Cargando…

One-year follow-up of B vitamin and Iron status in patients with phenylketonuria provided tetrahydrobiopterin (BH4)

BACKGROUND: People with Phenylketonuria (PKU) who respond to tetrahydrobiopterin (BH4) often decrease dependence on medical food (MF) following increased phenylalanine (phe) tolerance. Responders to BH4 may experience a reduction in certain nutrients if not compensated through intact foods or supple...

Descripción completa

Detalles Bibliográficos
Autores principales:	Brantley, Kristen D, Douglas, Teresa D, Singh, Rani H
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	BioMed Central 2018
Materias:	Research
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6206913/ https://www.ncbi.nlm.nih.gov/pubmed/30373601 http://dx.doi.org/10.1186/s13023-018-0923-2

Ejemplares similares

The effects of tetrahydrobiopterin (BH(4)) treatment on brain function in individuals with phenylketonuria()
por: Christ, Shawn E., et al.
Publicado: (2013)

Molecular genetics of phenylketonuria and tetrahydrobiopterin deficiency in Jordan
por: Carducci, Carla, et al.
Publicado: (2020)

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)

Influencing Factors on the Use of Tetrahydrobiopterin in Patients with Phenylketonuria
por: Gao, Hui
Publicado: (2022)

The neonatal tetrahydrobiopterin loading test in phenylketonuria: what is the predictive value?
por: Anjema, Karen, et al.
Publicado: (2016)

Consensus guideline for the diagnosis and treatment of tetrahydrobiopterin (BH(4)) deficiencies
por: Opladen, Thomas, et al.
Publicado: (2020)

Long-term follow-up of patients with phenylketonuria treated with tetrahydrobiopterin: a seven years experience
por: Scala, Iris, et al.
Publicado: (2015)

Tetrahydrobiopterin responsiveness in phenylketonuria: prediction with the 48-hour loading test and genotype
por: Anjema, Karen, et al.
Publicado: (2013)

Correlation between genotype and the tetrahydrobiopterin-responsive phenotype in Chinese patients with phenylketonuria
por: Tao, Jing, et al.
Publicado: (2015)

Tetrahydrobiopterin responsiveness in a series of 53 cases of phenylketonuria and hyperphenylalaninemia in Iran
por: Setoodeh, Aria, et al.
Publicado: (2015)

Correction to: Consensus guideline for the diagnosis and treatment of tetrahydrobiopterin (BH4) deficiencies
por: Opladen, Thomas, et al.
Publicado: (2020)

0888. Administration of tetrahydrobiopterin (BH4) protects renal microcirculation after ischemia and reperfusion
por: Rahmania, L, et al.
Publicado: (2014)

Longitudinal quality of life analysis in a phenylketonuria cohort provided sapropterin dihydrochloride
por: Douglas, Teresa D, et al.
Publicado: (2013)

Tetrahydrobiopterin (BH4) deficiency is associated with augmented inflammation and microvascular degeneration in the retina
por: Rivera, José Carlos, et al.
Publicado: (2017)

The Critical Role of Tetrahydrobiopterin (BH4) Metabolism in Modulating Radiosensitivity: BH4/NOS Axis as an Angel or a Devil
por: Feng, Yang, et al.
Publicado: (2021)

Massive parallel sequencing as a new diagnostic approach for phenylketonuria and tetrahydrobiopterin-deficiency in Thailand
por: Chaiyasap, Pongsathorn, et al.
Publicado: (2017)

Data on phenylalanine-to-tyrosine ratios in assessment of tetrahydrobiopterin (BH(4))-responsiveness in patients with hyperphenylalaninemia
por: Lampret, Barbka Repic, et al.
Publicado: (2022)

Increased NOS coupling by the metabolite tetrahydrobiopterin (BH4) reduces preeclampsia/IUGR consequences
por: Chatre, Laurent, et al.
Publicado: (2022)

Cuticle Integrity and Biogenic Amine Synthesis in Caenorhabditis elegans Require the Cofactor Tetrahydrobiopterin (BH4)
por: Loer, Curtis M., et al.
Publicado: (2015)

