One Year Experience of Pheburane (R) (Sodium Phenylbutyrate) Treatment in a Patient with Argininosuccinate Lyase Deficiency

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dc.contributor.authorUcar, Sema Kalkan
dc.contributor.authorOzbaran, Burcu
dc.contributor.authorAltinok, Yasemin Atik
dc.contributor.authorKose, Melis
dc.contributor.authorCanda, Ebru
dc.contributor.authorKagnici, Mehtap
dc.contributor.authorCoker, Mahmut
dc.contributor.editorZschocke, J
dc.contributor.editorBaumgartner, M
dc.contributor.editorMorava, E
dc.contributor.editorPatterson, M
dc.contributor.editorRahman, S
dc.contributor.editorPeters, V
dc.date.accessioned2019-10-27T22:28:44Z
dc.date.available2019-10-27T22:28:44Z
dc.date.issued2015
dc.departmentEge Üniversitesien_US
dc.description.abstractArgininosuccinate lyase deficiency (ASLD) is a urea cycle disorder (UCD) treated with dietary adjustment and nitrogen scavenging agents. "Pheburane (R)" is a new tasteless and odour-free formulation of sodium phenylbutyrate, indicated in the treatment of UCD. A male patient diagnosed with ASLD was put on treatment with the new formulation of sodium phenylbutyrate (granules) for a period of one year, at 500 mg/kg orally in 3 intakes/day. Plasma glutamine, arginine, citrulline, argininosuccinate, serum sodium, potassium, liver function tests and urine orotate all remained unchanged over this period. There was no difference in mean ammonia levels before and after treatment, and no hyperammonemia episode occurred during treatment with Pheburane (R). An improvement in a measurement of quality of life (QOL) was noted after treatment with Pheburane (R) r. Conclusion: Good metabolic control and improved QOL were achieved throughout the treatment period.en_US
dc.identifier.doi10.1007/8904_2014_361en_US
dc.identifier.endpage33en_US
dc.identifier.isbn978-3-662-46190-7; 978-3-662-46189-1
dc.identifier.issn2192-8304
dc.identifier.pmid25665834en_US
dc.identifier.startpage31en_US
dc.identifier.urihttps://doi.org/10.1007/8904_2014_361
dc.identifier.urihttps://hdl.handle.net/11454/50972
dc.identifier.volume19en_US
dc.identifier.wosWOS:000376982900005en_US
dc.identifier.wosqualityN/Aen_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherSpringer-Verlag Berlinen_US
dc.relation.ispartofJimd Reports, Vol 19en_US
dc.relation.ispartofseriesJIMD Reports
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleOne Year Experience of Pheburane (R) (Sodium Phenylbutyrate) Treatment in a Patient with Argininosuccinate Lyase Deficiencyen_US
dc.typeArticleen_US

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