ammonium hydroxide has been researched along with Propionic Acidemia in 14 studies
azane : Saturated acyclic nitrogen hydrides having the general formula NnHn+2.
Propionic Acidemia: Autosomal recessive metabolic disorder caused by mutations in PROPIONYL-COA CARBOXYLASE genes that result in dysfunction of branch chain amino acids and of the metabolism of certain fatty acids. Neonatal clinical onset is characterized by severe metabolic acidemia accompanied by hyperammonemia, HYPERGLYCEMIA, lethargy, vomiting, HYPOTONIA; and HEPATOMEGALY. Survivors of the neonatal onset propionic acidemia often show developmental retardation, and intolerance to dietary proteins. Late-onset form of the disease shows mild mental and/or developmental retardation, sometimes without metabolic acidemia.
| Excerpt | Relevance | Reference |
|---|---|---|
| "The objective of this study was to determine whether N-carbamylglutamate (NCG) reduces plasma levels of ammonia and glutamine and increases the rate of ureagenesis in patients with propionic acidemia (PA)." | 9.14 | N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia. ( Ah Mew, N; Daikhin, Y; McCarter, R; Nissim, I; Tuchman, M; Yudkoff, M, 2010) |
| "In propionic aciduria and methylmalonic aciduria, hyperammonemia as a symptom of metabolic decompensation is one of the major clinical problems." | 7.76 | N-carbamylglutamate in emergency management of hyperammonemia in neonatal acute onset propionic and methylmalonic aciduria. ( Donati, MA; Filippi, L; Fiorini, P; Gozzini, E; la Marca, G; Malvagia, S, 2010) |
| "5mEq/kg per day), or ornithine α-ketoglutarate (400mg/kg per day) (anaplerotic agents that could fill up the citric acid cycle) would affect plasma levels of glutamine and ammonia, the urinary excretion of Krebs cycle intermediates, and the clinical outcome in 3 patients with propionic acidemia." | 5.24 | Anaplerotic therapy in propionic acidemia. ( Bailey, C; Cantor, NL; Ernst, SL; Gappmaier, E; Longo, N; Pasquali, M; Price, LB, 2017) |
| "The objective of this study was to determine whether N-carbamylglutamate (NCG) reduces plasma levels of ammonia and glutamine and increases the rate of ureagenesis in patients with propionic acidemia (PA)." | 5.14 | N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia. ( Ah Mew, N; Daikhin, Y; McCarter, R; Nissim, I; Tuchman, M; Yudkoff, M, 2010) |
| "Life-threatening hyperammonemia occurs in both inherited and acquired liver diseases affecting ureagenesis, the main pathway for detoxification of neurotoxic ammonia in mammals." | 4.12 | O-GlcNAcylation enhances CPS1 catalytic efficiency for ammonia and promotes ureagenesis. ( Arena, P; Attanasio, S; Boffa, I; Brunetti-Pierri, N; Cuomo, P; D'Alessio, AM; De Angelis, A; Desviat, LR; Ferenbach, AT; Häberle, J; Lipshutz, GS; Makris, G; Martínez-Pizarro, A; Motta, A; Nitzahn, M; Nusco, E; Paris, D; Pravata, VM; Richard, E; Rüfenacht, V; Soria, LR; van Aalten, DMF, 2022) |
| "In propionic aciduria and methylmalonic aciduria, hyperammonemia as a symptom of metabolic decompensation is one of the major clinical problems." | 3.76 | N-carbamylglutamate in emergency management of hyperammonemia in neonatal acute onset propionic and methylmalonic aciduria. ( Donati, MA; Filippi, L; Fiorini, P; Gozzini, E; la Marca, G; Malvagia, S, 2010) |
| "Isovaleric aciduria (IVA), propionic aciduria (PA) and methylmalonic aciduria (MMA) are inherited organic acidurias (OAs) in which impaired organic acid metabolism induces hyperammonaemia arising partly from secondary deficiency of N-acetylglutamate (NAG) synthase." | 2.82 | Carglumic acid enhances rapid ammonia detoxification in classical organic acidurias with a favourable risk-benefit profile: a retrospective observational study. ( Baruteau, J; Cano, A; Chakrapani, A; Coker, M; Couce, ML; Del Toro, M; Delgado, MB; Donati, MA; Garcia-Cazorla, A; Gil-Ortega, D; Gomez-de Quero, P; Guffon, N; Hofstede, FC; Kalkan-Ucar, S; Lama-More, R; Le Mouhaer, J; Martinez-Pardo Casanova, M; Molina, A; Papadia, F; Pichard, S; Plisson, C; Rosello, P; Valayannopoulos, V, 2016) |
