Quantification of urinary derivatives of Phenylbutyric and Benzoic acids by LC-MS/MS as treatment compliance biomarkers in Urea Cycle disorders.

Fernando Andrade,Isidro Vitoria, Elena Martín Hernández,Guillem Pintos-Morell,Patricia Correcher,Rocío Puig-Piña,Pilar Quijada-Fraile,Luis Peña-Quintana, Ana Mª Marquez,Olatz Villate, Mª Teresa García Silva,Javier de Las Heras,Leticia Ceberio,Esmeralda Rodrigues,Teresa Almeida Campos,Raquel Yahyaoui,Javier Blasco,Inmaculada Vives-Piñera,David Gil,Mireia Del Toro,Mónica Ruiz-Pons,Elvira Cañedo,Miguel Angel Barba Romero, Mª Concepción García-Jiménez,Luis Aldámiz-Echevarría

Journal of Pharmaceutical and Biomedical Analysis（2019）

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摘要

•Evaluation of a new analytical method for Phenylacetylglutamine in urine.•Biomarker of monitoring and adherence to the treatment in urea cycle disorders.•Clinical data are correlated to Phenylacetylglutamine for the first time in Europe.•The use of spot urines is easier to send for analysis due to its stability.

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关键词

BA,Gln,HA,PAA,PBA,PAGln,UCD

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