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The intake of total protein, natural protein and protein substitute and growth of height and head circumference in Dutch infants with phenylketonuria

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Journal of Inherited Metabolic Disease

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Summary

In a previous study, Dutch children with phenylketonuria (PKU) were found to be slightly shorter than their healthy counterparts. In the literature, it has been hypothesized that a higher protein intake is necessary to optimize growth in PKU patients. The study aimed to investigate whether protein intake (total, natural and protein substitute) in this group might be an explanatory factor for the observed growth. Growth of height and head circumference and dietary data on protein intake (total, natural and protein substitute) from 174 Dutch PKU patients born between 1974 and 1996 were analysed retrospectively for the patients' first 3 years of life. Analyses were corrected for energy intake during the first year of life and for the clinical severity of the deficiency of phenylalanine hydroxylase by means of plasma phenylalanine concentration at birth. Neither protein nor energy intake correlated with height growth. A positive, statistically significant relation between head circumference growth and natural protein and total protein intake was found, but not with the intake of the protein substitute or energy. Therefore, this study suggests that improvement of the protein substitute rather than an increase of total protein intake may be important in optimizing head circumference growth in PKU patients.

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Authors and Affiliations

  1. Section of Metabolic Diseases, Beatrix Children's Hospital, University Medical Centre of Groningen, University of Groningen, Groningen, The Netherlands

    M. Hoeksma, M. Van Rijn, P. J. J. Sauer & F. J. van Spronsen

  2. Department of Dietetics, University Medical Centre of Groningen, University of Groningen, Groningen, The Netherlands

    M. Van Rijn

  3. TNO Prevention and Health, Leiden, The Netherlands

    P. H. Verkerk

  4. Department of Paediatrics, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands

    A. M. Bosch

  5. Department of Paediatrics, University Hospital VU, Amsterdam, The Netherlands

    M. F. Mulder

  6. Department of Paediatrics, Division of Metabolic Diseases and Genetics, Erasmus Medical Centre/Sophia Children's Hospital, Rotterdam, The Netherlands

    J. B. C. de Klerk

  7. Department of Metabolic Diseases, University Medical Centre Utrecht, Utrecht, The Netherlands

    T. J. De Koning

  8. Department of Paediatrics, University Hospital Maastricht, Maastricht, The Netherlands

    E. Rubio-Gozalbo

  9. Department of Metabolic Diseases, University Medical Centre Nijmegen, Nijmegen, The Netherlands

    M. De Vries

  10. Beatrix Children's Hospital, University Medical Centre of Groningen, PO Box 30.001, 9700 RB, Groningen, The Netherlands

    F. J. van Spronsen

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  1. M. Hoeksma
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  2. M. Van Rijn
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  3. P. H. Verkerk
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  4. A. M. Bosch
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  5. M. F. Mulder
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  6. J. B. C. de Klerk
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  7. T. J. De Koning
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  8. E. Rubio-Gozalbo
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  9. M. De Vries
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  10. P. J. J. Sauer
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  11. F. J. van Spronsen
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Corresponding author

Correspondence to F. J. van Spronsen.

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Hoeksma, M., Van Rijn, M., Verkerk, P.H. et al. The intake of total protein, natural protein and protein substitute and growth of height and head circumference in Dutch infants with phenylketonuria. J Inherit Metab Dis 28, 845–854 (2005). https://doi.org/10.1007/s10545-005-0122-x

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  • DOI: https://doi.org/10.1007/s10545-005-0122-x

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