| "Propionic acidemia is an inborn error of metabolism caused by a deficiency in the mitochondrial enzyme propionyl-CoA carboxylase that converts the propionyl CoA to methyl malonyl CoA." | 1.72 | Interorgan amino acid interchange in propionic acidemia: the missing key to understanding its physiopathology. ( Arrieta, F; Belanger-Quintana, A; Del Valle, M; Fernandez-Felix, BM; Garcia, F; Martinez-Pardo, M; Ruiz-Sala, P; Stanescu, S, 2022) |
| "Carglumic acid was well tolerated with no side effects noted." | 1.39 | Use of carglumic acid in the treatment of hyperammonaemia during metabolic decompensation of patients with propionic acidaemia. ( Abacan, M; Boneh, A, 2013) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 1 (7.14) | 29.6817 |
| 2010's | 11 (78.57) | 24.3611 |
| 2020's | 2 (14.29) | 2.80 |
| Authors | Studies |
|---|---|
| Stanescu, S | 1 |
| Belanger-Quintana, A | 1 |
| Fernandez-Felix, BM | 1 |
| Ruiz-Sala, P | 1 |
| Del Valle, M | 1 |
| Garcia, F | 1 |
| Arrieta, F | 1 |
| Martinez-Pardo, M | 1 |
| Soria, LR | 1 |
| Makris, G | 1 |
| D'Alessio, AM | 1 |
| De Angelis, A | 1 |
| Boffa, I | 1 |
| Pravata, VM | 1 |
| Rüfenacht, V | 1 |
| Attanasio, S | 1 |
| Nusco, E | 1 |
| Arena, P | 1 |
| Ferenbach, AT | 1 |
| Paris, D | 1 |
| Cuomo, P | 1 |
| Motta, A | 1 |
| Nitzahn, M | 1 |
| Lipshutz, GS | 1 |
| Martínez-Pizarro, A | 1 |
| Richard, E | 1 |
| Desviat, LR | 1 |
| Häberle, J | 2 |
| van Aalten, DMF | 1 |
| Brunetti-Pierri, N | 1 |
| Longo, N | 2 |
| Price, LB | 1 |
| Gappmaier, E | 1 |
| Cantor, NL | 1 |
| Ernst, SL | 1 |
| Bailey, C | 1 |
| Pasquali, M | 1 |
| Chakrapani, A | 2 |
| Ah Mew, N | 2 |
| Item, CB | 1 |
| Schanzer, A | 1 |
| Metz, T | 1 |
| Greber-Platzer, S | 1 |
| Lischka, J | 1 |
| Abacan, M | 1 |
| Boneh, A | 1 |
| Zwickler, T | 1 |
| Riderer, A | 1 |
| Haege, G | 1 |
| Hoffmann, GF | 1 |
| Kölker, S | 1 |
| Burgard, P | 1 |
| Chapman, KA | 1 |
| Collado, MS | 1 |
| Figler, RA | 1 |
| Hoang, SA | 1 |
| Armstrong, AJ | 1 |
| Cui, W | 1 |
| Purdy, M | 1 |
| Simmers, MB | 1 |
| Yazigi, NA | 1 |
| Summar, ML | 1 |
| Wamhoff, BR | 1 |
| Dash, A | 1 |
| Valayannopoulos, V | 1 |
| Baruteau, J | 1 |
| Delgado, MB | 1 |
| Cano, A | 1 |
| Couce, ML | 1 |
| Del Toro, M | 1 |
| Donati, MA | 2 |
| Garcia-Cazorla, A | 1 |
| Gil-Ortega, D | 1 |
| Gomez-de Quero, P | 1 |
| Guffon, N | 1 |
| Hofstede, FC | 1 |
| Kalkan-Ucar, S | 1 |
| Coker, M | 1 |
| Lama-More, R | 1 |
| Martinez-Pardo Casanova, M | 1 |
| Molina, A | 1 |
| Pichard, S | 1 |
| Papadia, F | 1 |
| Rosello, P | 1 |
| Plisson, C | 1 |
| Le Mouhaer, J | 1 |
| Schwahn, BC | 1 |
| Pieterse, L | 1 |
| Bisset, WM | 1 |
| Galloway, PG | 1 |
| Robinson, PH | 1 |
| Filippi, L | 1 |
| Gozzini, E | 1 |
| Fiorini, P | 1 |
| Malvagia, S | 1 |
| la Marca, G | 1 |
| McCarter, R | 1 |
| Daikhin, Y | 1 |
| Nissim, I | 1 |
| Yudkoff, M | 1 |
| Tuchman, M | 1 |
| Scholl-Bürgi, S | 1 |
| Sass, JO | 1 |
| Zschocke, J | 1 |
| Karall, D | 1 |
| Jones, S | 1 |
| Reed, CA | 1 |
| Vijay, S | 1 |
| Walter, JH | 1 |
| Morris, AA | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Safety & Efficacy of Investigational Products: Ornithine Alpha-ketoglutarate, Glutamine, or Disodium Citrate on Hyperammonemia in Propionic Acidemia.[NCT00645879] | Phase 1 | 3 participants (Actual) | Interventional | 2008-07-31 | Completed | ||
| Increasing Ureagenesis in Inborn Errors of Metabolism With N-Carbamylglutamate[NCT01341379] | Phase 2 | 0 participants (Actual) | Interventional | 2010-12-31 | Withdrawn (stopped due to Lack of funding) | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
2 reviews available for ammonium hydroxide and Propionic Acidemia
| Article | Year |
|---|---|
Hyperammonaemia in classic organic acidaemias: a review of the literature and two case histories.
