Cargando…

The Effect of Glycomacropeptide versus Amino Acids on Phenylalanine and Tyrosine Variability over 24 Hours in Children with PKU: A Randomized Controlled Trial

Introduction: In phenylketonuria (PKU), evidence suggests that casein glycomacropeptide supplemented with rate-limiting amino acids (CGMP-AA) is associated with better protein utilisation and less blood phenylalanine (Phe) variability. Aim: To study the impact of CGMP-AA on blood Phe variability usi...

Descripción completa

Detalles Bibliográficos
Autores principales:	Daly, Anne, Evans, Sharon, Chahal, Satnam, Santra, Saikat, Pinto, Alex, Gingell, Cerys, Rocha, Júlio César, van Spronsen, Francjan, Jackson, Richard, MacDonald, Anita
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2019
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6471794/ https://www.ncbi.nlm.nih.gov/pubmed/30823411 http://dx.doi.org/10.3390/nu11030520

Ejemplares similares

Glycomacropeptide: long-term use and impact on blood phenylalanine, growth and nutritional status in children with PKU
por: Daly, A., et al.
Publicado: (2019)

Glycomacropeptide in PKU—Does It Live Up to Its Potential?
por: Daly, Anne, et al.
Publicado: (2022)

The Effect of Various Doses of Phenylalanine Supplementation on Blood Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients
por: van Ginkel, Willem G., et al.
Publicado: (2019)

Comparison of Glycomacropeptide with Phenylalanine Free-Synthetic Amino Acids in Test Meals to PKU Patients: No Significant Differences in Biomarkers, Including Plasma Phe Levels
por: Ahring, Kirsten K., et al.
Publicado: (2018)

The influence of parental food preference and neophobia on children with phenylketonuria (PKU)
por: Evans, Sharon, et al.
Publicado: (2017)

Growth and Body Composition in PKU Children—A Three-Year Prospective Study Comparing the Effects of L-Amino Acid to Glycomacropeptide Protein Substitutes
por: Daly, Anne, et al.
Publicado: (2021)

How Does Feeding Development and Progression onto Solid Foods in PKU Compare with Non-PKU Children During Weaning?
por: Evans, Sharon, et al.
Publicado: (2019)

Evaluation of a New Glycomacropeptide-Based Protein Substitute in Powdered and Liquid Format in Patients with PKU
por: Delsoglio, Marta, et al.
Publicado: (2023)

Development of national consensus statements on food labelling interpretation and protein allocation in a low phenylalanine diet for PKU
por: Evans, Sharon, et al.
Publicado: (2019)

Large neutral amino acids in the treatment of PKU: from theory to practice
por: van Spronsen, Francjan J., et al.
Publicado: (2010)

The Impact of the Use of Glycomacropeptide on Satiety and Dietary Intake in Phenylketonuria
por: Daly, Anne, et al.
Publicado: (2020)

Dietary Liberalization in Tetrahydrobiopterin-Treated PKU Patients: Does It Improve Outcomes?
por: Evers, Roeland A. F., et al.
Publicado: (2022)

PKU dietary handbook to accompany PKU guidelines
por: MacDonald, A., et al.
Publicado: (2020)

Dried blood spot versus venous blood sampling for phenylalanine and tyrosine
por: van Vliet, Kimber, et al.
Publicado: (2020)

A Three-Year Longitudinal Study Comparing Bone Mass, Density, and Geometry Measured by DXA, pQCT, and Bone Turnover Markers in Children with PKU Taking L-Amino Acid or Glycomacropeptide Protein Substitutes
por: Daly, Anne, et al.
Publicado: (2021)

Correction to: PKU dietary handbook to accompany PKU guidelines
por: MacDonald, A., et al.
Publicado: (2020)

The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis
por: Pena, Maria João, et al.
Publicado: (2018)

Growth, Protein and Energy Intake in Children with PKU Taking a Weaning Protein Substitute in the First Two Years of Life: A Case-Control Study
por: Evans, Sharon, et al.
Publicado: (2019)

Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria
por: Daly, Anne, et al.
Publicado: (2020)

Is the Phenylalanine-Restricted Diet a Risk Factor for Overweight or Obesity in Patients with Phenylketonuria (PKU)? A Systematic Review and Meta-Analysis
por: Rodrigues, Catarina, et al.
Publicado: (2021)

Unveiling the Metabolic Effects of Glycomacropeptide
por: Pena, Maria João, et al.
Publicado: (2021)

Casein Glycomacropeptide: An Alternative Protein Substitute in Tyrosinemia Type I
por: Daly, Anne, et al.
Publicado: (2021)

Pre-attentive processing in children with early and continuously-treated PKU. Effects of concurrent Phe level and lifetime dietary control
por: de Sonneville, Leo M. J., et al.
Publicado: (2011)

Optimising amino acid absorption: essential to improve nitrogen balance and metabolic control in phenylketonuria
por: MacDonald, Anita, et al.
Publicado: (2018)

The Impact of the Quality of Nutrition and Lifestyle in the Reproductive Years of Women with PKU on the Long-Term Health of Their Children
por: Gama, Maria Inês, et al.
Publicado: (2022)

Protein Substitutes in PKU; Their Historical Evolution
por: Daly, Anne, et al.
Publicado: (2021)

PKU mutation p.G46S prevents the stereospecific binding of l‐phenylalanine to the dimer of human phenylalanine hydroxylase regulatory domain
por: Leandro, João, et al.
Publicado: (2017)

Patient’s thoughts and expectations about centres of expertise for PKU
por: van Wegberg, A. M. J., et al.
Publicado: (2021)

Dietary management of maternal phenylketonuria with glycomacropeptide and amino acids supplements: A case report
por: Pinto, A., et al.
Publicado: (2017)

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)

Protein Labelling Accuracy for UK Patients with PKU Following a Low Protein Diet
por: Kraleva, Dilyana, et al.
Publicado: (2020)

Long-term dietary intervention with low Phe and/or a specific nutrient combination improve certain aspects of brain functioning in phenylketonuria (PKU)
por: Bruinenberg, Vibeke M., et al.
Publicado: (2019)

Development of a Whole Blood Paper-Based Device for Phenylalanine Detection in the Context of PKU Therapy Monitoring
por: Robinson, Robert, et al.
Publicado: (2016)

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)

Presumptive brain influx of large neutral amino acids and the effect of phenylalanine supplementation in patients with Tyrosinemia type 1
por: van Ginkel, Willem G., et al.
Publicado: (2017)

Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective
por: Pena, Maria João, et al.
Publicado: (2021)

In Vivo Metabolic Responses to Different Formulations of Amino Acid Mixtures for the Treatment of Phenylketonuria (PKU)
por: Giarratana, Nadia, et al.
Publicado: (2022)

Untreated PKU Patients without Intellectual Disability: What Do They Teach Us?
por: van Vliet, Danique, et al.
Publicado: (2019)

Gut-Microbiome Composition in Response to Phenylketonuria Depends on Dietary Phenylalanine in BTBR Pah(enu2) Mice
por: van der Goot, Els, et al.
Publicado: (2022)

Creatine energy substrate increases bone density in the Pah(enu2) classical PKU mouse in the context of phenylalanine restriction
por: Dobrowolski, Steven F., et al.
Publicado: (2023)

Cannot write session to /tmp/vufind_sessions/sess_5d2f0i6u2k7susqdgb3k3uadih