penicillamine has been researched along with Maple Syrup Urine Disease in 2 studies
Penicillamine: 3-Mercapto-D-valine. The most characteristic degradation product of the penicillin antibiotics. It is used as an antirheumatic and as a chelating agent in Wilson's disease.
penicillamine : An alpha-amino acid having the structure of valine substituted at the beta position with a sulfanyl group.
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)
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 2 (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 |
|---|---|
| MILNE, MD | 1 |
| Schaub, J | 1 |
| Bremer, HJ | 1 |
1 review available for penicillamine and Maple Syrup Urine Disease
| Article | Year |
|---|---|
[Diet therapy of inborn errors of metabolism].
Topics: Adult; Carbohydrate Metabolism, Inborn Errors; Child; Cystinosis; Diet Therapy; Dietary Carbohydrate | 1970 |
1 other study available for penicillamine and Maple Syrup Urine Disease
| Article | Year |
|---|---|
DISORDERS OF AMINO-ACID TRANSPORT.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Cystinosis; Cystinuria; Dog Diseases; Dogs; Kidney Di | 1964 |