hydrogen carbonate has been researched along with Maple Syrup Urine Disease in 2 studies
Bicarbonates: Inorganic salts that contain the -HCO3 radical. They are an important factor in determining the pH of the blood and the concentration of bicarbonate ions is regulated by the kidney. Levels in the blood are an index of the alkali reserve or buffering capacity.
hydrogencarbonate : The carbon oxoanion resulting from the removal of a proton from carbonic acid.
Maple Syrup Urine Disease: An autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids (AMINO ACIDS, BRANCHED-CHAIN). These metabolites accumulate in body fluids and render a maple syrup odor. The disease is divided into classic, intermediate, intermittent, and thiamine responsive subtypes. The classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia. The intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting. (From Adams et al., Principles of Neurology, 6th ed, p936)
| Excerpt | Relevance | Reference |
|---|---|---|
| "Low-dose continuous infusions of [2H5]phenylalanine, [1-13C]propionate, and [1-13C]leucine were used to quantitate phenylalanine hydroxylation in phenylketonuria (PKU, four subjects), propionate oxidation in methylmalonic acidaemia (MMA, four subjects), and propionic acidaemia (PA, four subjects) and leucine oxidation in maple syrup urine disease (MSUD, four subjects)." | 3.68 | In vivo enzyme activity in inborn errors of metabolism. ( Halliday, D; Leonard, JV; Thompson, GN; Walter, JH, 1990) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 1 (50.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 1 (50.00) | 2.80 |
| Authors | Studies |
|---|---|
| Baertling, F | 1 |
| Wagner, M | 1 |
| Brunet, T | 1 |
| Sabir, H | 1 |
| Wieczorek, D | 1 |
| Meitinger, T | 1 |
| Meissner, T | 1 |
| Distelmaier, F | 1 |
| Thompson, GN | 1 |
| Walter, JH | 1 |
| Leonard, JV | 1 |
| Halliday, D | 1 |
2 other studies available for hydrogen carbonate and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Fatal metabolic decompensation in carbonic anhydrase VA deficiency despite early treatment and control of hyperammonemia.
Topics: Bicarbonates; Carbonic Anhydrases; Humans; Hyperammonemia; Liver; Maple Syrup Urine Disease | 2020 |
In vivo enzyme activity in inborn errors of metabolism.
Topics: Adult; Bicarbonates; Carbon Isotopes; Child; Child, Preschool; Deuterium; Female; Humans; Hydroxylat | 1990 |