Summar, ML, 2018) |
| "ARG1-deficient patients exhibit hyperargininemia with spastic paraparesis, progressive neurological and intellectual impairment, persistent growth retardation, and infrequent episodes of hyperammonemia, a clinical pattern that differs strikingly from other urea cycle disorders." | 2.52 | Arginase-1 deficiency. ( Baron, G; Funk, CD; Schulze, A; Sin, YY, 2015) |
| "Gene therapy for OTC deficiency is effective in animals, and work is ongoing to improve persistence and safety." | 2.45 | Ammonia toxicity and its prevention in inherited defects of the urea cycle. ( Walker, V, 2009) |
| " Four Phase 1 studies were conducted to characterize the bioavailability (BA) and/or bioequivalence (BE) of ACER-001 (in healthy volunteers) and taste assessment relative to NaPBA powder (in taste panelists)." | 1.91 | Taste-masked formulation of sodium phenylbutyrate (ACER-001) for the treatment of urea cycle disorders. ( Cederbaum, SD; Edwards, J; Kellmeyer, T; Peters, Y; Steiner, RD, 2023) |
| " Users rated the test as "often helpful" for ruling out PAA toxicity (44%), informing PB dosing decisions (42%), and assessing adherence (28%)." | 1.72 | Perceptions and use of phenylbutyrate metabolite testing in urea cycle disorders: Results of a clinician survey and analysis of a centralized testing database. ( Burdett, A; Ficicioglu, C; Hata, A; Liu, N; Porter, M; Sun, Q; Sutton, VR, 2022) |
| "Chronic inflammation is linked to diverse disease processes, but the intrinsic mechanisms that determine cellular sensitivity to inflammation are incompletely understood." | 1.56 | Glucose-dependent partitioning of arginine to the urea cycle protects β-cells from inflammation. ( Alvarez-Perez, JC; Avizonis, D; Badur, MG; Bird, GH; Bridon, G; Choi, DW; Danial, NN; Dhe-Paganon, S; Evans, L; Ficarro, SB; Fu, A; Garcia-Ocana, A; James Shapiro, AM; Jones, RG; Kamphorst, JJ; Karakose, E; Kin, T; Marto, JA; Rosselot, C; Seo, HS; Stewart, AF; Walensky, LD, 2020) |
| "Hyperammonemia was diagnosed in all cases during the evaluation of altered mental status, with 22% presenting with seizures." | 1.56 | A retrospective study of adult patients with noncirrhotic hyperammonemia. ( Baker, JJ; Barkoudah, E; Berry, GT; Khoury, CC; Krier, JB; Lin, AP; Mogensen, KM; Peake, RW; Sahai, I; Stergachis, AB; Sweetser, DA, 2020) |
| "The proportion of females with ornithine transcarbamylase deficiency (fOTC-D), particularly those being asymptomatic (asfOTC-D), was higher in the NA than in the EU sample." | 1.51 | Transatlantic combined and comparative data analysis of 1095 patients with urea cycle disorders-A successful strategy for clinical research of rare diseases. ( Batshaw, ML; Baumgartner, MR; Boy, N; Burgard, P; Burlina, AB; de Lonlay, P; Dionisi-Vici, C; Dobbelaere, D; Garbade, SF; Garcia-Cazorla, A; Hoffmann, GF; Kölker, S; McCandless, SE; Mew, NA; Posset, R; Seminara, J; Summar, M; Teles, EL; Vara, R, 2019) |
| "The most common UCD was ornithine transcarbamylase deficiency (OTCD), which accounted for 116 out of 177 patients." | 1.38 | Long-term outcome and intervention of urea cycle disorders in Japan. ( Endo, F; Horikawa, R; Kasahara, M; Kido, J; Matsuo, M; Mitsubuchi, H; Nakamura, K; Ohura, T; Shigematsu, Y; Takayanagi, M; Yorifuji, T; Yoshino, M, 2012) |
