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.

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 MF300264240370%0224640080%0.15
 Vitamin A from MF3211543284040631348229540430.60
 Vitamin A from NF4301656506114,6047%983523213,79510%0.99
Vitamin D, IU30148562194133%3%36511124333%3%0.58
 Vitamin D from MF30039696004858680.99
 Vitamin D from MF2423459996005009290.50
 Vitamin D from NF3022764490%33015993%1.00
Vitamin E, IU3013295513%7253723%0.002
 Vitamin E from MF30018280%015230%0.14
 Vitamin E from MF21131827815250.01
 Vitamin E from NF306103880%4102087%0.16
Vitamin K, µg301191604123%ND55919039323%ND0.27
 Vitamin K from MF300751120741310.91
 Vitamin K from MF21557510942751340.75
 Vitamin K from NF302910331147%2010230853%0.39

Water-soluble vitamins
Vitamin C, mg30781612763%0%4516429613%0%0.86
 Vitamin C from MF300611300741290.46
 Vitamin C from MF21426213344811310.19
 Vitamin C from NF30199421237%277219743%0.62
Thiamin, mg301.22.56.53%ND0.92.02.713%ND0.03
 Thiamin from MF3001.33.901.01.70.01
 Thiamin from MF210.
 Thiamin from NF300.51.03.033%
Riboflavin, mg301.02.64.010%ND1.82.94.70%ND0.33
 Riboflavin from MF3001.
 Riboflavin from MF250.
 Riboflavin from NF300.51.02.147%
Niacin, mg301631653%1632423%0.87
 Niacin from MF300165520%718260%0.52
 Niacin from MF2552157718270.68
 Niacin from NF307132637%6122543%0.19
Vitamin B-6, mg301.93.15.73%0%
 Vitamin B-6 from MF3001.
 Vitamin B-6 from MF2511.
 Vitamin B-6 from NF300.71.52.827%
Folate DFE, µg30504115919023%593119314613%0.58
 Folate DFE from MF300743150737%192733112627%0.80
 Folate DFE from MF24382851151245780011410.06
 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.
 Vitamin B-12 from MF252.
 Vitamin B-12 from NF300.11.58.153%
Choline, mg307431167463%0%7151286840%0%0.02
 Choline from MF30027762804307800.03
 Choline from MF211114145882535018060.003
 Choline from NF302565109100%3663172100%0.15
Pantothenate, mg30491517%ND610167%ND0.47
 Pantothenate from MF30051347110.21
 Pantothenate from MF25351347120.96
 Pantothenate from NF3013783%12493%0.38
Biotin, mg30186724223%ND2710442813%ND0.14
 Biotin from MF3004216023673590.06
 Biotin from MF25209716022544030.69
 Biotin from NF302612683%141393%0.32

Carnitine from MF, g300.0300.0570.100<0.001<0.001<0.001<0.0001
Taurine from MF, g300.0650.1300.3000.0850.1600.2580.27
Inositol from MF, g3000.0800.13500.0050.048<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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