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Practices in prescribing protein substitutes for pku in europe: No uniformity of approach

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Günden, F.

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Aguiar, A.
Ahring, K.
Almeida, M. F.
Assoun, M.
Belanger Quintana, A.
Bigot, S.
Bihet, G.
Malmberg, K. Blom
Burlina, A.
Bushueva, T.

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Elsevier

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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.

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Amino-acid mixture, Adult patients, Dietary-management, Metabolic-control, Phenylketonuria, Growth, Glycomacropeptide, Nitrogen, Infants, Obesity, Phenylketonuria, Protein substitute, L-amino acid supplements, Glycomacropeptide, Science & technology, Life sciences & biomedicine, Endocrinology & metabolism, Genetics & heredity, Medicine, research & experimental, Research & experimental medicine

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