trimethylamine has been researched along with Maple Syrup Urine Disease in 1 studies
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 |
|---|---|---|
| "The profiles of urinary volatiles from patients with phenylketonuria, maple syrup urine disease, isovaleric acidemia, or trimethylaminuria (fish-odor syndrome) were in each case vastly different from the normal urinary volatiles profile." | 3.66 | Profiles of urinary volatiles from metabolic disorders characterized by unusual odors. ( Burke, DG; Danks, D; Halpern, B; Malegan, D; McCairns, E; Schlesinger, P; Wilken, B, 1983) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 1 (100.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Burke, DG | 1 |
| Halpern, B | 1 |
| Malegan, D | 1 |
| McCairns, E | 1 |
| Danks, D | 1 |
| Schlesinger, P | 1 |
| Wilken, B | 1 |
1 other study available for trimethylamine and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Profiles of urinary volatiles from metabolic disorders characterized by unusual odors.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Chromatography, Gas; Flame Ionization; Hemi | 1983 |