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The American Journal of Clinical Nutrition, ISSN 0002-9165, 8/2016, Volume 104, Issue 2, pp. 334 - 345
Background: To prevent cognitive impairment, phenylketonuria requires lifelong management of blood phenylalanine (Phe) concentration with a low-Phe diet. The... 
5010 | medical food | inborn errors of amino acid metabolism | phenylalanine | threonine | tyrosine | 5009 | Energy and Protein Metabolism | sapropterin dihydrochloride | executive function
Journal Article
Journal of Parenteral and Enteral Nutrition, ISSN 0148-6071, 01/2019, Volume 43, Issue 1, pp. 70 - 80
Background Identification of patients at risk for malnutrition is important for timely nutrition intervention to reduce morbidity and mortality. Objective: The... 
length of hospital stay | Nutrition Focused Physical Assessment | specificity | malnutrition | Nutrition Focused Physical Exam | sensitivity | DEFINITION | HOSPITALIZED-PATIENTS | DIETETICS | ACADEMY | NUTRITION & DIETETICS | SUBJECTIVE GLOBAL ASSESSMENT | AMERICAN SOCIETY | TOOLS
Journal Article
Journal of Urology, The, ISSN 0022-5347, 2012, Volume 187, Issue 4, pp. e907 - e907
Journal Article
Molecular Genetics and Metabolism Reports, ISSN 2214-4269, 06/2018, Volume 15, pp. 30 - 35
Low bone mineral density (BMD) and subsequent skeletal fragility have emerged as a long-term complication of phenylketonuria (PKU). To determine if there are... 
Osteoporosis | Renal net acid | Amino acid | Glycomacropeptide | Trabecular bone score | Medical food | Urinary calcium excretion | Appendicular lean mass index | PROTEIN | NUTRITION MANAGEMENT | CHILDREN | METABOLISM | RECOMMENDATIONS | AMINO-ACIDS | OSTEOPENIA | GENETICS & HEREDITY | FRACTURES
Journal Article
Journal of nutrition and metabolism, ISSN 2090-0724, 2017, Volume 2017, p. 6859820
Nutrient status in phenylketonuria (PKU) requires surveillance due to the restrictive low-Phe diet in combination with amino acid medical foods (AA-MF) or... 
Journal Article
PLOS ONE, ISSN 1932-6203, 10/2016, Volume 11, Issue 10, p. e0163234
Background Glycomacropeptide (GMP) is a 64-amino acid glycophosphopeptide released from kappa-casein during cheesemaking that promotes satiety, reduces body... 
SKELETAL-MUSCLE | ABDOMINAL ADIPOSITY | MINERAL DENSITY | OBESITY | INSULIN-RESISTANCE | MULTIDISCIPLINARY SCIENCES | WHEY-PROTEIN | BOVINE GLYCOMACROPEPTIDE | BODY-MASS | ENERGY-INTAKE | RECEPTOR-ALPHA | Obesity - diet therapy | Oxidation-Reduction | Humans | Peptide Fragments - administration & dosage | Femur - drug effects | Obesity - physiopathology | Caseins - administration & dosage | Obesity - metabolism | Biomechanical Phenomena | Bone Density - drug effects | Phenotype | Animals | Body Composition - drug effects | Dietary Fats - administration & dosage | Female | Mice | Fatty Acids - metabolism | Energy Metabolism - drug effects | Femur - growth & development | Body weight | Dietary fat | Amino acids | Hostages | Fatty acids | Density | Casein | Analysis | Physiological aspects | Oxidation-reduction reaction | Bones | Genetic aspects | Femur | Adipose tissue | Body fat | Ductility | Liver | Biochemistry | Hormones | Males | Proteins | Genotype & phenotype | Protein composition | Mineralization | Animal tissues | Food composition | Adipose tissue (brown) | Bone strength | Bone composition | Obesity | Cytokines | Energy measurement | Bioavailability | Metabolism | Abdomen | Bone mineral content | Bone mass | Diet | Glycomacropeptide | Food intake | Osteogenesis imperfecta | Weaning | Kinases | Body composition | Body composition (biology) | Rodents | Oxidation | Gastrocnemius muscle | Food | Dual energy X-ray absorptiometry | Muscles | Inflammation | Brittleness | Feeding | Fracture toughness | Biomechanics | Bone mineral density | Bone | Females
Journal Article
Data in brief, ISSN 2352-3409, 08/2017, Volume 13, p. 377
This article provides original data on median dietary intake of 18 amino acids from amino acid medical foods, glycomacropeptide medical foods, and natural... 
Journal Article
American Journal of Clinical Nutrition, ISSN 0002-9165, 08/2016, Volume 104, Issue 2, pp. 334 - 345
Journal Article
Journal Article
The Journal of nutrition, ISSN 0022-3166, 02/2018, Volume 148, Issue 2, pp. 194 - 201
Background: Individuals with phenylketonuria (PKU) have a risk of cognitive impairment and inflammation. Many follow a low-phenylalanine (low-Phe) diet devoid... 
