Abstract
Introduction: In phenylketonuria (PKU), evidence suggests that casein glycomacropeptide supplemented with rate-limiting amino acids (CGMP-AA) is associated with better protein utilisation and less blood phenylalanine (Phe) variability. Aim: To study the impact of CGMP-AA on blood Phe variability using 3 different dietary regimens in children with PKU. Methods: This was a 6-week randomised controlled cross-over study comparing CGMP-AA vs. Phe-free l-amino acids (l-AA) assessing blood Phe and tyrosine (Tyr) variability over 24 h in 19 children (7 boys) with PKU, with a median age of 10 years (6–16). Subjects were randomised to 3 dietary regimens: (1) R1, CGMP-AA and usual dietary Phe (CGMP + Phe); (2) R2, CGMP-AA − Phe content of CGMP-AA from usual diet (CGMP − Phe); and (3) R3, l-AA and usual dietary Phe. Each regimen was administered for 14 days. Over the last 48 h on days 13 and 14, blood spots were collected every 4 h at 08 h, 12 h, 16 h, 20 h, 24 h, and 04 h. Isocaloric intake and the same meal plan and protein substitute dosage at standardised times were maintained when blood spots were collected. Results: Eighteen children completed the study. Median Phe concentrations over 24 h for each group were (range) R1, 290 (30–580), R2, 220 (10–670), R3, 165 (10–640) μmol/L. R1 vs. R2 and R1 vs. R3 p < 0.0001; R2 vs. R3 p = 0.0009. There was a significant difference in median Phe at each time point between R1 vs. R2, p = 0.0027 and R1 vs. R3, p < 0.0001, but not between any time points for R2 vs. R3. Tyr was significantly higher in both R1 and R2 [70 (20–240 μmol/L] compared to R3 [60 (10–200) μmol/L]. In children < 12 years, blood Phe remained in the target range (120–360 μmol/L), over 24 h, for 75% of the time in R1, 72% in R2 and 64% in R3; for children aged ≥ 12 years, blood Phe was in target range (120–600 μmol/L) in R1 and R2 for 100% of the time, but 64% in R3. Conclusions: The residual Phe in CGMP-AA increased blood Phe concentration in children. CGMP-AA appears to give less blood Phe variability compared to l-AA, but this effect may be masked by the increased blood Phe concentrations associated with its Phe contribution. Reducing dietary Phe intake to compensate for CGMP-AA Phe content may help.
Highlights
In phenylketonuria (PKU), evidence suggests that casein glycomacropeptide supplemented with rate-limiting amino acids (CGMP-Arachidonic acid (AA)) is associated with better protein utilisation and less blood phenylalanine (Phe) variability
In phenylketonuria (PKU), there is an inability to metabolise dietary phenylalanine (Phe) into tyrosine (Tyr) due to a deficiency of Phe hydroxylase. It is treated by a low Phe diet, and the major source of protein equivalent is provided by a Phe-free or low Phe protein substitute, in the form of either L-amino acids (L-AA) or casein glycomacropeptide (CGMP-AA)
In R2 and R3 with controlled Phe intake, the only influence on outcomes is the type of protein substitute consumed, and the evidence in this study suggests that CGMP-AA may have a positive physiological impact on reducing variability
Summary
In phenylketonuria (PKU), evidence suggests that casein glycomacropeptide supplemented with rate-limiting amino acids (CGMP-AA) is associated with better protein utilisation and less blood phenylalanine (Phe) variability. In phenylketonuria (PKU), there is an inability to metabolise dietary phenylalanine (Phe) into tyrosine (Tyr) due to a deficiency of Phe hydroxylase It is treated by a low Phe diet, and the major source of protein equivalent is provided by a Phe-free or low Phe protein substitute, in the form of either L-amino acids (L-AA) or casein glycomacropeptide (CGMP-AA). CGMP is a 64-amino acid peptide, found in the whey residue produced as a by-product of cheese production from κ casein This protein is naturally low in Phe as well as other indispensable, large neutral amino acids (histidine, leucine, tryptophan and Tyr). For patients prescribed 60 g/day protein equivalent from this source, it will supply
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