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.; Blom Malmberg, K.; Burlina, A.; Bushueva, T.; Caris, A.; Chan, H.; Clark, Rachael A; Clark, Sue; Cochrane, B.; Corthouts, K.; Dalmau, Judith; Dassy, M.; 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.G.; Jones, C.I.; Jonkers, C.F.; Joerg-Streller, M.; Kaalund-Hansen, K.; Kiss, E.; Lammardo, A.M.; Lang, K.; Lier, D.; Lilje, R.; Lowry, S.; Luyten, K.; MacDonald, A.; Meyer, U.; de Moor, J.; Pal, A.; Robert, C.M.; Robertson, L.; Rocha, J.C.; Rohde, C.; Ross, K.; Saruhan, S.; Sjöqvist, E.; Skeath, R.; Stoelen, L.; ter Horst, N.M.; Terry, A.; Timmer, C.; Tuncer, N.; Vande Kerckhove, K.; van den Ploeg, L.; van Rijn, M.; van Spronsen, Francjan J.; van Teeffelen-Heithoff, A.; van Wegberg, A.; van Wyk, K.; Vasconcelos, C.; Vitoria, I.; Wildgoose, J.; Webster, D.; White, F.J.; Zweers, H.
(2015) Molecular Genetics and Metabolism, volume 115, issue 1, pp. 17 - 22
(Article)
Abstract
Background: There appears little consensus concerning protein requirements in phenylketonuria (PKU). Methods: A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein
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substitute. Data were analysed by European region using descriptive statistics. Results: The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n. =. 24 centres) (infants . 2-3. g/kg/day; 1-3. years of age, >. 2-3. g/kg/day; 4-10. years of age, >. 1.5-2.5. g/kg/day) and Southern Europe (n. =. 10 centres) (infants . 2-2.5. g/kg/day; 4-10. years of age, >. 1.5-2. g/kg/day) and with Western Europe (n. =. 25 centres) giving the least (infants . 2-2.5. g/kg/day, 1-3. years of age, 1.5-2. g/kg/day; 4-10. years of age, 1-1.5. g/kg/day). Total protein prescription was similar in patients aged >. 10. years (1-1.5. g/kg/day) and maternal patients (1-1.5. g/kg/day). Conclusions: The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population. © 2015 Elsevier Inc..
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Keywords: amino acid, casein, caseinomacropeptide, peptide fragment, phenylalanine, protein intake, age distribution, Article, child, childhood disease, clinical practice, consensus, cross-sectional study, Eastern Europe, Europe, human, infant disease, major clinical study, Northern European, patient monitoring, phenylketonuria, preschool child, prescription, priority journal, protein intake, questionnaire, school child, Southern Europe, Western Europe, administration and dosage, adult, diet therapy, dietary supplement, female, infant, male, newborn, Turkey, world health organization, Adult, Amino Acids, Caseins, Child, Child, Preschool, Dietary Proteins, Dietary Supplements, Female, Humans, Infant, Infant, Newborn, Male, Peptide Fragments, Phenylalanine, Phenylketonurias, Surveys and Questionnaires, World Health Organization
ISSN: 1096-7192
Publisher: Academic Press Inc.
Note: cited By 5
(Peer reviewed)