urea has been researched along with Carbamoyl Phosphate Synthase (Ammonia) Deficiency Disease in 15 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 |
|---|---|---|
| "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) |
| " In contrast, hyperargininemia found in patients with arginase 1 deficiency is associated with pyramidal tract findings and spasticity, without significant hyperammonemia." | 4.82 | Clinical consequences of urea cycle enzyme deficiencies and potential links to arginine and nitric oxide metabolism. ( Brunetti-Pierri, N; Carter, S; Garlick, P; Jahoor, F; Kleppe, S; Lee, B; Marini, J; O'Brien, W; Scaglia, F, 2004) |
| "Most often, urea cycle disorders have been described as acute onset hyperammonemia in the newborn period; however, there is a growing awareness that urea cycle disorders can present at almost any age, frequently in the critical care setting." | 4.82 | Unmasked adult-onset urea cycle disorders in the critical care setting. ( Barr, F; Christman, BW; Dawling, S; Lee, B; Rhead, WJ; Singh, RH; Smith, W; Sniderman King, L; Summar, ML, 2005) |
| "One patient with OTC deficiency has had a liver transplant." | 1.35 | Hereditary urea cycle diseases in Finland. ( Keskinen, P; Salo, M; Siitonen, A, 2008) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 1 (6.67) | 18.2507 |
| 2000's | 7 (46.67) | 29.6817 |
| 2010's | 3 (20.00) | 24.3611 |
| 2020's | 4 (26.67) | 2.80 |
| Authors | Studies |
|---|---|
| Zhang, L | 1 |
| Zou, Y | 1 |
| Lu, Y | 1 |
| Li, Z | 1 |
| Gao, F | 1 |
| Zhang, S | 3 |
| Hu, Y | 3 |
| Wu, Z | 3 |
| Zhou, X | 3 |
| Wu, T | 3 |
| Li, P | 3 |
| Lian, Q | 3 |
| Xu, S | 3 |
| Gu, J | 3 |
| Chen, L | 3 |
| Wu, G | 3 |
| Zhang, T | 3 |
| Tang, J | 3 |
| Xue, J | 3 |
| Nitzahn, M | 2 |
| Allegri, G | 1 |
| Khoja, S | 1 |
| Truong, B | 1 |
| Makris, G | 1 |
| Häberle, J | 2 |
| Lipshutz, GS | 2 |
| Ah Mew, N | 1 |
| McCarter, R | 1 |
| Daikhin, Y | 1 |
| Lichter-Konecki, U | 1 |
| Nissim, I | 1 |
| Yudkoff, M | 1 |
| Tuchman, M | 1 |
| Díez-Fernández, C | 2 |
| Gallego, J | 1 |
| Cervera, J | 2 |
| Rubio, V | 2 |
| de Cima, S | 1 |
| Polo, LM | 1 |
| Martínez, AI | 1 |
| Fita, I | 1 |
| Keskinen, P | 1 |
| Siitonen, A | 1 |
| Salo, M | 1 |
| Enns, GM | 2 |
| Scaglia, F | 1 |
| Brunetti-Pierri, N | 1 |
| Kleppe, S | 1 |
| Marini, J | 1 |
| Carter, S | 2 |
| Garlick, P | 1 |
| Jahoor, F | 1 |
| O'Brien, W | 1 |
| Lee, B | 4 |
| McBride, KL | 1 |
| Miller, G | 1 |
| Karpen, S | 1 |
| Goss, J | 1 |
| Summar, ML | 2 |
| Barr, F | 1 |
| Dawling, S | 1 |
| Smith, W | 2 |
| Singh, RH | 2 |
| Rhead, WJ | 3 |
| Sniderman King, L | 2 |
| Christman, BW | 1 |
| Berry, SA | 1 |
| Berry, GT | 1 |
| Brusilow, SW | 1 |
| Hamosh, A | 1 |
| Saudubray, JM | 1 |
| Touati, G | 1 |
| Delonlay, P | 1 |
| Jouvet, P | 1 |
| Narcy, C | 1 |
| Laurent, J | 1 |
| Rabier, D | 1 |
| Kamoun, P | 1 |
| Jan, D | 1 |
| Revillon, Y | 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 |
6 reviews available for urea and Carbamoyl Phosphate Synthase (Ammonia) Deficiency Disease
| Article | Year |
|---|---|
Unraveling the therapeutic potential of carbamoyl phosphate synthetase 1 (CPS1) in human diseases.
Topics: Ammonia; Animals; Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency | 2023 |
CPS1: Looking at an ancient enzyme in a modern light.
Topics: Ammonia; Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; | 2020 |
Neurologic damage and neurocognitive dysfunction in urea cycle disorders.
