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Protein requirements in infants and children: a longitudinal study of children treated for phenylketonuria

Insights

This study compared two protein intake recommendations for children with phenylketonuria (PKU). While both groups remained healthy, some children on the Food and Agricultural Organization (FAO) diet showed reduced growth and potential bone issues.

Area of Science:

  • Biochemistry
  • Pediatrics
  • Nutritional Science

Background:

  • Phenylketonuria (PKU) requires careful protein management from birth.
  • Existing protein intake guidelines vary, necessitating comparative studies.

Purpose of the Study:

  • To assess the adequacy of two different protein intake recommendations for children with PKU.
  • To monitor biochemical control and physical development in PKU patients.

Main Methods:

  • Longitudinal follow-up of two groups of children with PKU from birth.
  • Dietary protein intake adherence to Recommended Dietary Allowance (RDA) and Food and Agricultural Organization (FAO) guidelines.
  • Monitoring of biochemical markers, growth parameters (length, weight), hematology, and bone health (hand x-ray).

Main Results:

  • Both groups of children with PKU were biochemically well-controlled and generally healthy.
  • A subset of children in the FAO group exhibited a decline in length growth percentile.
  • Two children in the FAO group showed signs suggestive of developing osteoporosis.

Conclusions:

  • The FAO's recommended safe protein intake level for egg or milk protein may be marginal for some children with PKU.
  • Current protein intake guidelines may require reassessment for optimal growth and bone health in PKU management.

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