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

• Published in: Journal of nutrition and metabolism Vol. 2017; no. 2017; pp. 1 - 17
• Main Authors: Levy, Harvey L., Gleason, Sally T., Rohr, Frances, Murali, Sangita G., Ney, Denise M., Stroup, Bridget M., van Calcar, Sandra C.
• Format: Journal Article
• Language: English
• Published: Cairo, Egypt Hindawi Publishing Corporation 01.01.2017; Hindawi; Hindawi Limited; Wiley
• Subjects: Adolescents; Adults; Amino acids; Analysis; Bioavailability; Choline; Clinical Study; Diet; Dietary intake; Dietary minerals; Dietary supplements; DNA methylation; Evaluation; Fasting; Fatty acids; Folic acid; Food; Intelligence gathering; Metabolic disorders; Metabolomics; Micronutrients; Nutrient deficiency; Nutrient status; Nutrition research; Nutritional status; Phenylalanine; Phenylketonuria; Proteins; Sapropterin dihydrochloride; Selenium; Sodium; Sugar; Urine; Vitamins; Youth
• ISSN: 2090-0724; 2090-0732
• DOI: 10.1155/2017/6859820

Background. Nutrient status in phenylketonuria (PKU) requires surveillance due to the restrictive low-Phe diet in combination with amino acid medical foods (AA-MF) or glycomacropeptide medical foods (GMP-MF). Micronutrient profiles of medical foods are diverse, and optimal micronutrient supplementation in PKU has not been established. Methods. In a crossover design, 30 participants with PKU were randomized to consume AA-MF and Glytactin™ GMP-MF in combination with a low-Phe diet for 3 weeks each. Fasting venipunctures, medical food logs, and 3-day food records were obtained. Metabolomic analyses were completed in plasma and urine by Metabolon, Inc. Results. The low-Phe diets in combination with AA-MF and GMP-MF were generally adequate based on Dietary Reference Intakes, clinical measures, and metabolomics. Without micronutrient supplementation of medical foods, >70% of participants would have inadequate intakes for 11 micronutrients. Despite micronutrient supplementation of medical foods, inadequate intakes of potassium in 93% of participants and choline in >40% and excessive intakes of sodium in >63% of participants and folic acid in >27% were observed. Sugar intake was excessive and provided 27% of energy. Conclusions. Nutrient status was similar with AA-MF and Glytactin GMP-MF. More research related to micronutrient supplementation of medical foods for the management of PKU is needed.