title: Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach.
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, >2-3g/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).
  
  
  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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mesh_terms: D000328:Adult; D000596:Amino Acids / Q000008:administration & dosage*; D002364:Caseins / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D004044:Dietary Proteins / Q000008:administration & dosage*; D019587:Dietary Supplements; D005060:Europe; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010446:Peptide Fragments / Q000008:administration & dosage*; D010649:Phenylalanine; D010661:Phenylketonurias / Q000178:diet therapy*; D011795:Surveys and Questionnaires; D014421:Turkey; D014944:World Health Organization
chemical_list: D000596:Amino Acids; D002364:Caseins; D004044:Dietary Proteins; D010446:Peptide Fragments; C065989:caseinomacropeptide; D010649:Phenylalanine
keywords: Glycomacropeptide; Phenylketonuria; Protein substitute; l-Amino acid supplements