Cargando…

Effect of dietary regime on metabolic control in phenylketonuria: Is exact calculation of phenylalanine intake really necessary?

BACKGROUND: A phenylalanine (Phe) restricted dietary management is required in phenylketonuria (PKU) to maintain good metabolic control. Nevertheless, five different models of dietary regimes, which differ in their accuracy of Phe documentation, are used. To investigate the effect of the dietary reg...

Descripción completa

Detalles Bibliográficos
Autores principales:	Rohde, Carmen, Thiele, Alena Gerlinde, Och, Ulrike, Schönherr, Katrin, Meyer, Uta, Rosenbaum-Fabian, Stefanie, Maddalon, Cornelia, Matzken, Sabine, Blessing, Holger, Lang, Frauke, Jörg-Streller, Monika, Beblo, Skadi
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Elsevier 2015
Materias:	Research Paper
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5471407/ https://www.ncbi.nlm.nih.gov/pubmed/28649540 http://dx.doi.org/10.1016/j.ymgmr.2015.09.006

Ejemplares similares

Preventing maternal phenylketonuria (PKU) syndrome: important factors to achieve good metabolic control throughout pregnancy
por: Rohde, Carmen, et al.
Publicado: (2021)

Psychological well‐being of early and continuously treated phenylketonuria patients
por: Thiele, Alena Gerlinde, et al.
Publicado: (2021)

Ten years of specialized adult care for phenylketonuria – a single-centre experience
por: Mütze, Ulrike, et al.
Publicado: (2016)

The challenge of long-term tetrahydrobiopterin (BH(4)) therapy in phenylketonuria: Effects on metabolic control, nutritional habits and nutrient supply
por: Thiele, Alena G., et al.
Publicado: (2015)

Metabolomics of Dietary Fatty Acid Restriction in Patients with Phenylketonuria
por: Mütze, Ulrike, et al.
Publicado: (2012)

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

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)

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)

Dietary and metabolic effects on the oral status of patients with phenylketonuria: a nation-based cross-sectional study
por: Bingöl, Anne Carolin, et al.
Publicado: (2023)

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

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

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

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

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)

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

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

Food Regime for Phenylketonuria: Presenting Complications and Possible Solutions
por: Kumar Dalei, Sudipt, et al.
Publicado: (2022)

Is CME Really Necessary?
por: Hall, G H
Publicado: (1994)

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)

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)

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)

Nutrient intake, body composition, and blood phenylalanine control in children with phenylketonuria compared to healthy controls
por: Sailer, Melissa, et al.
Publicado: (2020)

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)

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

Influence of blood phenylalanine level variations on the development of executive functions and social cognition in children with phenylketonuria
por: de Almeida Duarte, Cristiane Mendes, et al.
Publicado: (2023)

Cannot write session to /tmp/vufind_sessions/sess_a2usan1l3v8oljgk0thol4r8pc