| "Creatine synthesis is a major component of arginine metabolism, amounting to more than 20% of the dietary intake of this amino acid." | 1.36 | Creatine metabolism and the urea cycle. ( Brosnan, JT; Brosnan, ME, 2010) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 1 (2.78) | 29.6817 |
| 2010's | 23 (63.89) | 24.3611 |
| 2020's | 12 (33.33) | 2.80 |
| Authors | Studies |
|---|---|
| Hajaj, E | 1 |
| Sciacovelli, M | 1 |
| Frezza, C | 1 |
| Erez, A | 1 |
| Ficicioglu, C | 1 |
| Liu, N | 1 |
| Sun, Q | 3 |
| Burdett, A | 1 |
| Hata, A | 1 |
| Porter, M | 1 |
| Sutton, VR | 3 |
| Mao, X | 1 |
| Chen, H | 1 |
| Lin, AZ | 1 |
| Kim, S | 1 |
| Burczynski, ME | 1 |
| Na, E | 1 |
| Halasz, G | 1 |
| Sleeman, MW | 1 |
| Murphy, AJ | 1 |
| Okamoto, H | 1 |
| Cheng, X | 1 |
| Siri, B | 1 |
| Olivieri, G | 1 |
| Angeloni, A | 1 |
| Cairoli, S | 1 |
| Carducci, C | 1 |
| Cotugno, G | 1 |
| Di Michele, S | 1 |
| Giovanniello, T | 1 |
| La Marca, G | 1 |
| Lepri, FR | 1 |
| Novelli, A | 1 |
| Rossi, C | 1 |
| Semeraro, M | 1 |
| Dionisi-Vici, C | 3 |
| Lopes, FF | 1 |
| Lamberty Faverzani, J | 1 |
| Hammerschmidt, T | 1 |
| Aguilar Delgado, C | 1 |
| Ferreira de Oliveira, J | 1 |
| Wajner, M | 1 |
| Regla Vargas, C | 1 |
| Cederbaum, SD | 1 |
| Edwards, J | 1 |
| Kellmeyer, T | 1 |
| Peters, Y | 1 |
| Steiner, RD | 1 |
| Imbard, A | 1 |
| Bouchereau, J | 1 |
| Arnoux, JB | 1 |
| Brassier, A | 2 |
| Schiff, M | 2 |
| Bérat, CM | 1 |
| Pontoizeau, C | 1 |
| Benoist, JF | 1 |
| Josse, C | 1 |
| Montestruc, F | 1 |
| de Lonlay, P | 2 |
| Sacchini, M | 1 |
| Procopio, E | 1 |
| Pochiero, F | 1 |
| Scaturro, G | 1 |
| Daniotti, M | 1 |
| Donati, MA | 1 |
| Fu, A | 1 |
| Alvarez-Perez, JC | 1 |
| Avizonis, D | 1 |
| Kin, T | 1 |
| Ficarro, SB | 1 |
| Choi, DW | 1 |
| Karakose, E | 1 |
| Badur, MG | 1 |
| Evans, L | 1 |
| Rosselot, C | 1 |
| Bridon, G | 1 |
| Bird, GH | 1 |
| Seo, HS | 1 |
| Dhe-Paganon, S | 1 |
| Kamphorst, JJ | 1 |
| Stewart, AF | 1 |
| James Shapiro, AM | 1 |
| Marto, JA | 1 |
| Walensky, LD | 1 |
| Jones, RG | 1 |
| Garcia-Ocana, A | 1 |
| Danial, NN | 1 |
| Stergachis, AB | 1 |
| Mogensen, KM | 1 |
| Khoury, CC | 1 |
| Lin, AP | 1 |
| Peake, RW | 1 |
| Baker, JJ | 1 |
| Barkoudah, E | 1 |
| Sahai, I | 1 |
| Sweetser, DA | 1 |
| Berry, GT | 1 |
| Krier, JB | 1 |
| Nitzahn, M | 1 |
| Lipshutz, GS | 1 |
| Sonaimuthu, P | 1 |
| Senkevitch, E | 1 |
| Haskins, N | 1 |
| Uapinyoying, P | 1 |
| McNutt, M | 1 |
| Morizono, H | 1 |
| Tuchman, M | 1 |
| Caldovic, L | 1 |
| Jiang, Y | 1 |
| Almannai, M | 1 |
| Elsea, SH | 2 |
| Meyburg, J | 2 |
| Opladen, T | 2 |
| Spiekerkötter, U | 1 |
| Schlune, A | 1 |
| Schenk, JP | 1 |
| Schmidt, J | 1 |
| Weitz, J | 1 |
| Okun, J | 1 |
| Bürger, F | 1 |
| Omran, TB | 1 |
| Abdoh, G | 1 |
| Al Rifai, H | 1 |
| Monavari, A | 1 |
| Konstantopoulou, V | 1 |
| Kölker, S | 3 |
| Yudkoff, M | 2 |
| Hoffmann, GF | 3 |
| Summar, ML | 1 |
| Mew, NA | 2 |
