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

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dc.contributor.authorKose, Melis
dc.contributor.authorCanda, Ebru
dc.contributor.authorKagnici, Mehtap
dc.contributor.authorUcar, Sema Kalkan
dc.contributor.authorCoker, Mahmut
dc.date.accessioned2019-10-27T11:21:48Z
dc.date.available2019-10-27T11:21:48Z
dc.date.issued2017
dc.departmentEge Üniversitesien_US
dc.description.abstractIn 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.en_US
dc.identifier.doi10.1155/2017/1045031en_US
dc.identifier.issn2090-6803
dc.identifier.issn2090-6811
dc.identifier.pmid28589054en_US
dc.identifier.urihttps://doi.org/10.1155/2017/1045031
dc.identifier.urihttps://hdl.handle.net/11454/32959
dc.identifier.wosWOS:000402083100001en_US
dc.identifier.wosqualityN/Aen_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherHindawi Ltden_US
dc.relation.ispartofCase Reports in Pediatricsen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleA Patient with MSUD: Acute Management with Sodium Phenylacetate/Sodium Benzoate and Sodium Phenylbutyrateen_US
dc.typeArticleen_US

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