Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach

A. Aguiar, K. Ahring, M. F. Almeida, M. Assoun, A. Belanger Quintana, S. Bigot, G. Bihet, K. Blom Malmberg, A. Burlina, T. Bushueva, A. Caris, H. Chan, A. Clark, S. Clark, B. Cochrane, K. Corthouts, J. Dalmau, M. Dassy, A. de Meyer, B. DidyczM. Diels, K. Dokupil, S. Dubois, K. Eftring, J. Ekengren, C. Ellerton, S. Evans, A. Faria, A. Fischer, S. Ford, P. Freisinger, M. Giżewska, H. Gokmen-Ozel, J. Gribben, F. Gunden, M. Heddrich-Ellerbrok, S. Heiber, C. Heidenborg, C. Jankowski, R. Janssen-Regelink, I. Jones, C. Jonkers, M. Joerg-Streller, K. Kaalund-Hansen, E. Kiss, A. M. Lammardo, K. Lang, D. Lier, R. Lilje, S. Lowry, K. Luyten, A. Macdonald, U. Meyer, D. Moor, A. Pal, M. Robert, L. Robertson, J. C. Rocha, C. Rohde, K. Ross, S. Saruhan, E. Sjöqvist, R. Skeath, L. Stoelen, N. M. ter Horst, A. Terry, C. Timmer, N. Tuncer, K. Vande Kerckhove, L. van der Ploeg, M. van Rijn, F. J. van Spronsen, A. van Teeffelen-Heithoff, A. van Wegberg, K. van Wyk, C. Vasconcelos, I. Vitoria, J. Wildgoose, D. Webster, F. J. White, H. Zweers

Research output: Contribution to JournalArticleAcademicpeer-review

Abstract

There appears little consensus concerning protein requirements in phenylketonuria (PKU). 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. 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 <1 year, >2-3g/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 <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >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 <1 year, >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). 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
Original languageEnglish
Pages (from-to)17-22
JournalMolecular genetics and metabolism
Volume115
Issue number1
DOIs
Publication statusPublished - 2015

Cite this