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
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 supplementat...
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Wiley
2017-01-01
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| Series: | Journal of Nutrition and Metabolism |
| Online Access: | http://dx.doi.org/10.1155/2017/6859820 |
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| author | Bridget M. Stroup Denise M. Ney Sangita G. Murali Frances Rohr Sally T. Gleason Sandra C. van Calcar Harvey L. Levy |
| author_facet | Bridget M. Stroup Denise M. Ney Sangita G. Murali Frances Rohr Sally T. Gleason Sandra C. van Calcar Harvey L. Levy |
| author_sort | Bridget M. Stroup |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-c0f4f2280b594aa68f864c659e78b6db |
| institution | Kabale University |
| issn | 2090-0724 2090-0732 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of Nutrition and Metabolism |
| spelling | doaj-art-c0f4f2280b594aa68f864c659e78b6db2025-02-03T06:44:47ZengWileyJournal of Nutrition and Metabolism2090-07242090-07322017-01-01201710.1155/2017/68598206859820Metabolomic Insights into the Nutritional Status of Adults and Adolescents with Phenylketonuria Consuming a Low-Phenylalanine Diet in Combination with Amino Acid and Glycomacropeptide Medical FoodsBridget M. Stroup0Denise M. Ney1Sangita G. Murali2Frances Rohr3Sally T. Gleason4Sandra C. van Calcar5Harvey L. Levy6Department of Nutritional Sciences, University of Wisconsin-Madison, Madison, WI, USADepartment of Nutritional Sciences, University of Wisconsin-Madison, Madison, WI, USADepartment of Nutritional Sciences, University of Wisconsin-Madison, Madison, WI, USABoston Children’s Hospital, Harvard Medical School, Boston, MA, USADepartment of Nutritional Sciences, University of Wisconsin-Madison, Madison, WI, USADepartment of Molecular and Medical Genetics, School of Medicine, Oregon Health and Science University, Portland, OR, USABoston Children’s Hospital, Harvard Medical School, Boston, MA, USABackground. 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.http://dx.doi.org/10.1155/2017/6859820 |
| spellingShingle | Bridget M. Stroup Denise M. Ney Sangita G. Murali Frances Rohr Sally T. Gleason Sandra C. van Calcar Harvey L. Levy 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 Journal of Nutrition and Metabolism |
| title | 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 |
| title_full | 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 |
| title_fullStr | 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 |
| title_full_unstemmed | 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 |
| title_short | 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 |
| title_sort | 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 |
| url | http://dx.doi.org/10.1155/2017/6859820 |
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