Clinical Study

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

Table 6

Main study conclusions.

(1)Similar total dietary intakes of most micronutrients when participants consumed AA-MF or Glytactin GMP-MF and no differences in intakes of micronutrients from natural foods were observed. Thus, differences in micronutrient intakes were driven by the diverse micronutrient supplementation profiles of the medical foods.

(2)Participants obtained adequate intakes (≥EAR) of most micronutrients. However, inadequate intakes (i.e., <EAR) of potassium for 93% of participants and choline for >40% of participants were observed.

(3)Participants had excessive intakes (>UL) of chemically derived folic acid and magnesium from medical foods, and >63% of participants had excessive intakes of sodium driven by natural (likely processed) food intake. Average sugar intake as a percentage of energy was 27% and was excessive (>DGA) for 97% of participants.

(4)Without micronutrient supplementation of medical foods, >70% of participants would have inadequate intakes (≤EAR) for 11 micronutrients (biotin, choline, pantothenate, vitamins D and E, potassium, calcium, iodine, magnesium, selenium, and zinc). Greater than 90% of participants would obtain adequate intake (≥EAR) of vitamin A from natural foods alone due to high intakes of provitamin A carotenoids from green leafy vegetables, squashes, carrots, and tomatoes.

(5)Of 30 participants, only 13 consumed MLPF with both AA-MF and GMP-MF treatments. MPLF comprised approximately 10% of median calories.

(6)Increased urinary excretion of sulfate, taurine, and taurine-related metabolites with AA-MF may be related to increased need to excrete sulfur with higher dietary intake of sulfur-containing amino acids, Met and Cys, from AA-MF compared with Glytactin GMP-MF.