urea has been researched along with Maple Syrup Urine Disease in 12 studies
pseudourea: clinical use; structure
isourea : A carboximidic acid that is the imidic acid tautomer of urea, H2NC(=NH)OH, and its hydrocarbyl derivatives.
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 | 9 (75.00) | 18.7374 |
| 1990's | 2 (16.67) | 18.2507 |
| 2000's | 1 (8.33) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Barschak, AG | 1 |
| Marchesan, C | 1 |
| Sitta, A | 1 |
| Deon, M | 1 |
| Giugliani, R | 1 |
| Wajner, M | 1 |
| Vargas, CR | 1 |
| Wellner, D | 1 |
| Meister, A | 1 |
| Morris, AA | 1 |
| Leonard, JV | 2 |
| Snyderman, SE | 1 |
| Omenn, GS | 1 |
| Dixon, MA | 1 |
| Frimpter, GW | 1 |
| Martin, JJ | 2 |
| Schlote, W | 2 |
| Hooft, C | 1 |
| Carton, D | 1 |
| Plöchl, E | 1 |
| Menkes, JH | 1 |
7 reviews available for urea and Maple Syrup Urine Disease
| Article | Year |
|---|---|
A survey of inborn errors of amino acid metabolism and transport in man.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Amino Acids, Branched-Chain; Biological Transport | 1981 |
Early recognition of metabolic decompensation.
Topics: Biomarkers; Brain Diseases, Metabolic; Child; Fatty Acids; Glycogen Storage Disease Type I; Humans; | 1997 |
The dietary therapy of inherited metabolic disease.
Topics: Alkaptonuria; Amino Acid Metabolism, Inborn Errors; Carbohydrate Metabolism, Inborn Errors; Child; C | 1975 |
Inborn errors of metabolism: clues to understanding human behavioral disorders.
Topics: Adrenal Hyperplasia, Congenital; Brain; Galactosemias; Glycine; Hepatolenticular Degeneration; Heter | 1976 |
Intercurrent illness in inborn errors of intermediary metabolism.
Topics: Acidosis; Child; Child, Preschool; Emergencies; Fatty Acids; Health Education; Humans; Infant; Infan | 1992 |
Aminoacidurias due to inherited disorders of metabolism. 2.
Topics: Acidosis; Alanine; Amino Acid Metabolism, Inborn Errors; Arginase; Arginine; Carbamates; Citrulline; | 1973 |
[Diseases of newborn infants based on inborn anomalies of metabolism. 1. Theoretical principles].
Topics: Adrenal Hyperplasia, Congenital; Amino Acid Metabolism, Inborn Errors; Amino Acids; Carbohydrate Met | 1970 |
5 other studies available for urea and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles.
Topics: Antioxidants; Cholesterol; Cholesterol, HDL; Cholesterol, LDL; Creatine; Glucose; Humans; Hydro-Lyas | 2008 |
Neuropathological study of aminoacidurias.
Topics: Age Factors; Amino Acid Metabolism, Inborn Errors; Central Nervous System; Cystathionine; Female; Gl | 1972 |
Pediatric clinical aspects of aminoacidopathies.
Topics: Age Factors; Amino Acid Metabolism, Inborn Errors; Arginine; Child; Child, Preschool; Female; Homocy | 1972 |
Central nervous system lesions in disorders of amino-acid metabolism. A neuropathological study.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Brain; Central Nervous System; Chi | 1972 |
Disorders of amino acid metabolism--1971.
Topics: Amino Acid Metabolism, Inborn Errors; Histidine; Homocystinuria; Humans; Maple Syrup Urine Disease; | 1971 |