| Nagamani, SCS | 2 |
| Agarwal, U | 1 |
| Tam, A | 1 |
| Azamian, M | 1 |
| McMeans, A | 1 |
| Didelija, IC | 1 |
| Mohammad, MA | 1 |
| Marini, JC | 1 |
| Burrage, LC | 1 |
| Thistlethwaite, L | 1 |
| Stroup, BM | 1 |
| Miller, MJ | 1 |
| Craigen, W | 1 |
| Scaglia, F | 1 |
| Graham, B | 1 |
| Kennedy, AD | 1 |
| Milosavljevic, A | 1 |
| Lee, BH | 1 |
| Soria, LR | 2 |
| Brunetti-Pierri, N | 2 |
| Posset, R | 2 |
| Garbade, SF | 1 |
| Boy, N | 1 |
| Burlina, AB | 2 |
| Dobbelaere, D | 2 |
| Garcia-Cazorla, A | 2 |
| Teles, EL | 2 |
| Vara, R | 2 |
| Batshaw, ML | 1 |
| Baumgartner, MR | 1 |
| McCandless, SE | 1 |
| Seminara, J | 1 |
| Summar, M | 1 |
| Burgard, P | 3 |
| Ninković, D | 1 |
| Mustapić, Ž | 1 |
| Bartoniček, D | 1 |
| Benjak, V | 1 |
| Ćuk, M | 1 |
| Buljević, AD | 1 |
| Grčić, BF | 1 |
| Fumić, K | 1 |
| Grizelj, R | 1 |
| Lehman, I | 1 |
| Ramadža, DP | 1 |
| Sarnavka, V | 1 |
| Slaviček, J | 1 |
| Kastelić, JS | 1 |
| Barišić, N | 1 |
| Barić, I | 1 |
| Ah Mew, N | 1 |
| Lichter-Konecki, U | 2 |
| Nadkarni, V | 1 |
| Moudgil, A | 1 |
| Cook, N | 1 |
| Poeschl, J | 1 |
| Meyer, MT | 1 |
| Dimmock, D | 1 |
| Baumgart, S | 1 |
| Helman, G | 1 |
| Pacheco-Colón, I | 1 |
| Gropman, AL | 1 |
| MacLeod, EL | 1 |
| Hall, KD | 1 |
| McGuire, PJ | 1 |
| Sin, YY | 1 |
| Baron, G | 1 |
| Schulze, A | 1 |
| Funk, CD | 1 |
| Lindner, M | 1 |
| Das, AM | 1 |
| Marquardt, T | 1 |
| Khan, A | 1 |
| Emre, SH | 1 |
| Burton, BK | 1 |
| Barshop, BA | 1 |
| Böhm, T | 1 |
| Zangerl, K | 1 |
| Mayorandan, S | 1 |
| Dürr, UH | 1 |
| Rosenkranz, B | 1 |
| Rennecke, J | 1 |
| Derbinski, J | 1 |
| Anstey, JR | 1 |
| Haydon, TP | 1 |
| Ghanpur, RB | 1 |
| de Jong, G | 1 |
| Valayannopoulos, V | 1 |
| Cortès-Saladelafont, E | 1 |
| Couce, ML | 1 |
| Sykut-Cegielska, J | 1 |
| Häberle, J | 1 |
| Lund, AM | 1 |
| Chakrapani, A | 1 |
| Walter, JH | 1 |
| Zeman, J | 1 |
| Açıkalın, A | 1 |
| Dişel, NR | 1 |
| Walker, V | 2 |
| Bonora-Centelles, A | 1 |
| Donato, MT | 1 |
| Lahoz, A | 1 |
| Pareja, E | 1 |
| Mir, J | 1 |
| Castell, JV | 1 |
| Gómez-Lechón, MJ | 1 |
| Brosnan, JT | 1 |
| Brosnan, ME | 1 |
| Diaz, GA | 1 |
| Merritt, JL | 1 |
| Feigenbaum, A | 1 |
| Jomphe, C | 1 |
| Marier, JF | 1 |
| Beliveau, M | 1 |
| Mauney, J | 1 |
| Dickinson, K | 1 |
| Martinez, A | 1 |
| Mokhtarani, M | 1 |
| Scharschmidt, B | 1 |
| Rhead, W | 1 |
| Kido, J | 1 |
| Nakamura, K | 1 |
| Mitsubuchi, H | 1 |
| Ohura, T | 1 |
| Takayanagi, M | 1 |
| Matsuo, M | 1 |
| Yoshino, M | 1 |
| Shigematsu, Y | 1 |
| Yorifuji, T | 1 |
| Kasahara, M | 1 |
| Horikawa, R | 1 |
| Endo, F | 1 |
| Krauss, M | 1 |
| Schaller, S | 1 |
| Borchers, S | 1 |
| Findeisen, R | 1 |
| Lippert, J | 1 |
| Kuepfer, L | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Longitudinal Study of Urea Cycle Disorders[NCT00237315] | 1,009 participants (Anticipated) | Observational | 2006-02-28 | Recruiting | |||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
8 reviews available for urea and Inborn Urea Cycle Disorder
| Article | Year |
|---|---|
The context-specific roles of urea cycle enzymes in tumorigenesis.
