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Trace element excess in PKU diets?

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Journal of Inherited Metabolic Disease

Summary

Knowledge of trace element requirements of infants with phenylketonuria (PKU) fed a semisynthetic diet is limited. Three infants with PKU detected early were studied longitudinally in classical balance studies for 72 h, under domestic conditions, at the ages of 2, 5, 8, 12 and 16 weeks. Iron, copper and manganese concentrations in the diet and faeces were determined by atomic absorption spectroscopy. The median concentrations in the diet (4.8 mg Fe/L, 1.7 mg Cu/L, 0.43 mg Mn/L) exceed those in human milk. This is mainly due to supplementation of the amino acid preparation used. The increased intake led to a significantly higher daily retention of Cu and Mn from the PKU-diet fed, with a median of 0.17 mg Cu/kg and 6.4 µg Mn/kg body weight; the median retention of Fe was 0.24 mg Fe/kg. Our results confirmed the doubts about the suitability of the present trace element supplementation in formula for infants with PKU during the first four months of life.

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Authors and Affiliations

  1. Department of Paediatrics, University of Kiel, Schwanenweg 20, D-2300, Kiel 1, Germany

    E. Sievers, H. -D. Oldigs, K. Dörner & J. Schaub

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  1. E. Sievers
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  2. H. -D. Oldigs
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  3. K. Dörner
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  4. J. Schaub
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Additional information

A preliminary report of part of this work has been presented at the annual meeting of the European Society for Pediatric Research in Oslo, 1988. (Sievers, E., Oldigs, H.-D. Dörner, K. and Schaub, J. Trace element overload in PKU diet?Pediatr. Res. 24 (1988) 270, Abstract)

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Sievers, E., Oldigs, H.D., Dörner, K. et al. Trace element excess in PKU diets?. J Inherit Metab Dis 13, 897–905 (1990). https://doi.org/10.1007/BF01800217

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