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Journal of Nutrition and Metabolism

Journal of Nutrition and Metabolism / 2017 / Article / Tab 4

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 4

Treatment comparison of vitamin and additive nutrient intake distributions in comparison with reference intake cutoffs with AA-MF and GMP-MF1.
VitaminsAA-MFGMP-MF
nPercentile nutrient intakesInadequate and excessive intakes2Percentile nutrient intakesInadequate and excessive intakes
10thMedian90th<EAR or AI>UL10thMedian90th<EAR or AI>UL
Fat-soluble vitamins
Vitamin A, IU303454699617,5720%—2479808916,3203%—0.39
 Vitamin A from MF30026424037—0%022464008—0%0.15
 Vitamin A from MF321154328404063——134822954043——0.60
 Vitamin A from NF4301656506114,6047%—983523213,79510%—0.99
Vitamin D, IU30148562194133%3%36511124333%3%0.58†
 Vitamin D from MF300396960——0485868——0.99†
 Vitamin D from MF24234599960——0500929——0.50†
 Vitamin D from NF3022764490%—33015993%—1.00
Vitamin E, IU3013295513%—7253723%—0.002
 Vitamin E from MF3001828—0%01523—0%0.14†
 Vitamin E from MF21131827——81525——0.01
 Vitamin E from NF306103880%—4102087%—0.16†
Vitamin K, µg301191604123%ND55919039323%ND0.27
 Vitamin K from MF30075112——074131——0.91†
 Vitamin K from MF215575109——4275134——0.75
 Vitamin K from NF302910331147%—2010230853%—0.39
Water-soluble vitamins
Vitamin C, mg30781612763%0%4516429613%0%0.86
 Vitamin C from MF30061130——074129——0.46†
 Vitamin C from MF214262133——4481131——0.19
 Vitamin C from NF30199421237%—277219743%—0.62
Thiamin, mg301.22.56.53%ND0.92.02.713%ND0.03†
 Thiamin from MF3001.33.9——01.01.7——0.01
 Thiamin from MF210.61.33.7——0.61.01.8——0.02
 Thiamin from NF300.51.03.033%—0.40.91.763%—0.15†
Riboflavin, mg301.02.64.010%ND1.82.94.70%ND0.33†
 Riboflavin from MF3001.52.8——1.11.82.9——0.12
 Riboflavin from MF250.71.52.9——1.01.72.8——0.41
 Riboflavin from NF300.51.02.147%—0.40.81.963%—0.31†
Niacin, mg301631653%—1632423%—0.87†
 Niacin from MF3001655—20%71826—0%0.52†
 Niacin from MF2552157——71827——0.68†
 Niacin from NF307132637%—6122543%—0.19
Vitamin B-6, mg301.93.15.73%0%1.83.15.70%0%0.65†
 Vitamin B-6 from MF3001.72.8——1.11.82.9——0.28
 Vitamin B-6 from MF2511.72.9——1.11.82.9——0.62
 Vitamin B-6 from NF300.71.52.827%—0.51.24.337%—0.34†
Folate DFE, µg30504115919023%—593119314613%—0.58†
 Folate DFE from MF3007431507—37%1927331126—27%0.80†
 Folate DFE from MF243828511512——4578001141——0.06
 Folate DFE from NF3016034784740%—11636479043%—0.35
Vitamin B-12, mg302.36.511.810%ND4.17.113.90%ND0.23†
 Vitamin B-12 from MF3005.16.8——2.44.89.4——0.14
 Vitamin B-12 from MF252.25.47.0——2.44.510.8——0.71
 Vitamin B-12 from NF300.11.58.153%—0.21.18.763%—0.71†
Choline, mg307431167463%0%7151286840%0%0.02
 Choline from MF300277628——0430780——0.03
 Choline from MF21111414588——253501806——0.003
 Choline from NF302565109100%—3663172100%—0.15
Pantothenate, mg30491517%ND610167%ND0.47†
 Pantothenate from MF300513——4711——0.21
 Pantothenate from MF253513——4712——0.96
 Pantothenate from NF3013783%—12493%—0.38†
Biotin, mg30186724223%ND2710442813%ND0.14
 Biotin from MF30042160——2367359——0.06
 Biotin from MF252097160——2254403——0.69
 Biotin from NF302612683%—141393%—0.32†
Other
Carnitine from MF, g300.0300.0570.100——<0.001<0.001<0.001——<0.0001†
Taurine from MF, g300.0650.1300.300——0.0850.1600.258——0.27
Inositol from MF, g3000.0800.135——00.0050.048——<0.0001†
1Nutrient intakes were based on 3-day food records (n = 30); statistical analysis included ANOVA with effects for treatment, genotype (classical or variant PKU), and treatment-genotype interaction. The values in this table represent the treatment comparison; 2inadequate and excessive intakes are expressed as a percentage of 30 subjects. United States’ Dietary Reference Intake (DRI) cutoffs, based on the AMDR, EAR, AI, and UL, were compared to nutrient intakes per the sex and age of each individual subject [25, 26]. The United States Dietary Guidelines for Americans 2015–2020 reference cutoffs were used to evaluate dietary saturated fat and sugar intake of subjects [24]; 3subanalyses for micronutrient intakes from medical foods were conducted due to the high rates of use of AA-MF that lacked micronutrient supplementation. These subanalyses aimed to compare micronutrient intakes from medical foods that were supplemented with micronutrients. Due to the diverse micronutrient supplementation profiles of medical foods, sample sizes for each micronutrient vary; 4natural foods were defined as all foods and beverages that were not medical foods intended for the treatment of PKU; 5per the DRI, a UL for select nutrients has not been determined due to a lack of scientific evidence; †Kruskal–Wallis test was used when data were skewed; AMDR, acceptable macronutrient distribution range; AI, adequate intake; AA-MF, amino acid medical food; DFE, dietary folate equivalents; DGA, Dietary Guidelines for Americans; EAR, estimated average requirement; GMP-MF, glycomacropeptide medical food; MF, medical food; ND, not determined; NF, natural food; PKU, phenylketonuria; UL, upper tolerable intake levels.

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