Page last updated: 2024-11-07

penicillamine and Maple Syrup Urine Disease

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)

Research

Studies (2)

TimeframeStudies, this research(%)All Research%
pre-19902 (100.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
MILNE, MD1
Schaub, J1
Bremer, HJ1

Reviews

1 review available for penicillamine and Maple Syrup Urine Disease

ArticleYear
[Diet therapy of inborn errors of metabolism].
    Medizinische Klinik, 1970, Aug-01, Volume: 65, Issue:32

    Topics: Adult; Carbohydrate Metabolism, Inborn Errors; Child; Cystinosis; Diet Therapy; Dietary Carbohydrate

1970

Other Studies

1 other study available for penicillamine and Maple Syrup Urine Disease

ArticleYear
DISORDERS OF AMINO-ACID TRANSPORT.
    British medical journal, 1964, Feb-08, Volume: 1, Issue:5379

    Topics: Amino Acid Metabolism, Inborn Errors; Animals; Cystinosis; Cystinuria; Dog Diseases; Dogs; Kidney Di

1964