lactic acid has been researched along with Propionic Acidemia in 7 studies
Lactic Acid: A normal intermediate in the fermentation (oxidation, metabolism) of sugar. The concentrated form is used internally to prevent gastrointestinal fermentation. (From Stedman, 26th ed)
2-hydroxypropanoic acid : A 2-hydroxy monocarboxylic acid that is propanoic acid in which one of the alpha-hydrogens is replaced by a hydroxy group.
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 |
|---|---|---|
| "Increased propionylcarnitine levels in newborn screening are indicative for a group of potentially severe disorders including propionic acidemia (PA), methylmalonic acidemias and combined remethylation disorders (MMACBL)." | 7.85 | Simultaneous determination of 3-hydroxypropionic acid, methylmalonic acid and methylcitric acid in dried blood spots: Second-tier LC-MS/MS assay for newborn screening of propionic acidemia, methylmalonic acidemias and combined remethylation disorders. ( Baráth, Á; Baumgartner, MR; Fingerhut, R; Gramer, G; Hoffmann, GF; Klinke, G; Kölker, S; Monostori, P; Okun, JG; Richter, S, 2017) |
| "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) |
| "Propionate, 3-hydroxypropionate (3HP), methylcitrate, related compounds, and ammonium accumulate in body fluids of patients with disorders of propionyl-CoA metabolism, such as propionic acidemia." | 3.85 | Inter-relations between 3-hydroxypropionate and propionate metabolism in rat liver: relevance to disorders of propionyl-CoA metabolism. ( Brunengraber, H; Chapman, KA; Cline, GW; Han, Y; Hess, JP; Tochtrop, GP; Wilson, KA; Zhang, GF; Zhang, M, 2017) |
| "Increased propionylcarnitine levels in newborn screening are indicative for a group of potentially severe disorders including propionic acidemia (PA), methylmalonic acidemias and combined remethylation disorders (MMACBL)." | 3.85 | Simultaneous determination of 3-hydroxypropionic acid, methylmalonic acid and methylcitric acid in dried blood spots: Second-tier LC-MS/MS assay for newborn screening of propionic acidemia, methylmalonic acidemias and combined remethylation disorders. ( Baráth, Á; Baumgartner, MR; Fingerhut, R; Gramer, G; Hoffmann, GF; Klinke, G; Kölker, S; Monostori, P; Okun, JG; Richter, S, 2017) |
| "Propionic acidemia is the result of a deficiency in propionyl-CoA carboxylase activity." | 1.48 | Autism spectrum disorders in propionic acidemia patients. ( Arnoux, JB; Barbier, V; Bonnefont, JP; Brassier, A; Canouï, P; de la Bâtie, CD; De Lonlay, P; Gobin, S; Guemann, AS; Habarou, F; Lacaille, F; Ottolenghi, C; Ouss, L; Pontoizeau, C; Roda, C; Valayannopoulos, V, 2018) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 1 (14.29) | 29.6817 |
| 2010's | 6 (85.71) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Wilson, KA | 1 |
| Han, Y | 1 |
| Zhang, M | 1 |
| Hess, JP | 1 |
| Chapman, KA | 2 |
| Cline, GW | 1 |
| Tochtrop, GP | 1 |
| Brunengraber, H | 1 |
| Zhang, GF | 1 |
| Longo, N | 1 |
| Price, LB | 1 |
| Gappmaier, E | 1 |
| Cantor, NL | 1 |
| Ernst, SL | 1 |
| Bailey, C | 1 |
| Pasquali, M | 1 |
| de la Bâtie, CD | 1 |
| Barbier, V | 2 |
| Roda, C | 1 |
| Brassier, A | 1 |
| Arnoux, JB | 2 |
| Valayannopoulos, V | 2 |
| Guemann, AS | 1 |
| Pontoizeau, C | 1 |
| Gobin, S | 1 |
| Habarou, F | 2 |
| Lacaille, F | 1 |
| Bonnefont, JP | 2 |
| Canouï, P | 1 |
| Ottolenghi, C | 2 |
| De Lonlay, P | 2 |
| Ouss, L | 1 |
| Monostori, P | 1 |
| Klinke, G | 1 |
| Richter, S | 1 |
| Baráth, Á | 1 |
| Fingerhut, R | 1 |
| Baumgartner, MR | 1 |
| Kölker, S | 1 |
| Hoffmann, GF | 1 |
| Gramer, G | 1 |
| Okun, JG | 1 |
| Wongkittichote, P | 1 |
| Ah Mew, N | 1 |
| Nizon, M | 1 |
| Desguerre, I | 1 |
| Boddaert, N | 1 |
| Acquaviva, C | 1 |
| Benoist, JF | 1 |
| Rabier, D | 1 |
| Touati, G | 1 |
| Scholl-Bürgi, S | 1 |
| Haberlandt, E | 1 |
| Gotwald, T | 1 |
| Albrecht, U | 1 |
| Baumgartner Sigl, S | 1 |
| Rauchenzauner, M | 1 |
| Rostásy, K | 1 |
| Karall, D | 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 | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
1 review available for lactic acid and Propionic Acidemia
| Article | Year |
|---|---|
Propionyl-CoA carboxylase - A review.
Topics: Carbon-Carbon Ligases; Humans; Hyperammonemia; Lactic Acid; Methylmalonyl-CoA Decarboxylase; Mutatio | 2017 |
1 trial available for lactic acid 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 |
5 other studies available for lactic acid and Propionic Acidemia
| Article | Year |
|---|---|
Inter-relations between 3-hydroxypropionate and propionate metabolism in rat liver: relevance to disorders of propionyl-CoA metabolism.
Topics: Acyl Coenzyme A; Ammonium Compounds; Animals; Carbon Isotopes; Citrates; Citric Acid Cycle; Lactic A | 2017 |
Autism spectrum disorders in propionic acidemia patients.
Topics: Adolescent; Adult; Autism Spectrum Disorder; Child; Child, Preschool; Female; Humans; Intellectual D | 2018 |
Simultaneous determination of 3-hydroxypropionic acid, methylmalonic acid and methylcitric acid in dried blood spots: Second-tier LC-MS/MS assay for newborn screening of propionic acidemia, methylmalonic acidemias and combined remethylation disorders.
Topics: Amino Acid Metabolism, Inborn Errors; Chromatography, Liquid; Citrates; Dried Blood Spot Testing; Fe | 2017 |
Long-term neurological outcome of a cohort of 80 patients with classical organic acidurias.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Female; Humans; La | 2013 |
Stroke-like episodes in propionic acidemia caused by central focal metabolic decompensation.
Topics: Amino Acids; Basal Ganglia; Cerebral Cortex; Child; Electroencephalography; Female; Functional Later | 2009 |