ClinicalStudy
Comparison of Glycomacropeptide with Phenylalanine
Free-SyntheticAminoAcidsinTestMealstoPKUPatients:
No Significant Differences in Biomarkers, Including Plasma
Phe Levels
KirstenK.Ahring ,
1,2,3
AllanM.Lund,
2,3
ErikJensen,
4
ThomasG.Jensen,
5
Karen Brøndum-Nielsen,
1
Michael Pedersen,
6
AllanBardow,
7
JensJuulHolst ,
8
JensF.Rehfeld,
9
andLisbethB.Møller
2
1
e PKU Clinic, Kennedy Centre, Centre for Paediatric and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet,
Copenhagen, enmark
2
epartment of Clinical Genetics, Applied Human Molecular Genetics, Kennedy Center, Rigshospitalet, enmark
3
Centre for Inherited Metabolic iseases, Centre for Paediatric and Adolescent Medicine, Copenhagen University Hospital,
Rigshospitalet, Copenhagen, enmark
4
Arla Foods Ingredients Group P/S, Viby J, enmark
5
epartment of Biomedicine, Aarhus University, Aarhus, enmark
6
epartment of Clinical Medicine, Aarhus University Hospital, Aarhus, enmark
7
epartment of Odontology, Copenhagen University, Copenhagen, enmark
8
Institute of Clinical Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, enmark
9
epartment of Clinical Biochemistry, University of Copenhagen, Rigshospitalet, Copenhagen, enmark
Correspondence should be addressed to Kirsten K. Ahring; kka@kennedy.dk
Received 14 July 2017; Accepted 11 October 2017; Published 8 January 2018
Academic Editor: Ina Knerr
Copyright © 2018 Kirsten K. Ahring et al. is is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Introduction. Management of phenylketonuria (PKU) is achieved through low-phenylalanine (Phe) diet, supplemented with low-
protein food and mixture of free-synthetic (FS) amino acid (AA). Casein glycomacropeptide (CGMP) is a natural peptide released
in whey during cheese-making and does not contain Phe. Lacprodan
®
CGMP-20 used in this study contained a small amount
of Phe due to minor presence of other proteins/peptides. Objective. e purpose of this study was to compare absorption of
CGMP-20 to FSAA with the aim of evaluating short-term effects on plasma AAs as well as biomarkers related to food intake.
Methods. is study included 8 patients, who had four visits and tested four drink mixtures (DM1–4), consisting of CGMP, FSAA,
or a combination. Plasma blood samples were collected at baseline, 15, 30, 60, 120, and 240 minutes (min) after the meal. AA
profiles and ghrelin were determined 6 times, while surrogate biomarkers were determined at baseline and 240 min. A visual
analogue scale (VAS) was used for evaluation of taste and satiety. Results. e surrogate biomarker concentrations and VAS
scores for satiety and taste were nonsignificant between the four DMs, and there were only few significant results for AA profiles
(not Phe). Conclusion. CGMP and FSAA had the overall same nonsignificant short-term effect on biomarkers, including Phe.
is combination of FSAA and CGMP is a suitable supplement for PKU patients.
1.Introduction
Phenylketonuria (PKU) is an inborn error of metabolism. If
left untreated, severe brain damage will occur [1–3]. e
primary aim of treatment of those suffering from PKU is to
control the blood phenylalanine (Phe) concentration in
order to prevent neurological damage [2]. PKU treatment is
based on a low-protein (LP) diet in combination with free-
synthetic (FS) amino acid (AA) supplements without Phe
and enriched with vitamins, minerals, trace elements, and in
Hindawi
Journal of Nutrition and Metabolism
Volume 2018, Article ID 6352919, 11 pages
https://doi.org/10.1155/2018/6352919