1. Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach
- Author
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J. Wildgoose, K. Kaalund-Hansen, K. Eftring, C. Jankowski, D. Lier, S. Saruhan, S. Bigot, M. van Rijn, I. Jones, A. Clark, A. De Meyer, K. Luyten, Carmen Rohde, K. Lang, N. Tuncer, Sharon Evans, E. Sjoqvist, K. Vande Kerckhove, F.J. White, M. Heddrich-Ellerbrok, K. Blom Malmberg, R.G. Janssen-Regelink, H. Zweers, Hulya Gokmen-Ozel, A. Terry, Karen Corthouts, A.M. Lammardo, Júlio César Rocha, Clara Vasconcelos, Maria Gizewska, C. Ellerton, Martine Dassy, H. Chan, S. Lowry, K. van Wyk, N.M. Ter Horst, S. Clark, K. Dokupil, A. Faria, K. Ahring, C. Timmer, A. Belanger Quintana, Isidro Vitoria, F. Gunden, M. Diels, Sylvain Dubois, A. van Teeffelen-Heithoff, M. Joerg-Streller, S. Heiber, T. Bushueva, L. Stoelen, Alessandro P. Burlina, M. Assoun, Bozena Didycz, Abhijit Ricky Pal, D. Webster, C. Jonkers, L. van der Ploeg, G. Bihet, D. Moor, R. Skeath, J. Ekengren, U. Meyer, A. Aguiar, Jaime Dalmau, Anita MacDonald, Kathleen Ross, A. Fischer, M. Robert, F. J. van Spronsen, A.M.J. van Wegberg, Joanna Gribben, Carina Heidenborg, Suzanne Ford, Barbara Cochrane, M.F. Almeida, R. Lilje, L. Robertson, Peter Freisinger, A. Caris, E. Kiss, Extramural researchers, Endocrinology, and Center for Liver, Digestive and Metabolic Diseases (CLDM)
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Male ,Pediatrics ,age distribution ,Southern Europe ,Turkey ,phenylalanine ,Phenylketonurias ,Cross-sectional study ,patient monitoring ,Endocrinology, Diabetes and Metabolism ,lnfectious Diseases and Global Health Radboud Institute for Molecular Life Sciences [Radboudumc 4] ,INFANTS ,METABOLIC-CONTROL ,casein ,preschool child ,Biochemistry ,Endocrinology ,newborn ,Surveys and Questionnaires ,infant disease ,Phenylketonuria ,ADULT PATIENTS ,Amino Acids ,Child ,Protein substitute ,administration and dosage ,GLYCOMACROPEPTIDE ,education.field_of_study ,L-Amino acid supplements ,childhood disease ,adult ,Northern European ,Caseins ,Protein intake ,clinical practice ,Europe ,AMINO-ACID MIXTURE ,Chemistry ,female ,priority journal ,Child, Preschool ,OBESITY ,Western europe ,GROWTH ,world health organization, Adult ,Dietary Proteins ,DIETARY-MANAGEMENT ,amino acid ,medicine.medical_specialty ,Diet therapy ,Population ,Western Europe ,Eastern Europe ,World Health Organization ,Article ,caseinomacropeptide ,Genetics ,medicine ,cross-sectional study ,Humans ,human ,Preschool ,education ,Molecular Biology ,Biology ,prescription ,business.industry ,questionnaire ,Infant, Newborn ,Dietary management ,Infant ,school child ,medicine.disease ,protein intake ,major clinical study ,Obesity ,Peptide Fragments ,NITROGEN ,protein intake, age distribution ,diet therapy ,peptide fragment ,consensus ,dietary supplement ,Dietary Supplements ,Human medicine ,business ,Glycomacropeptide - 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 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. (C) 2015 Elsevier Inc. All rights reserved.
- Published
- 2015
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