Genotype-phenotype associations in French patients with phenylketonuria and importance of genotype for full assessment of tetrahydrobiopterin responsiveness
por: Jeannesson-Thivisol, Elise, et al.
Publicado: (2015)

Protein Substitute Requirements of Patients with Phenylketonuria on BH4 Treatment: A Systematic Review and Meta-Analysis
por: Ilgaz, Fatma, et al.
Publicado: (2021)

Large neutral amino acid status in association with P:T ratio and diet in adult and pediatric patients with phenylketonuria
por: Douglas, Teresa D., et al.
Publicado: (2019)

The Predictive Value of Genetic Analyses in the Diagnosis of Tetrahydrobiopterin (BH4)-Responsiveness in Chinese Phenylalanine Hydroxylase Deficiency Patients
por: Zhu, Tianwen, et al.
Publicado: (2017)

Tetrahydrobiopterin (BH(4)) Supplementation Prevents the Cardiorenal Effects of Diabetes in Mice by Reducing Oxidative Stress, Inflammation and Fibrosis
por: Novoa, Ulises, et al.
Publicado: (2022)

Role of endothelial colony forming cells (ECFCs) Tetrahydrobiopterin (BH4) in determining ECFCs functionality in coronary artery disease (CAD) patients
por: Sen, Atanu, et al.
Publicado: (2022)

Genotypes of 2579 patients with phenylketonuria reveal a high rate of BH4 non-responders in Russia
por: Gundorova, Polina, et al.
Publicado: (2019)

Alteration of Striatal Tetrahydrobiopterin in Iron-Induced Unilateral Model of Parkinson's Disease
por: Aryal, Bijay, et al.
Publicado: (2014)

Evaluation of Tetrahydrobiopterin Therapy with Large Neutral Amino Acid Supplementation in Phenylketonuria: Effects on Potential Peripheral Biomarkers, Melatonin and Dopamine, for Brain Monoamine Neurotransmitters
por: Yano, Shoji, et al.
Publicado: (2016)

Carbohydrate status in patients with phenylketonuria
por: Couce, María L., et al.
Publicado: (2018)

Food insecurity in females with phenylketonuria
por: Coakley, Kathryn E., et al.
Publicado: (2020)

Genotype‐phenotype correlations and BH(4) estimated responsiveness in patients with phenylketonuria from Rio de Janeiro, Southeast Brazil
por: Vieira Neto, Eduardo, et al.
Publicado: (2019)

A key role for tetrahydrobiopterin‐dependent endothelial NOS regulation in resistance arteries: studies in endothelial cell tetrahydrobiopterin‐deficient mice
por: Chuaiphichai, Surawee, et al.
Publicado: (2017)

Status of nutrients important in brain function in phenylketonuria: a systematic review and meta-analysis
por: Montoya Parra, Gina A., et al.
Publicado: (2018)

Nutritional status of patients with phenylketonuria in Japan
por: Okano, Yoshiyuki, et al.
Publicado: (2016)

Vitamin K Status in Adherent and Non-Adherent Patients with Phenylketonuria: A Cross-Sectional Study
por: Mozrzymas, Renata, et al.
Publicado: (2020)

Central Tetrahydrobiopterin Concentration in Neurodevelopmental Disorders
por: Frye, Richard E.
Publicado: (2010)

Modulation of Radiation Response by the Tetrahydrobiopterin Pathway
por: Pathak, Rupak, et al.
Publicado: (2015)

A requirement for Gch1 and tetrahydrobiopterin in embryonic development
por: Douglas, Gillian, et al.
Publicado: (2015)

Nutrient Status among Latvian Children with Phenylketonuria
por: Lubina, Olga, et al.
Publicado: (2023)

Phenylketonuria
por: Hudson, Frederick
Publicado: (1969)

Cannot write session to /tmp/vufind_sessions/sess_kc85dpisjpjnt5j4n8v8iafmls