Page last updated: 2024-10-27

flumazenil and Maple Syrup Urine Disease

flumazenil has been researched along with Maple Syrup Urine Disease in 2 studies

Flumazenil: A potent benzodiazepine receptor antagonist. Since it reverses the sedative and other actions of benzodiazepines, it has been suggested as an antidote to benzodiazepine overdoses.
flumazenil : An organic heterotricyclic compound that is 5,6-dihydro-4H-imidazo[1,5-a][1,4]benzodiazepine which is substituted at positions 3, 5, 6, and 8 by ethoxycarbonyl, methyl, oxo, and fluoro groups, respectively. It is used as an antidote to benzodiazepine overdose.

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-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's2 (100.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Quental, S2
Gusmão, A1
Rodríguez-Pombo, P1
Ugarte, M1
Vilarinho, L2
Amorim, A2
Prata, MJ2
Macedo-Ribeiro, S1
Matos, R1
Martins, E1
Teles, EL1
Rodrigues, E1
Diogo, L1
Garcia, P1
Eusébio, F1
Gaspar, A1
Sequeira, S1
Furtado, F1
Lança, I1

Other Studies

2 other studies available for flumazenil and Maple Syrup Urine Disease

ArticleYear
Revisiting MSUD in Portuguese Gypsies: evidence for a founder mutation and for a mutational hotspot within the BCKDHA gene.
    Annals of human genetics, 2009, Volume: 73, Issue:Pt 3

    Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Chromosome Mapping; Cohort Studies; DNA Mutationa

2009
Molecular and structural analyses of maple syrup urine disease and identification of a founder mutation in a Portuguese Gypsy community.
    Molecular genetics and metabolism, 2008, Volume: 94, Issue:2

    Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Amino Acids, Branched-Chain; Female; Humans; Male

2008