Cargando…

Clinical relevance of the discrepancy in phenylalanine concentrations analyzed using tandem mass spectrometry compared with ion-exchange chromatography in phenylketonuria

INTRODUCTION: Metabolic control of phenylketonuria (PKU) and compliance with the low-phenylalanine (phe) diet are frequently assessed by measuring blood phe concentrations in dried blood spots (DBS) collected by patients instead of plasma phe concentrations. OBJECTIVE: Our objective was to investiga...

Descripción completa

Detalles Bibliográficos
Autores principales:	Stroup, Bridget M., Held, Patrice K., Williams, Phillip, Clayton, Murray K., Murali, Sangita G., Rice, Gregory M., Ney, Denise M.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Elsevier 2016
Materias:	Research Paper
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4789345/ https://www.ncbi.nlm.nih.gov/pubmed/27014575 http://dx.doi.org/10.1016/j.ymgmr.2016.01.001

Ejemplares similares

Glycomacropeptide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial(1)(2)
por: Ney, Denise M, et al.
Publicado: (2016)

Metabolomic Insights into the Nutritional Status of Adults and Adolescents with Phenylketonuria Consuming a Low-Phenylalanine Diet in Combination with Amino Acid and Glycomacropeptide Medical Foods
por: Stroup, Bridget M., et al.
Publicado: (2017)

Dietary amino acid intakes associated with a low-phenylalanine diet combined with amino acid medical foods and glycomacropeptide medical foods and neuropsychological outcomes in subjects with phenylketonuria
por: Stroup, Bridget M., et al.
Publicado: (2017)

Amino Acid Medical Foods Provide a High Dietary Acid Load and Increase Urinary Excretion of Renal Net Acid, Calcium, and Magnesium Compared with Glycomacropeptide Medical Foods in Phenylketonuria
por: Stroup, Bridget M., et al.
Publicado: (2017)

Sex effects of dietary protein source and acid load on renal and bone status in the Pah(enu2) mouse model of phenylketonuria
por: Stroup, Bridget M., et al.
Publicado: (2019)

Sex differences in body composition and bone mineral density in phenylketonuria: A cross-sectional study
por: Stroup, Bridget M., et al.
Publicado: (2018)

Age-Related Psychophysiological Vulnerability to Phenylalanine in Phenylketonuria
por: Leuzzi, Vincenzo, et al.
Publicado: (2014)

Low Bone Strength Is a Manifestation of Phenylketonuria in Mice and Is Attenuated by a Glycomacropeptide Diet
por: Solverson, Patrick, et al.
Publicado: (2012)

Therapeutic implication of L-phenylalanine aggregation mechanism and its modulation by D-phenylalanine in phenylketonuria
por: Singh, Virender, et al.
Publicado: (2014)

Mutations of the phenylalanine hydroxylase gene in Iranian patients with phenylketonuria
por: Biglari, Alireza, et al.
Publicado: (2015)

Blood phenylalanine instability strongly correlates with anxiety in phenylketonuria
por: Didycz, Bozena, et al.
Publicado: (2017)

Phenylalanine free infant formula in the dietary management of phenylketonuria
por: Yilmaz, Ozlem, et al.
Publicado: (2023)

Phenylketonuria in the Latvian population: Molecular basis, phenylalanine levels, and patient compliance
por: Kreile, M., et al.
Publicado: (2020)

Mutations of the phenylalanine hydroxylase gene in patients with phenylketonuria in Shanxi, China
por: Zhou, Yong-An, et al.
Publicado: (2012)

Mutation analysis of Phenylalanine hydroxylase gene in Iranian patients with Phenylketonuria
por: Rastegar Moghadam, Mahsa, et al.
Publicado: (2018)

Comparison of phenylalanine tolerance in singleton and twin pregnancies in patients with phenylketonuria
por: Hozyasz, Kamil Konrad, et al.
Publicado: (2020)

Phenylalanine hydroxylase mRNA rescues the phenylketonuria phenotype in mice
por: Cacicedo, Maximiliano L., et al.
Publicado: (2022)

Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein
por: McWhorter, Nicole, et al.
Publicado: (2022)

Neuroradiological findings of an adolescent with early treated phenylketonuria: is phenylalanine restriction enough?
por: Beckhauser, Mayara Thays, et al.
Publicado: (2011)

