Daly, Anne, Evans, Sharon, Chahal, Satnam, Santra, Saikat, Pinto, Alex, Gingell, Cerys, Rocha, Julio Cesar, van Spronsen, Francjan, Jackson, Richard and MacDonald, Anita
(2019)
The Effect of Glycomacropeptide versus Amino Acids on Phenylalanine and Tyrosine Variability over 24 Hours in Children with PKU: A Randomized Controlled Trial.
NUTRIENTS, 11 (3).
E520-.
Text
The Effect of Glycomacropeptide versus Amino Acids on Phenylalanine and Tyrosine Variability over 24 Hours in Children with PKU: A Randomized Controlled Trial.pdf - Published version Download (1MB) | Preview |
Abstract
<b>Introduction:</b> 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. <b>Aim:</b> To study the impact of CGMP-AA on blood Phe variability using 3 different dietary regimens in children with PKU. <b>Methods:</b> 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. <b>Results:</b> 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 <i>p</i> < 0.0001; R2 vs. R3 <i>p</i> = 0.0009. There was a significant difference in median Phe at each time point between R1 vs. R2, <i>p</i> = 0.0027 and R1 vs. R3, <i>p</i> < 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. <b>Conclusions:</b> 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.
Item Type: | Article |
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Uncontrolled Keywords: | glycomacropeptide, phenylalanine, phenylketonuria, phenylalanine variability, amino acids, tyrosine |
Depositing User: | Symplectic Admin |
Date Deposited: | 05 Jun 2019 09:03 |
Last Modified: | 24 Jan 2024 20:04 |
DOI: | 10.3390/nu11030520 |
Open Access URL: | https://doi.org/10.3390/nu11030520 |
Related URLs: | |
URI: | https://livrepository.liverpool.ac.uk/id/eprint/3044531 |