A Patient with MSUD: Acute Management with Sodium Phenylacetate/Sodium Benzoate and Sodium Phenylbutyrate.

Title A Patient with MSUD: Acute Management with Sodium Phenylacetate/Sodium Benzoate and Sodium Phenylbutyrate.
Authors M. Köse; E. Canda; M. Kagnici; S.Kalkan Uçar; M. Çoker
Journal Case Rep Pediatr
DOI 10.1155/2017/1045031
Abstract

In treatment of metabolic imbalances caused by maple syrup urine disease (MSUD), peritoneal dialysis, and hemofiltration, pharmacological treatments for elimination of toxic metabolites can be used in addition to basic dietary modifications. Therapy with sodium phenylacetate/benzoate or sodium phenylbutyrate (NaPB) in urea-cycle disorder cases has been associated with a reduction in branched-chain amino acid (BCAA) concentrations when the patients are on adequate dietary protein intake. Moreover, NaPB in treatment of MSUD patients is also associated with reduction of BCAA levels in a limited number of cases. However, there are not enough studies in the literature about application and efficacy of this treatment. Our case report sets an example of an alternative treatment's efficacy when extracorporeal procedures are not available due to technical difficulties during attack period of the disease.

Citation M. Köse; E. Canda; M. Kagnici; S.Kalkan Uçar; M. Çoker.A Patient with MSUD: Acute Management with Sodium Phenylacetate/Sodium Benzoate and Sodium Phenylbutyrate.. Case Rep Pediatr. 2017;2017:1045031. doi:10.1155/2017/1045031

Related Elements

Sodium

11 Na 22.989769280 Sodium

Sodium Bohr ModelSee more Sodium products. Sodium (atomic symbol: Na, atomic number: 11) is a Block D, Group 5, Period 4 element with an atomic weight of 22.989769. The number of electrons in each of Sodium's shells is [2, 8, 1] and its electron configuration is [Ne] 3s1. The sodium atom has a radius of 185.8 pm and a Van der Waals radius of 227 pm. Sodium was discovered and first isolated by Sir Humphrey Davy in 1807. In its elemental form, sodium has a silvery-white metallic appearance. It is the sixth most abundant element, making up 2.6 % of the earth's crust. Sodium does not occur in nature as a free element and must be extracted from its compounds (e.g., feldspars, sodalite, and rock salt). The name Sodium is thought to come from the Arabic word suda, meaning "headache" (due to sodium carbonate's headache-alleviating properties), and its elemental symbol Na comes from natrium, its Latin name.

Related Forms & Applications

Acetates Benzoates Organometallics
Publish your research on the American Elements website