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 2
Daily macronutrient intake profiles in combination with AA-MF and GMP-MF1.
Median and percentile nutrient intakes
AA-MF
GMP-MF
10th
Median
90th
10th
Median
90th
kcal
1471
2076
2711
1523
2148
3152
0.33
kcal/kg
23
29
43
21
30
46
0.33
kcal from MF
162
393
913
340
694
1007
0.002
kcal from NF
969
1597
2247
905
1532
2467
0.53
g protein
61
76
107
56
81
109
0.81
g protein/kg
0.85
1.10
1.50
0.80
1.10
1.70
1.00
g PE from MF
33
59
75
30
53
73
0.98
g PE from MF/kg
0.47
0.78
1.10
0.45
0.76
1.03
0.94
% PE from MF
34%
77%
84%
40%
71%
86%
0.58
g protein from NF
12
20
46
11
22
58
0.88
mg Phe
487
924
1973
544
1014
2592
0.25
mg Phe from MF
0
0
0
50
85
137
0.0001†
mg Phe from NF
487
924
1973
419
929
2524
0.97
g carbohydrate
203
294
406
195
347
473
0.10
g carbohydrate from MF
1
36
125
42
91
166
0.0001†
g carbohydrate from NF
150
237
363
127
240
369
0.52
g sugar/d
67
122
243
78
129
227
0.69
g sugar from MF
0
36
102
22
53
109
0.10†
% energy from MF from sugar
0%
34%
51%
20%
30%
55%
0.18
g sugar from NF
19
89
170
35
69
153
0.94
g fiber
10
17
39
9
18
28
0.93
g fiber from MF
0
0
17
0
2
4
0.67
g fiber from NF
10
15
24
9
16
28
0.64
g fat
37
64
92
35
65
102
0.62
g fat from MF
0
1
20
0
15
26
0.0004
g fat from NF
31
58
82
23
53
93
0.63
g saturated fat
8
15
26
10
21
31
0.046
g saturated fat from MF
0
0
1
0
4
11
0.005
g saturated fat from NF
8
15
25
5
13
28
0.85
g trans fat2
0.1
0.4
1.3
0.03
0.4
1.8
0.66
mg cholesterol2
9
30
118
4
40
136
0.76
1Nutrient intakes were based on 3-day food records (n = 30). Statistical analysis included ANOVA with effects for treatment, genotype, and treatment-genotype interaction. The values in this table represent the treatment comparison; 2AA-MF and GMP-MF did not contain trans fat nor cholesterol; †Kruskal–Wallis test was used when data were skewed; AA-MF, amino acid medical food; GMP-MF, glycomacropeptide medical food; MF, medical food; NF, natural food; PE, protein equivalent.
