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Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach
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2015 (English)In: Molecular Genetics and Metabolism, ISSN 1096-7192, E-ISSN 1096-7206, Vol. 115, no 1, 17-22 p.Article in journal (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
2015. Vol. 115, no 1, 17-22 p.
Keyword [en]
Phenylketonuria, Protein substitute, L-Amino acid supplements, Glycomacropeptide
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Other Health Sciences
Identifiers
URN: urn:nbn:se:uu:diva-256539DOI: 10.1016/j.ymgme.2015.03.006ISI: 000355031800003PubMedID: 25862610OAI: oai:DiVA.org:uu-256539DiVA: diva2:826414
Available from: 2015-06-25 Created: 2015-06-24 Last updated: 2017-12-04Bibliographically approved

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Malmberg, Karin Blom

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