TY - JOUR
T1 - 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
AU - Stroup, Bridget M.
AU - Ney, Denise M.
AU - Murali, Sangita G.
AU - Rohr, Frances
AU - Gleason, Sally T.
AU - Van Calcar, Sandra C.
AU - Levy, Harvey L.
N1 - Funding Information:
Denise M. Ney is a coinventor on U.S. Patent 8,604,168 B2, “Glycomacropeptide Medical Foods for Nutritional Management of Phenylketonuria and Other Metabolic Disorders,” which is held by the Wisconsin Alumni Research Foundation and licensed to Cambrooke *erapeutics, LLC. Denise M. Ney is a consultant to Arla Foods Ingredients and Agropur. Fran Rohr has received consulting fees from Cambrooke *erapeutics.
Funding Information:
*e authors would like to thank Nivi Nair for her contributions to the data collection of this study and Dr. Katarzyna Broniowska from Metabolon, Inc., for assistance in interpretation of metabolomic analyses. *is work was supported by the Department of Health and Human Services grant R01 FD003711 from the FDA OXce of Orphan Products Development to Ney, P30-HD-03352, and by the Clinical and Translational Science Award (CTSA) program, through the NIH National Center for Advancing Translational Sciences (NCATS) grant UL1TR000427. Cam-brooke *erapeutics, Inc., and Arla Foods Ingredients donated funds for the metabolomic analyses.
Publisher Copyright:
© 2017 Bridget M. Stroup et al.
PY - 2017
Y1 - 2017
N2 - 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.
AB - 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.
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U2 - 10.1155/2017/6859820
DO - 10.1155/2017/6859820
M3 - Article
AN - SCOPUS:85042669071
VL - 2017
JO - Journal of Nutrition and Metabolism
JF - Journal of Nutrition and Metabolism
SN - 2090-0724
M1 - 6859820
ER -