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

Autor: Mireia del Toro, Inmaculada Vives-Piñera, Mónica Ruiz-Pons, Teresa Campos, Luis Aldámiz-Echevarría, Mª Concepción García-Jiménez, Olatz Villate, Javier Blasco, Miguel A. Barba Romero, Guillem Pintos-Morell, Ana Mª Marquez, Isidro Vitoria, Raquel Yahyaoui, David Gil, Mª Teresa García Silva, Rocío Puig-Piña, Javier de las Heras, Elena Martín Hernández, Elvira Cañedo, Luis Peña-Quintana, Esmeralda Rodrigues, Pilar Quijada-Fraile, Fernando Andrade, Patricia Correcher, Leticia Ceberio
Rok vydání: 2019
Předmět:
Adult
Male
Adolescent
Tandem mass spectrometry
Urinary system
Glutamine
Hyperammonaemia
Clinical Biochemistry
Pharmaceutical Science
Urine
Phenylacetic acid
01 natural sciences
Benzoates
Urea cycle disorders
Analytical Chemistry
Medication Adherence
chemistry.chemical_compound
Young Adult
Tandem Mass Spectrometry
Drug Discovery
parasitic diseases
Humans
Benzoic acid
Child
Urea Cycle Disorders
Inborn
Spectroscopy
Chromatography
High Pressure Liquid
Chromatography
010405 organic chemistry
Benzoic acid
Hyperammonaemia
Phenylbutyric acid
Stability
Tandem mass spectrometry
Urea cycle disorders
010401 analytical chemistry
Infant
Newborn
Infant
Phenylbutyrates
0104 chemical sciences
Phenylacetylglutamine
chemistry
Urea cycle
Child
Preschool
Phenylbutyric acid
Female
Drug Monitoring
Stability
Biomarkers
Zdroj: JOURNAL OF PHARMACEUTICAL AND BIOMEDICAL ANALYSIS
r-IIS La Fe. Repositorio Institucional de Producción Científica del Instituto de Investigación Sanitaria La Fe
instname
Journal of Pharmaceutical and Biomedical Analysis
r-IGTP. Repositorio Institucional de Producción Científica del Instituto de Investigación Germans Trias i Pujol
r-IGTP: Repositorio Institucional de Producción Científica del Instituto de Investigación Germans Trias i Pujol
Institut de Recerca Germans Trias i Pujol (IGTP)
ISSN: 0731-7085
Popis: 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 degrees C. This procedure is useful for the optimization and monitorization of the drug dose allowing the use of spot urine samples. (C) 2019 Elsevier B.V. All rights reserved.
Databáze: OpenAIRE
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