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Trace element excess in PKU diets?

E Sievers1, H D Oldigs, K Dörner

  • 1Department of Paediatrics, University of Kiel, FRG.

Journal of Inherited Metabolic Disease
|January 1, 1990
PubMed
Summary
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Infants with phenylketonuria (PKU) fed specialized diets show higher copper and manganese retention. Current trace element supplementation in PKU formulas may need adjustment for early infancy.

Area of Science:

  • Pediatric Nutrition
  • Metabolic Disorders
  • Trace Element Metabolism

Background:

  • Limited data exists on trace element requirements for infants with phenylketonuria (PKU) on semisynthetic diets.
  • Human milk trace element concentrations serve as a reference, but PKU formulas differ significantly.

Purpose of the Study:

  • To assess the balance and retention of iron, copper, and manganese in early-diagnosed infants with PKU.
  • To evaluate the suitability of current trace element supplementation in PKU formulas for the first four months of life.

Main Methods:

  • Longitudinal balance studies (72h) were conducted on three infants with PKU at 2, 5, 8, 12, and 16 weeks of age.
  • Dietary and fecal concentrations of iron, copper, and manganese were measured using atomic absorption spectroscopy.

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Main Results:

  • Median trace element concentrations in PKU diets (Fe: 4.8 mg/L, Cu: 1.7 mg/L, Mn: 0.43 mg/L) were higher than in human milk, primarily due to amino acid preparation supplementation.
  • Significantly higher daily retention of copper (0.17 mg/kg) and manganese (6.4 µg/kg) was observed in infants fed the PKU diet.
  • Median iron retention was 0.24 mg/kg.

Conclusions:

  • The increased intake of trace elements from PKU formulas leads to higher retention rates in infants.
  • Results raise concerns regarding the adequacy and appropriateness of current trace element supplementation levels in PKU formulas for infants under four months.
  • Further research is needed to establish optimal trace element profiles for PKU management in early infancy.