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 5
Treatment comparison of mineral intake distributions in comparison with reference intake cutoffs with AA-MF and GMP-MF1.
Minerals
AA-MF
GMP-MF
n
Percentile nutrient intakes
Inadequate and excessive intakes2
Percentile nutrient intakes
Inadequate and excessive intakes
10th
Median
90th
<EAR or AI
>UL
10th
Median
90th
<EAR or AI
>UL
Calcium, mg
30
839
1468
2402
10%
0%
586
1529
2453
20%
7%
1.00†
Calcium from MF
30
106
1103
2077
—
—
63
1153
2225
—
—
0.99
Calcium from MF3
27
552
1151
2129
—
—
60
1255
2391
—
—
0.62†
Calcium from NF4
30
183
313
784
93%
—
156
401
726
90%
—
0.80†
Copper, µg
30
1.2
1.9
4.2
3%
7%
0.6
1.5
2.7
13%
0%
0.0006†
Copper from MF
30
0
1.2
3.0
—
—
0
0.6
1.1
—
—
0.005
Copper from MF
21
0.6
1.2
2.8
—
—
0.4
0.7
1.1
—
—
0.0001
Copper from NF
30
0.5
0.9
3.4
33%
—
0.4
0.8
1.9
40%
—
0.27†
Iodine, µg
30
13
140
235
23%
0%
12
153
262
33%
0%
0.66†
Iodine from MF
30
0
132
223
—
—
0
141
239
—
—
0.48†
Iodine from MF
21
77
137
223
—
—
78
149
241
—
—
0.32
Iodine from NF
30
2
9
42
93%
—
0
8
48
97%
—
0.26
Iron, mg
30
14
25
43
0%
3%
9
23
31
3%
0%
0.02
Iron from MF
30
0
15
31
—
—
0
14
23
—
—
0.14†
Iron from MF
21
10
16
31
—
—
8
14
23
—
—
0.02
Iron from NF
30
5
8
22
37%
—
4
8
17
33%
—
0.15
Magnesium, mg
30
281
501
746
13%
—
196
514
782
23%
—
0.78†
Magnesium from MF
30
32
320
570
—
37%
41
353
578
—
53%
0.77†
Magnesium from MF
27
126
321
570
—
—
38
354
600
—
—
0.80†
Magnesium from NF
30
110
171
320
90%
—
77
165
292
87%
—
0.41
Manganese, mg
30
1.9
3.9
7.1
10%
0%
1.8
3.8
5.3
13%
3%
0.26
Manganese from MF
30
0
1.6
3.4
—
—
0
1.6
3.0
—
—
0.84†
Manganese from MF
21
0.9
1.7
3.4
—
—
0.6
1.9
3.1
—
—
0.41†
Manganese from NF
30
1.1
2.2
6.3
40%
—
0.9
1.9
3.7
50%
—
0.10
Phosphorus, mg
30
952
1616
2431
7%
0%
774
1536
2204
10%
0%
0.60
Phosphorus from MF
30
81
972
2036
—
—
253
965
1708
—
—
0.71†
Phosphorus from MF
27
531
1032
2047
—
—
240
984
1800
—
—
0.26
Phosphorus from NF
30
329
526
1007
73%
—
259
538
1064
67%
—
0.80
Potassium, mg
30
1809
2810
4179
93%
ND5
2014
3350
4540
93%
ND
0.15
Potassium from MF
30
0
600
2558
—
—
718
1146
1781
—
—
0.08†
Potassium from MF
25
60
880
2673
—
—
680
1120
1815
—
—
0.46†
Potassium from NF
30
938
1910
3011
97%
—
1077
1867
3204
100%
—
1.00†
Selenium, µg
30
54
93
133
7%
0%
25
88
142
17%
0%
0.50†
Selenium from MF
30
9
58
89
—
—
0
55
89
—
—
0.34†
Selenium from MF
23
26
59
89
—
—
29
60
93
—
—
0.31
Selenium from NF
30
12
29
88
83%
—
8
27
103
73%
—
0.95
Sodium, mg
30
1816
2637
3879
7%
63%
2052
3261
4645
7%
83%
0.048
Sodium from MF
30
0
413
1175
—
—
580
1140
2040
—
—
<0.0001†
Sodium from MF
25
17
583
1198
—
—
480
1123
2280
—
—
0.0003
Sodium from NF
30
1363
2206
3138
17%
—
1007
2041
3383
23%
—
0.69
Zinc, mg
30
5
16
41
13%
13%
5
13
31
20%
10%
0.12
Zinc from MF
30
0
12
30
—
—
0
9
17
—
—
0.05
Zinc from MF
21
6
12
28
—
—
6
10
17
—
—
0.14†
Zinc from NF
30
3
4
17
77%
—
2
4
19
83%
—
0.64†
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; 2United 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]; 3sub-analyses for micronutrient intakes from medical foods were conducted due to the high rates of use of AA-MF that lacked micronutrient mineral 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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