Comparison of Glycomacropeptide with Phenylalanine Free-Synthetic Amino Acids in Test Meals to PKU Patients: No Significant Differences in Biomarkers, Including Plasma Phe Levels

被引:16
作者
Ahring, Kirsten K. [1 ,2 ,3 ]
Lund, Allan M. [2 ,3 ]
Jensen, Erik [4 ]
Jensen, Thomas G. [5 ]
Brondum-Nielsen, Karen [1 ]
Pedersen, Michael [6 ]
Bardow, Allan [7 ]
Holst, Jens Juul [8 ]
Rehfeld, Jens F. [9 ]
Moller, Lisbeth B. [2 ]
机构
[1] Rigshosp, Copenhagen Univ Hosp, Ctr Paediat & Adolescent Med, PKU Clin,Kennedy Ctr, Copenhagen, Denmark
[2] Rigshosp, Kennedy Ctr, Appl Human Mol Genet, Dept Clin Genet, Copenhagen, Denmark
[3] Rigshosp, Copenhagen Univ Hosp, Ctr Paediat & Adolescent Med, Ctr Inherited Metab Dis, Copenhagen, Denmark
[4] Arla Foods Ingredients Grp PS, Viby J, Denmark
[5] Aarhus Univ, Dept Biomed, Aarhus, Denmark
[6] Aarhus Univ Hosp, Dept Clin Med, Aarhus, Denmark
[7] Univ Copenhagen, Dept Odontol, Copenhagen, Denmark
[8] Rigshosp, Copenhagen Univ Hosp, Inst Clin Med, Copenhagen, Denmark
[9] Univ Copenhagen, Rigshosp, Dept Clin Biochem, Copenhagen, Denmark
关键词
D O I
10.1155/2018/6352919
中图分类号
R15 [营养卫生、食品卫生]; TS201 [基础科学];
学科分类号
100403 ;
摘要
Introduction. Management of phenylketonuria (PKU) is achieved through low-phenylalanine (Phe) diet, supplemented with lowprotein 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 (R) CGMP-20 used in this study contained a small amount of Phe due to minor presence of other proteins/peptides. Objective The 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 The 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. This combination of FSAA and CGMP is a suitable supplement for PKU patients.
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页数:11
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共 46 条
  • [1] ABX H., 2008, DIAGNOSTIC TEST GLUC
  • [2] Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach
    Aguiar, A.
    Ahring, K.
    Almeida, M. F.
    Assoun, M.
    Belanger Quintana, A.
    Bigot, S.
    Bihet, G.
    Malmberg, K. Blom
    Burlina, A.
    Bushueva, T.
    Caris, A.
    Chan, H.
    Clark, A.
    Clark, S.
    Cochrane, B.
    Corthouts, K.
    Dalmau, J.
    Dassy, M.
    De Meyer, A.
    Didycz, B.
    Diels, M.
    Dokupil, K.
    Dubois, S.
    Eftring, K.
    Ekengren, J.
    Ellerton, C.
    Evans, S.
    Faria, A.
    Fischer, A.
    Ford, S.
    Freisinger, P.
    Gizewska, M.
    Gokmen-Ozel, H.
    Gribben, J.
    Gunden, F.
    Heddrich-Ellerbrok, M.
    Heiber, S.
    Heidenborg, C.
    Jankowski, C.
    Janssen-Regelink, R.
    Jones, I.
    Jonkers, C.
    Joerg-Streller, M.
    Kaalund-Hansen, K.
    Kiss, E.
    Lammardo, A. M.
    Lang, K.
    Lier, D.
    Lilje, R.
    Lowry, S.
    [J]. MOLECULAR GENETICS AND METABOLISM, 2015, 115 (01) : 17 - 22
  • [3] Andreasen S., 2015, GHRELIN ELISA KIT
  • [4] [Anonymous], 2016, SPECIAL ASSAYS MONIT
  • [5] [Anonymous], 2014, DIAGNOSTIC TEST BLOO
  • [6] LAT1 gene variants -: potential factors influencing the clinical course of phenylketonuria
    Bik-Multanowski, Miroslaw
    Pietrzyk, Jacek J.
    [J]. JOURNAL OF INHERITED METABOLIC DISEASE, 2006, 29 (05) : 684 - 684
  • [7] Genetics of Phenylketonuria: Then and Now
    Blau, Nenad
    [J]. HUMAN MUTATION, 2016, 37 (06) : 508 - 515
  • [8] Casein peptide release and passage to the blood in humans during digestion of milk or yogurt
    Chabance, B
    Marteau, P
    Rambaud, JC
    Migliore-Samour, D
    Boynard, M
    Perrotin, P
    Guillet, R
    Jollès, P
    Fiat, AM
    [J]. BIOCHIMIE, 1998, 80 (02) : 155 - 165
  • [9] Phenylketonuria: nutritional advances and challenges
    Giovannini, Marcello
    Verduci, Elvira
    Salvatici, Elisabetta
    Paci, Sabrina
    Riva, Enrica
    [J]. NUTRITION & METABOLISM, 2012, 9
  • [10] MUTATIONS IN THE PHENYLALANINE-HYDROXYLASE GENE - METHODS FOR THEIR CHARACTERIZATION
    GULDBERG, P
    GUTTLER, F
    [J]. ACTA PAEDIATRICA, 1994, 83 : 27 - 33