citric acid, anhydrous has been researched along with Maple Syrup Urine Disease in 1 studies
Citric Acid: A key intermediate in metabolism. It is an acid compound found in citrus fruits. The salts of citric acid (citrates) can be used as anticoagulants due to their calcium chelating ability.
citric acid : A tricarboxylic acid that is propane-1,2,3-tricarboxylic acid bearing a hydroxy substituent at position 2. It is an important metabolite in the pathway of all aerobic organisms.
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 |
|---|---|---|
| "We report on 2 women with organic acidemias, one with classical maple syrup urine disease and another with mild propionic acidemia in which protein restricted diets and carnitine supplementation were successfully employed to manage pregnancies." | 7.68 | Case reports of successful pregnancy in women with maple syrup urine disease and propionic acidemia. ( Barness, LA; Davidson, SR; Harding, CO; Van Calcar, SC; Wolff, JA, 1992) |
| "We report on 2 women with organic acidemias, one with classical maple syrup urine disease and another with mild propionic acidemia in which protein restricted diets and carnitine supplementation were successfully employed to manage pregnancies." | 3.68 | Case reports of successful pregnancy in women with maple syrup urine disease and propionic acidemia. ( Barness, LA; Davidson, SR; Harding, CO; Van Calcar, SC; Wolff, JA, 1992) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 1 (100.00) | 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 |
|---|---|
| Van Calcar, SC | 1 |
| Harding, CO | 1 |
| Davidson, SR | 1 |
| Barness, LA | 1 |
| Wolff, JA | 1 |
1 other study available for citric acid, anhydrous and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Case reports of successful pregnancy in women with maple syrup urine disease and propionic acidemia.
Topics: Adult; Amino Acids; Carbon-Carbon Ligases; Carnitine; Citrates; Citric Acid; Female; Fetal Growth Re | 1992 |