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

Data de publicació:

Autors de IIS La Fe

Autors aliens a IIS La Fe

  • Aguiar, A
  • Ahring, K
  • Almeida, MF
  • Assoun, M
  • Quintana, AB
  • Bigot, S
  • Bihet, G
  • Malmberg, KB
  • Burlina, A
  • Bushueva, T
  • Caris, A
  • Chan, H
  • Clark, A
  • Clark, S
  • Cochrane, B
  • Corthouts, K
  • 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, AM
  • Lang, K
  • Lier, D
  • Lilje, R
  • Lowry, S
  • Luyten, K
  • MacDonald, A
  • Meyer, U
  • Moor, D
  • Pal, A
  • Robert, M
  • Robertson, L
  • Rocha, JC
  • Rohde, C
  • Ross, K
  • Saruhan, S
  • Sjoqvist, E
  • Skeath, R
  • Stoelen, L
  • Ter Horst, NM
  • Terry, A
  • Timmer, C
  • Tuncer, N
  • Vande Kerckhove, K
  • van der Ploeg, L
  • van Rijn, M
  • van Spronsen, FJ
  • van Teeffelen-Heithoff, A
  • van Wegberg, A
  • van Wyk, K
  • Vasconcelos, C
  • Wildgoose, J
  • Webster, D
  • White, FJ
  • Zweers, H

Grups d'Investigació

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 <1 year, >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 <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). 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.

Dades de la publicació

ISSN/ISSNe:
1096-7192, 1096-7206

MOLECULAR GENETICS AND METABOLISM  ACADEMIC PRESS INC ELSEVIER SCIENCE

Tipus:
Article
Pàgines:
17-22
PubMed:
25862610
Factor d'Impacte:
1,541 SCImago ℠
Quartil:
Q1 SCImago ℠

Cites Rebudes en Web of Science: 25

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Keywords

  • Phenylketonuria; Protein substitute; L-Amino acid supplements; Glycomacropeptide

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