Please use this identifier to cite or link to this item: http://hdl.handle.net/10553/70033
Title: Quantification of urinary derivatives of Phenylbutyric and Benzoic acids by LC-MS/MS as treatment compliance biomarkers in Urea Cycle disorders
Authors: Andrade, Fernando
Vitoria, Isidro
Martín Hernández, Elena
Pintos-Morell, Guillem
Correcher, Patricia
Puig-Piña, Rocío
Quijada-Fraile, Pilar
Peña Quintana, Luis 
Marquez, Ana Mª
Villate, Olatz
García Silva, Mª Teresa
de las Heras, Javier
Ceberio, Leticia
Rodrigues, Esmeralda
Almeida Campos, Teresa
Yahyaoui, Raquel
Blasco, Javier
Vives-Piñera, Inmaculada
Gil, David
del Toro, Mireia
Ruiz-Pons, Mónica
Cañedo, Elvira
Barba Romero, Miguel Angel
García-Jiménez, Mª Concepción
Aldámiz-Echevarría, Luis
UNESCO Clasification: 32 Ciencias médicas
Keywords: Benzoic Acid
Hyperammonaemia
Phenylbutyric Acid
Stability
Tandem Mass Spectrometry, et al
Issue Date: 2019
Journal: Journal of Pharmaceutical and Biomedical Analysis 
Abstract: Purpose: Salts of phenylacetic acid (PAA) and phenylbutyric acid (PBA) have been used for nitrogen elimination as a treatment for hyperammonaemia caused by urea cycle disorders (UCD). A new analytical method for PBA measurement in urine which helps to evaluate the drug adherence has been implemented. Methods: Urine specimens from UCD patients receiving PBA were analysed by tandem mass spectrometry to measure urine phenylacetylglutamine (PAGln). Some clinical and biochemical data for each patient were collected. Results: Our study included 87 samples from 40 UCD patients. The PAGln levels did not correlate with height, weight or age. However, the PAGln values showed correlation with PBA dose (r = 0.383, P = 0.015). Plasma glutamine and ammonia levels presented a positive correlation (r = 0.537, P < 0.001). The stability for PAGln in urine was determined at different storage temperatures. Conclusions: We have developed a simple method for the determination of PAGln in urine, which acts as useful biomarker of effective drug delivery. PAGln in urine is stable at room temperature at least for 15 days, and for several months when frozen at -20 °C. This procedure is useful for the optimization and monitorization of the drug dose allowing the use of spot urine samples.
URI: http://hdl.handle.net/10553/70033
ISSN: 0731-7085
DOI: 10.1016/j.jpba.2019.112798
Source: Journal of Pharmaceutical and Biomedical Analysis [ISSN 0731-7085], v. 176
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