Rare Diseases Symptoms Automatic Extraction

Unrestricted fruits and vegetables in the PKU diet: a 1-year follow-up.

[phenylketonuria]

Phenylketonuria (PKU) therapy demands phenylalanine (Phe) calculation. In most countries, almost all food is taken into account, even fruits and vegetables. We investigated whether unrestricted consumption of fruits and vegetables negatively influences metabolic control. Nineteen PKU children (2-10 years) started with 2 weeks of free or restricted fruit and vegetable intake. After 2 weeks, the regime changed from free to restricted or restricted to free (cross-over design). Over the first 4 weeks, dried blood Phe concentration was measured, fruit and vegetable consumption recorded and nutrient intake calculated from diet records. Thereafter the diet was changed to free use of fruits and vegetables for all patients. Six and 12 months later, diet and Phe concentrations were monitored. Median Phe intake increased significantly by 65 mg/day (week 4, P<0.001), 68 mg/day (month 6, P<0.001) and 70 mg/day (month 12, P<0.001). Dried blood Phe concentrations remained stable (P=0.894), as did the frequency of Phe concentrations above the recommended range (P=0.592). In conclusion, PKU diet liberalization for fruits and vegetables seems unproblematic.

Diseases presenting "vegetable intake" symptom

  • phenylketonuria

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