Formulation and PEGylation optimization of the therapeutic PEGylated phenylalanine ammonia lyase for the treatment of phenylketonuria
por: Bell, Sean M., et al.
Publicado: (2017)

Comparison of Tandem Mass Spectrometry and the Fluorometric Method—Parallel Phenylalanine Measurement on a Large Fresh Sample Series and Implications for Newborn Screening for Phenylketonuria
por: Perko, Dasa, et al.
Publicado: (2023)

Preparation of a Selective L-Phenylalanine Imprinted Polymer Implicated in Patients with Phenylketonuria
por: Najafizadeh, Parvaneh, et al.
Publicado: (2014)

Long-Term Metabolic Correction of Phenylketonuria by AAV-Delivered Phenylalanine Amino Lyase
por: Tao, Rui, et al.
Publicado: (2020)

Improving phenylalanine and micronutrients status of children with phenylketonuria: a pilot randomized study
por: Zamani, Reza, et al.
Publicado: (2021)

Long-Term Metabolic Correction of Phenylketonuria by AAV-Delivered Phenylalanine Amino Lyase
por: Tao, Rui, et al.
Publicado: (2022)

Improved attention linked to sustained phenylalanine reduction in adults with early‐treated phenylketonuria
por: Bilder, Deborah A., et al.
Publicado: (2021)

Phenylalanine Tolerance over Time in Phenylketonuria: A Systematic Review and Meta-Analysis
por: Pinto, Alex, et al.
Publicado: (2023)

Monitoring phenylalanine concentrations in the follow‐up of phenylketonuria patients: An inventory of pre‐analytical and analytical variation
por: Coene, Karlien L. M., et al.
Publicado: (2020)

Differential Effects of Dietary Fat Content and Protein Source on Bone Phenotype and Fatty Acid Oxidation in Female C57Bl/6 Mice
por: Sawin, Emily A., et al.
Publicado: (2016)

Propagation of Plasma L-Phenylalanine Concentration Fluctuations to the Neurovascular Unit in Phenylketonuria: An in silico Study
por: Taslimifar, Mehdi, et al.
Publicado: (2019)

The spectrum of phenylalanine hydroxylase variants and genotype–phenotype correlation in phenylketonuria patients in Gansu, China
por: Zhang, Chuan, et al.
Publicado: (2023)

Aspartame and Phenylketonuria: an analysis of the daily phenylalanine intake of aspartame-containing drugs marketed in France
por: Maler, Victor, et al.
Publicado: (2023)

Lipid nanoparticle delivers phenylalanine ammonia lyase mRNA to the liver leading to catabolism and clearance of phenylalanine in a phenylketonuria mouse model
por: Diaz-Trelles, Ramon, et al.
Publicado: (2022)

Blood phenylalanine reduction reverses gene expression changes observed in a mouse model of phenylketonuria
por: Manek, Rachna, et al.
Publicado: (2021)

Doxycycline hinders phenylalanine fibril assemblies revealing a potential novel therapeutic approach in phenylketonuria
por: De Luigi, Ada, et al.
Publicado: (2015)

The evaluation of phenylalanine levels in Estonian phenylketonuria patients during eight years by electronic laboratory records
por: Lilleväli, Hardo, et al.
Publicado: (2019)

The Impact of Phenylalanine Levels on Cognitive Outcomes in Adults With Phenylketonuria: Effects Across Tasks and Developmental Stages
por: Romani, Cristina, et al.
Publicado: (2017)

Effect of dietary regime on metabolic control in phenylketonuria: Is exact calculation of phenylalanine intake really necessary?
por: Rohde, Carmen, et al.
Publicado: (2015)

Intrinsic property of phenylalanine to trigger protein aggregation and hemolysis has a direct relevance to phenylketonuria
por: Anand, Bibin G., et al.
Publicado: (2017)

Cognitive Outcomes and Relationships with Phenylalanine in Phenylketonuria: A Comparison between Italian and English Adult Samples
por: Romani, Cristina, et al.
Publicado: (2020)

Cannot write session to /tmp/vufind_sessions/sess_c34mk8ig20l7q6l9p2rjhfn2pt