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 3

Modified low-protein daily food 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
Energy, kcal/d	73	211	463	85	257	690	0.31
Protein, g/d	0.1	0.6	1.3	0.2	0.4	1.6	0.71
Phe, mg/d	3	13	60	3	20	93	0.32
Carbohydrate, g/d	17	51	103	14	62	125	0.49^‡
Fiber, g/d	0	0.3	5	0	1	6	0.13
Fat, g/d	0	1	5	0.5	3	18	0.13^‡
Saturated fat, g/d	0	0.2	2	0	0.5	4	0.25^‡

¹Nutrient intakes from MLPF were based on 3-day food records (n = 13; classical PKU, n = 10; variant PKU, n = 3). Participants that did not consume MLPF at the end of both treatments were removed from the analysis. MLPF were defined as foods modified to be low in protein and specifically made for individuals with disorders that have dietary protein restrictions. Statistical analysis included ANOVA with effects for treatment, genotype, and treatment-genotype interaction. The values in this table represent the treatment comparison. There were no significant differences due to genotype or the treatment-genotype interaction (data not shown); ^‡Kruskal–Wallis test was used when data were skewed; MLPF, modified low-protein foods.