A5015781343- Metabolism and Genetic Disorders
- Diet and metabolism studies
- Amino Acid Enzymes and Metabolism
- Folate and B Vitamins Research
- Muscle metabolism and nutrition
- Pharmacological Effects and Toxicity Studies
- Neonatal Health and Biochemistry
- Mitochondrial Function and Pathology
- Biochemical and Molecular Research
- Clinical Nutrition and Gastroenterology
- Genomics and Rare Diseases
- Peroxisome Proliferator-Activated Receptors
- Trace Elements in Health
- Hormonal and reproductive studies
- Genetic Syndromes and Imprinting
- Iron Metabolism and Disorders
- Metabolomics and Mass Spectrometry Studies
- Selenium in Biological Systems
- Intestinal Malrotation and Obstruction Disorders
- Hyperglycemia and glycemic control in critically ill and hospitalized patients
- Cancer, Hypoxia, and Metabolism
- Nutrition, Genetics, and Disease
- Digestive system and related health
- Neurogenetic and Muscular Disorders Research
- Body Composition Measurement Techniques
University of Pittsburgh
2014
Nutricia Research (Netherlands)
2011
Children's Hospital Colorado
1991-2011
University of Colorado Denver
1991-2011
Ross Laboratories (United States)
1994-2006
Abbott (United States)
1995-2003
The Ohio State University
2002
Abbott (Sweden)
1999
Abbott (Germany)
1995
Boston Children's Hospital
1993
Growth and metabolic status of 25 infants with PKU were evaluated based on protain intake. Food A‐fed received a medical food containing 3.12g protein equivalent per 100 kcal B‐fed 2.74 g kcal. percentiles in the A group significantly greater than those for B at 6 months age (Food percentiles: crown‐heel length 55, head circumference 60, weight 73. 28; 29, 39). At study entrance, only two groups differed; had mean percentile ( p < 0.05). Mean phenylalanine (PHE) intake was 38% by infants....
There are inconsistent reports on the lactose and/or galactose content of some foods traditionally restricted from diet for classic galactosemia. Therefore, samples cheeses, caseinates, and canned black, pinto, kidney, garbanzo beans were analyzed free using HPLC with refractive index or pulsed amperometric detection. Galactose concentrations in several hard aged cheeses three mild/medium Cheddars, produced by smaller local dairies, was <10 mg/100 g sample compared to 55.4 four sharp...
Because of reports poor growth, a study was conducted for 6 months in 35 infants with classic phenylketonuria diagnosed during the neonatal period who were fed Phenex-1 Amino Acid Modified Medical Food With Iron (Ross Products Division, Columbus, OH, U.S.A.).as their primary protein source.Diet diaries and anthropometric measures obtained monthly as part larger which nutrition status evaluated.In 6-month-old infants, mean percentiles crown-heel length (59.14+/-4.31 SEM), head circumference...
Background Investigators in Italy and Spain have suggested that therapy for patients with phenylketonuria (PKU) may result essential fatty acid (EFA) deficiency. Objectives of this study were to determine if the diets PKU United States provided adequate EFA intakes whether could form long-chain polyunsaturated acids. Methods Patients (1–13 years age) classic undergoing their non-PKU sibling closest age compared. Nutrient calculated from 3-day diet diaries. Fatty acids plasma erythrocytes...
The objectives of this study were to determine if Phenex-1, amino-acid modified medical food with iron maintained normal indices protein status in infants phenylketonuria (PKU) and investigate factors that influence plasma amino acid concentrations.A was conducted for six months 35 classical PKU diagnosed the neonatal period. Diet diaries concentrations obtained monthly. Blood analysis albumin, blood urea nitrogen (BUN), retinol binding (RBP) transthyretin at one, three study.Mean (+/-SEM)...
Glutaric aciduria type 1 (GA-1) is a cerebral organic characterized by striatal injury and progressive movement disorder. Nutrition management shifted from general restriction of intact protein to targeted lysine tryptophan. Recent guidelines advocate for low-lysine diet using lysine-free, tryptophan-reduced medical foods. GA-1 guideline recommendations dietary patients over the age six are unclear, ranging avoiding excessive intake counting milligrams intake. A 22–question survey on...
Adequacy of nutrient intakes adolescents with and without phenylketonuria (PKU) infants children maple syrup urine disease (MSUD) were assessed using 3-day diet records sorted by age the subject. Mean all nutrients greater than two-thirds Recommended Dietary Allowances (RDA) or Estimated Safe Adequate Daily Intakes (ESADDI) for studied, exception selenium (Se) in PKU adolescents, which averaged 27.8 micrograms. For PKU, > 50% RDA ESADDI was provided elemental modified protein hydrolysate...