Topics: Ammonia; Carcinogenesis; Cell Line, Tumor; Cell Proliferation; Cell Transformation, Neoplastic; Gene | 2021 |
CPS1: Looking at an ancient enzyme in a modern light.
Topics: Ammonia; Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; | 2020 |
Inborn Errors of Metabolism with Hyperammonemia: Urea Cycle Defects and Related Disorders.
Topics: Ammonia; Emergency Treatment; Humans; Hyperammonemia; Infant; Infant, Newborn; Urea; Urea Cycle Diso | 2018 |
Ammonia and autophagy: An emerging relationship with implications for disorders with hyperammonemia.
Topics: Ammonia; Animals; Autophagy; Glutamate-Ammonia Ligase; Glutamine; Homeostasis; Humans; Hyperammonemi | 2019 |
Progress and challenges in development of new therapies for urea cycle disorders.
Topics: Animals; Cell- and Tissue-Based Therapy; Combined Modality Therapy; Disease Management; Disease Susc | 2019 |
Arginase-1 deficiency.
Topics: Animals; Arginase; Disease Models, Animal; Genetic Association Studies; Humans; Hyperargininemia; Li | 2015 |
Ammonia toxicity and its prevention in inherited defects of the urea cycle.
Topics: Adult; Ammonia; Animals; Arginine; Genetic Therapy; Humans; Hyperammonemia; Ornithine Carbamoyltrans | 2009 |
Severe hyperammonaemia in adults not explained by liver disease.
Topics: Adult; Amino Acids; Ammonia; Biological Transport; Blood-Brain Barrier; Brain; Glutamine; Humans; Hy | 2012 |
3 trials available for urea and Inborn Urea Cycle Disorder
| Article | Year |
|---|---|
Human heterologous liver cells transiently improve hyperammonemia and ureagenesis in individuals with severe urea cycle disorders.
Topics: Ammonia; Biomarkers; Cell Transplantation; Europe; Female; Humans; Hyperammonemia; Infant; Infant, N | 2018 |
A randomized trial to study the comparative efficacy of phenylbutyrate and benzoate on nitrogen excretion and ureagenesis in healthy volunteers.
Topics: Adult; Benzoates; Female; Healthy Volunteers; Humans; Male; Middle Aged; Nitrogen; Phenylbutyrates; | 2018 |
Ammonia control in children with urea cycle disorders (UCDs); phase 2 comparison of sodium phenylbutyrate and glycerol phenylbutyrate.
Topics: Adolescent; Ammonia; Child; Dose-Response Relationship, Drug; Glycerol; Humans; Male; Phenylbutyrate | 2011 |
25 other studies available for urea and Inborn Urea Cycle Disorder
| Article | Year |
|---|---|
Perceptions and use of phenylbutyrate metabolite testing in urea cycle disorders: Results of a clinician survey and analysis of a centralized testing database.
Topics: Humans; Hyperammonemia; Nitrogen; Phenylbutyrates; Surveys and Questionnaires; Urea; Urea Cycle Diso | 2022 |
Glutaminase 2 knockdown reduces hyperammonemia and associated lethality of urea cycle disorder mouse model.
Topics: Ammonia; Animals; Disease Models, Animal; Glutaminase; Glutamine; Humans; Hyperammonemia; Liver; Mic | 2022 |
The diagnostic challenge of mild citrulline elevation at newborn screening.
Topics: Citrulline; Citrullinemia; Humans; Infant, Newborn; Neonatal Screening; Urea; Urea Cycle Disorders, | 2022 |
Evaluation of oxidative damage to biomolecules and inflammation in patients with urea cycle disorders.
Topics: Adolescent; Child; Child, Preschool; Cytokines; Humans; Infant; Infant, Newborn; Inflammation; Oxida | 2023 |
Taste-masked formulation of sodium phenylbutyrate (ACER-001) for the treatment of urea cycle disorders.
Topics: Humans; Hyperammonemia; Nitrogen; Phenylbutyrates; Powders; Rare Diseases; Taste; Urea; Urea Cycle D | 2023 |
Citrulline in the management of patients with urea cycle disorders.