MEDICAL FOODS | MICROBIOTA METABOLISM | GLYCOMACROPEPTIDE | deoxycarnitine | GUT MICROBIOTA | trimethylamine N-oxide | NUTRITION MANAGEMENT | CHILDREN | SUPPLEMENTATION | BIOMARKERS | dihomo-gamma-linolenic acid | eicosapentaenoic acid | NUTRITION & DIETETICS | PLASMA | hydroxy 3-methylglutarate | inflammation | docosahexaenoic acid | trimethylamine | acylcarnitines | gamma-linolenic acid | PHENYLALANINE-HYDROXYLASE DEFICIENCY | Metabolomics | Biomarkers - urine | Phenylketonurias - diet therapy | Humans | Middle Aged | Male | Carnitine - metabolism | Gastrointestinal Microbiome - physiology | Amino Acids - administration & dosage | Erythrocytes - chemistry | Caseins - administration & dosage | Fatty Acids - administration & dosage | Adult | Female | Betaine - blood | Fatty Acids - blood | Methylamines - urine | Carnitine - administration & dosage | Fatty Acids, Essential - metabolism | Fasting | Peptide Fragments - administration & dosage | Biomarkers - analysis | Carnitine - blood | Lipid Metabolism | Biomarkers - blood | Cholesterol - metabolism | Betaine - analogs & derivatives | Cross-Over Studies | Adolescent | Phenylketonurias - metabolism | Dietary Supplements | Phenylketonuria | Care and treatment | Dosage and administration | Bioavailability | Lipid metabolism | Research | Low-phenylalanine diet | Methods | Carnitine | Nutrient Physiology, Metabolism, and Nutrient-Nutrient Interactions | 3-hydroxy 3-methylglutarate | dihomo-γ-linolenic acid
Journal Article
Genetics in Medicine, ISSN 1098-3600, 2019, Volume 21, Issue 9, pp. 1977 - 1986
Purpose: Untargeted metabolomic analysis is increasingly being used in the screening and management of individuals with inborn errors of metabolism (IEM). We... 
arginase deficiency | branched-chain amino acids | metabolomics | urea cycle disorder | guanidino compounds | CEREBROSPINAL-FLUID | CEREBRAL-CORTEX | DEFICIENCY | INBORN-ERRORS | THERAPY | PLASMA | URINE | GENETICS & HEREDITY | ARGININE | MUTATIONS | Index Medicus | Metabolomics
Journal Article
Molecular Genetics and Metabolism, ISSN 1096-7192, 06/2017, Volume 121, Issue 2, pp. 96 - 103
Deficiencies of the monoamine neurotransmitters, such as dopamine synthesized from Tyr and serotonin synthesized from Trp, are of concern in PKU. Our objective... 
Catecholamines | 6-sulfatoxymelatonin | Phenol sulfate | Serotonin | Glycomacropeptide | Intestinal microbiota | MEDICINE, RESEARCH & EXPERIMENTAL | BACTERIA | NUTRITIONAL MANAGEMENT | GUT MICROBIOTA | MODEL | DIET | AMINES | ENDOCRINOLOGY & METABOLISM | GENETICS & HEREDITY | PHENYLALANINE | BRAIN | Amino Acids - blood | Humans | Middle Aged | Caseins - metabolism | Biological Availability | Male | Catecholamines - urine | Tryptophan - administration & dosage | Amino Acids - administration & dosage | Tryptophan - metabolism | Caseins - administration & dosage | Young Adult | Amino Acids - metabolism | Serotonin - blood | Caseins - blood | Peptide Fragments - blood | Adult | Female | Kynurenine - metabolism | Peptide Fragments - metabolism | Peptide Fragments - administration & dosage | Gastrointestinal Microbiome | Phenylketonurias - urine | Food, Formulated | Metabolomics - methods | Prebiotics | Cross-Over Studies | Melatonin - analogs & derivatives | Phenylketonurias - blood | Tyrosine - metabolism | Diet | Melatonin - urine | Metabolic Networks and Pathways | Serotonin - metabolism | Adolescent | Phenylketonurias - metabolism | Tyrosine - administration & dosage | Tyrosine | Tryptophan | Physiological aspects | Phenols | Analysis | Amino acids | Phenylketonuria | Microbiota (Symbiotic organisms) | glycomacropeptide | intestinal microbiota | catecholamines | serotonin | phenol sulfate
Journal Article
Journal of Nutrition and Metabolism, ISSN 2090-0724, 2017, Volume 2017, pp. 1909101 - 12
. Skeletal fragility is a complication of phenylketonuria (PKU). A diet containing amino acids compared with glycomacropeptide reduces bone size and strength... 
Complications and side effects | Phenylketonuria | Care and treatment | Functional foods | Nutritional aspects | Physiological aspects | Amino acids | Research | Urine | Load | Calcium | Dietary minerals | Clinical trials | Bioavailability | Metabolism | Studies | Proteins | Medicine | Diet | Rodents | Bone density | Magnesium | Public health | Endocrinology | Food | Clinical Study
Journal Article
01/2017, ISBN 9780355104660
Phenylketonuria (PKU; OMIM 261600) is autosomal recessive inborn error of Phe metabolism that results in a loss of function of hepatic phenylalanine... 
Medicine | Nutrition | Biochemistry
Dissertation
Data in Brief, ISSN 2352-3409, 08/2017, Volume 13, pp. 377 - 384
This article provides original data on median dietary intake of 18 amino acids from amino acid medical foods, glycomacropeptide medical foods, and natural... 
Tyrosine | Executive function | Leucine | Delis-Kaplan Executive Function System | Arginine | Cambridge Neuropsychological Test Automated Battery
Journal Article