Pinto, Alex;
Daly, Anne;
Newby, Camille;
Robotham, Abigail;
Heales, Simon;
Eaton, Simon;
Aitkenhead, Helen;
... MacDonald, Anita; + view all
(2024)
The effects of casein glycomacropeptide on general health status in children with PKU: A randomized crossover trial.
Molecular Genetics and Metabolism
, 143
(4)
, Article 108607. 10.1016/j.ymgme.2024.108607.
(In press).
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Abstract
In PKU, it is suggested that casein glycomacropeptide based protein substitute (GMP) may have physiological advantage when satiety, oxidative stress, renal function and inflammation are considered. Its prebiotic properties may also help gastrointestinal (GI) tolerance. In children with PKU, a randomized/crossover trial comparing phenylalanine-free amino acids (AA) vs GMP as the single source of protein substitute for 12-weeks in each arm was conducted. There was a 4-week wash out period with AA in-between. At baseline and end of each intervention, blood and fecal samples were taken to monitor gut health, oxidative stress, renal function, inflammatory markers and plasma amino acids. Satiety and Pediatric Quality of Life (PedsQL) GI symptoms questionnaires were completed. Usual weekly blood spots for phenylalanine and tyrosine were done. Twelve patients (8 males; aged 4-9y) with PKU participated. GMP improved the following GI symptoms: stomach pain (p = 0.003), heartburn and reflux (p = 0.041) wind and bloating (p = 0.018). With GMP, there was also a trend for less constipation (p = 0.068), discomfort with eating (p = 0.065) and nausea and vomiting (p = 0.087). There were no changes on stool gut health markers (IgA, short chain fatty acids and fecal calprotectin). There were no statistically significant differences for renal, oxidative stress, inflammatory and gut health markers or measures of satiety except for adiponectin (p = 0.028) and total antioxidant capacity (p = 0.049), although the latter was possibly without clinical significance. Mean dried blood spot phenylalanine (Phe) was 114 μmol/L higher with GMP vs AA (p < 0.001). There was no difference in tyrosine levels. In conclusion, GI symptoms statistically significantly improved with GMP versus AA. The Phe content of GMP may present challenges when it is used as the only protein substitute in children with classical PKU with low Phe tolerance.
| Type: | Article |
|---|---|
| Title: | The effects of casein glycomacropeptide on general health status in children with PKU: A randomized crossover trial |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1016/j.ymgme.2024.108607 |
| Publisher version: | http://dx.doi.org/10.1016/j.ymgme.2024.108607 |
| Language: | English |
| Additional information: | © 2024 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). |
| Keywords: | Phenylketonuria, Glycomacropeptide, Amino acids, Gastrointestinal symptoms, Renal function, Oxidative stress, Inflammation, Gut health, Satiety |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Biology and Cancer Dept |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/10200626 |
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