Topics: Ammonia; Arginine; Citrulline; Drug-Related Side Effects and Adverse Reactions; Humans; Retrospectiv | 2023 |
Transition to glycerol phenylbutyrate for the management of urea cycle disorders: clinical experiences.
Topics: Ammonia; Glutamine; Humans; Quality of Life; Urea; Urea Cycle Disorders, Inborn | 2023 |
Glucose-dependent partitioning of arginine to the urea cycle protects β-cells from inflammation.
Topics: Adolescent; Adult; Aged; Arginine; Aspartic Acid; Cell Survival; Citric Acid Cycle; Female; Glucose; | 2020 |
A retrospective study of adult patients with noncirrhotic hyperammonemia.
Topics: Adult; Age of Onset; Aged; Ammonia; Female; Humans; Hyperammonemia; Male; Middle Aged; Retrospective | 2020 |
Gene delivery corrects N-acetylglutamate synthase deficiency and enables insights in the physiological impact of L-arginine activation of N-acetylglutamate synthase.
Topics: Amino-Acid N-Acetyltransferase; Animals; Arginine; Citrulline; Dependovirus; Disease Models, Animal; | 2021 |
Quantitation of phenylbutyrate metabolites by UPLC-MS/MS demonstrates inverse correlation of phenylacetate:phenylacetylglutamine ratio with plasma glutamine levels.
Topics: Ammonia; Argininosuccinic Aciduria; Chromatography, Liquid; Female; Glutamine; Glycerol; Humans; Lim | 2017 |
Untargeted metabolomic profiling reveals multiple pathway perturbations and new clinical biomarkers in urea cycle disorders.
Topics: Adolescent; Adult; Biomarkers; Child; Child, Preschool; Female; Humans; Male; Mass Spectrometry; Met | 2019 |
Transatlantic combined and comparative data analysis of 1095 patients with urea cycle disorders-A successful strategy for clinical research of rare diseases.
Topics: Cohort Studies; Data Analysis; Delayed Diagnosis; Europe; Female; Humans; Infant, Newborn; Male; Neo | 2019 |
The Therapeutic Hypothermia in Treatment of Hyperammonemic Encephalopathy due to Urea Cycle Disorders and Organic Acidemias.
Topics: Humans; Hyperammonemia; Hypothermia, Induced; Hypoxia-Ischemia, Brain; Infant, Newborn; Treatment Ou | 2019 |
Feasibility of adjunct therapeutic hypothermia treatment for hyperammonemia and encephalopathy due to urea cycle disorders and organic acidemias.
Topics: Adolescent; Child; Child, Preschool; Developmental Disabilities; Humans; Hyperammonemia; Hypothermia | 2013 |
The urea cycle disorders.
Topics: Brain; Diagnosis, Differential; Humans; Hyperammonemia; Ornithine Carbamoyltransferase Deficiency Di | 2014 |
Computational modeling to predict nitrogen balance during acute metabolic decompensation in patients with urea cycle disorders.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Essential; Diet; Dietary Proteins; Glucose; Human | 2016 |
In vivo monitoring of urea cycle activity with (13)C-acetate as a tracer of ureagenesis.
Topics: Administration, Oral; Adolescent; Adult; Carbon Isotopes; Child; Child, Preschool; Female; Humans; H | 2016 |
Initial presentation of a urea cycle disorder in adulthood: an under-recognised cause of severe neurological dysfunction.
Topics: Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Nervous System Diseases; Severity | 2015 |
Age at disease onset and peak ammonium level rather than interventional variables predict the neurological outcome in urea cycle disorders.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Ammonium Compounds; Argininosuccinate Synthase; Ch | 2016 |
A rare cause of postpartum coma: isolated hyperammonemia due to urea cycle disorder.
Topics: Ammonia; Coma; Female; Humans; Hyperammonemia; Ornithine Carbamoyltransferase Deficiency Disease; Po | 2016 |
Functional characterization of hepatocytes for cell transplantation: customized cell preparation for each receptor.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Biological Assay; Cell Separation; Cell Survival; Cell T | 2010 |
Creatine metabolism and the urea cycle.
Topics: Animals; Creatine; Glycine; Humans; Kidney; Urea; Urea Cycle Disorders, Inborn | 2010 |
Long-term outcome and intervention of urea cycle disorders in Japan.
Topics: Age of Onset; Ammonia; Female; Humans; Japan; Male; Ornithine Carbamoyltransferase Deficiency Diseas | 2012 |
Integrating cellular metabolism into a multiscale whole-body model.
Topics: Acetaminophen; Allopurinol; Ammonia; Chemical and Drug Induced Liver Injury; Computational Biology; | 2012 |