Topics: Ammonia; Glucose; Glutamates; Humans; Hyperammonemia; Propionic Acidemia | 2018 |
Amino acid metabolism in patients with propionic acidaemia.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Ammonia; Citric Acid Cycle; Diet; | 2012 |
3 trials available for ammonium hydroxide and Propionic Acidemia
| Article | Year |
|---|---|
Anaplerotic therapy in propionic acidemia.
Topics: Amino Acids; Ammonia; Carbon-Carbon Ligases; Child; Child, Preschool; Citrates; Citric Acid; Citric | 2017 |
Carglumic acid enhances rapid ammonia detoxification in classical organic acidurias with a favourable risk-benefit profile: a retrospective observational study.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Female; Glutamates; Humans; Hyperammonemia; Infant, N | 2016 |
N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia.
Topics: Administration, Oral; Adolescent; Ammonia; Blood Chemical Analysis; Child; Child, Preschool; Confide | 2010 |
9 other studies available for ammonium hydroxide and Propionic Acidemia
| Article | Year |
|---|---|
Interorgan amino acid interchange in propionic acidemia: the missing key to understanding its physiopathology.
Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Glutamine; Humans; Hyperammonem | 2022 |
O-GlcNAcylation enhances CPS1 catalytic efficiency for ammonia and promotes ureagenesis.
Topics: Acetylglucosamine; Ammonia; Animals; Biocatalysis; Carbamoyl-Phosphate Synthase (Ammonia); Disease M | 2022 |
Demethylation of the hypoxia induction factor 1 binding site of GPX3 at excess blood ammonia in propionic acidemia.
Topics: Ammonia; Binding Sites; Demethylation; DNA Methylation; Glutathione Peroxidase; Humans; Hyperammonem | 2019 |
Use of carglumic acid in the treatment of hyperammonaemia during metabolic decompensation of patients with propionic acidaemia.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Carnitine; Child, Preschool; Female; Glutamates; Huma | 2013 |
Usefulness of biochemical parameters in decision-making on the start of emergency treatment in patients with propionic acidemia.
Topics: Acid-Base Equilibrium; Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Appetite; C | 2014 |
Recapitulation of metabolic defects in a model of propionic acidemia using patient-derived primary hepatocytes.
Topics: Actins; Amino Acids, Branched-Chain; Ammonia; Carbon-Carbon Ligases; Cells, Cultured; Child; Fibrobl | 2016 |
Biochemical efficacy of N-carbamylglutamate in neonatal severe hyperammonaemia due to propionic acidaemia.
Topics: Ammonia; Dose-Response Relationship, Drug; Female; Follow-Up Studies; Glutamates; Humans; Hyperammon | 2010 |
N-carbamylglutamate in emergency management of hyperammonemia in neonatal acute onset propionic and methylmalonic aciduria.
Topics: Acute Disease; Amino Acid Metabolism, Inborn Errors; Ammonia; Emergency Medical Services; Female; Gl | 2010 |
N-carbamylglutamate for neonatal hyperammonaemia in propionic acidaemia.
Topics: Ammonia; Biomarkers; Female; Glutamates; Humans; Hyperammonemia; Infant, Newborn; Intubation, Gastro | 2008 |