Topics: Brain Edema; Carbamoyl-Phosphate Synthase I Deficiency Disease; Cognition Disorders; Glutamine; Huma | 2008 |
Clinical consequences of urea cycle enzyme deficiencies and potential links to arginine and nitric oxide metabolism.
Topics: Animals; Arginine; Argininosuccinic Aciduria; Carbamoyl-Phosphate Synthase I Deficiency Disease; Enz | 2004 |
Unmasked adult-onset urea cycle disorders in the critical care setting.
Topics: Adult; Age of Onset; Brain Diseases, Metabolic, Inborn; Carbamoyl-Phosphate Synthase I Deficiency Di | 2005 |
Genetic counseling issues in urea cycle disorders.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Carbamoyl-Phosphate Synthase I Deficiency Disease; Chro | 2005 |
2 trials available for urea and Carbamoyl Phosphate Synthase (Ammonia) Deficiency Disease
| Article | Year |
|---|---|
Augmenting ureagenesis in patients with partial carbamyl phosphate synthetase 1 deficiency with N-carbamyl-L-glutamate.
Topics: Adolescent; Adult; Ammonia; Carbamoyl-Phosphate Synthase I Deficiency Disease; Child; Child, Prescho | 2014 |
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 |
7 other studies available for urea and Carbamoyl Phosphate Synthase (Ammonia) Deficiency Disease
| Article | Year |
|---|---|
Deficiency of Carbamoyl Phosphate Synthetase 1 Engenders Radioresistance in Hepatocellular Carcinoma via Deubiquitinating c-Myc.
Topics: Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; Carbamyl | 2023 |
Deficiency of Carbamoyl Phosphate Synthetase 1 Engenders Radioresistance in Hepatocellular Carcinoma via Deubiquitinating c-Myc.
Topics: Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; Carbamyl | 2023 |
Deficiency of Carbamoyl Phosphate Synthetase 1 Engenders Radioresistance in Hepatocellular Carcinoma via Deubiquitinating c-Myc.
Topics: Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; Carbamyl | 2023 |
Deficiency of Carbamoyl Phosphate Synthetase 1 Engenders Radioresistance in Hepatocellular Carcinoma via Deubiquitinating c-Myc.
Topics: Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; Carbamyl | 2023 |
Deficiency of Carbamoyl Phosphate Synthetase 1 Engenders Radioresistance in Hepatocellular Carcinoma via Deubiquitinating c-Myc.
Topics: Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; Carbamyl | 2023 |
Deficiency of Carbamoyl Phosphate Synthetase 1 Engenders Radioresistance in Hepatocellular Carcinoma via Deubiquitinating c-Myc.
Topics: Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; Carbamyl | 2023 |
Deficiency of Carbamoyl Phosphate Synthetase 1 Engenders Radioresistance in Hepatocellular Carcinoma via Deubiquitinating c-Myc.
Topics: Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; Carbamyl | 2023 |
Deficiency of Carbamoyl Phosphate Synthetase 1 Engenders Radioresistance in Hepatocellular Carcinoma via Deubiquitinating c-Myc.
Topics: Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; Carbamyl | 2023 |
Deficiency of Carbamoyl Phosphate Synthetase 1 Engenders Radioresistance in Hepatocellular Carcinoma via Deubiquitinating c-Myc.
Topics: Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; Carbamyl | 2023 |
Split AAV-Mediated Gene Therapy Restores Ureagenesis in a Murine Model of Carbamoyl Phosphate Synthetase 1 Deficiency.
Topics: Ammonia; Animals; Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency | 2020 |
The Study of Carbamoyl Phosphate Synthetase 1 Deficiency Sheds Light on the Mechanism for Switching On/Off the Urea Cycle.
Topics: Ammonia; Carbamoyl-Phosphate Synthase (Ammonia); Carbamoyl-Phosphate Synthase I Deficiency Disease; | 2015 |
Structure of human carbamoyl phosphate synthetase: deciphering the on/off switch of human ureagenesis.
Topics: Amino Acid Motifs; Ammonia; Animals; Baculoviridae; Carbamoyl-Phosphate Synthase (Ammonia); Carbamoy | 2015 |
Hereditary urea cycle diseases in Finland.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Argininosuccinic Aciduria; Carbamoyl-Phosph | 2008 |
Developmental outcomes with early orthotopic liver transplantation for infants with neonatal-onset urea cycle defects and a female patient with late-onset ornithine transcarbamylase deficiency.
Topics: Ammonia; Carbamoyl-Phosphate Synthase I Deficiency Disease; Child Development; Child, Preschool; Com | 2004 |
Liver transplantation in urea cycle disorders.
Topics: Amino Acid Metabolism, Inborn Errors; Carbamoyl-Phosphate Synthase I Deficiency Disease; Child; Chil | 1999 |