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Clinical nutrition, enteral and parenteral nutrition

PKU patients on a relaxed diet may be at risk for micronutrient deficiencies

European Journal of Clinical Nutrition volume 68, pages 119–124 (2014)Cite this article

Subjects

Abstract

Objective:

To investigate micronutrient supply in phenylketonuria (PKU) patients on a relaxed diet.

Subjects/Methods:

Sixty-seven patients (6–45 years) with a phenylalanine tolerance ⩾600 mg/day were included in the study. From a 3-day diet record, protein supply as well as consumption of essential amino acids and several micronutrients were assessed and compared with the current recommendations and data for the healthy population.

Results:

Protein supply and consumption of all essential amino acids were sufficient in all patients. Supply of micronutrients depended on dietary regime. Patients with a total protein supply of 120% or more of the recommended amount and at least 0.5 g protein per kg body weight from amino-acid mixture (AAM) were sufficiently supplied with all investigated micronutrients. All patients without AAM supplement showed severe micronutrient deficiencies in their diet records.

Conclusion:

PKU patients under a relaxed diet are at risk of an insufficient nutrient supply, if they have first no substitution with AAM, second a protein supply less than 0.5 g per kg body weight from AAM or third a total protein supply less than 120% of the recommendations. Therefore, close monitoring, specific dietary counseling and potential supplementation is mandatory to prevent micronutrient deficiencies in PKU patients.

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Acknowledgements

We thank the patients and their parents for their participation and efforts to meet the protocol requirements. The unrestricted financial support by Nutricia GmbH, Erlangen, is greatly appreciated. Evelyn Penske and Andrea Grieser were a big help in creating the data base.

Author Contributions

The trial was designed and submitted to authorities by SB and CR with substantial consultation by CK and WK; patients were recruited and examined by CR, AGT, MA, UM, CB, ATH, SS, CH and SB, dietary records were analyzed by CR; data management was maintained by CR, JG and SB; statistical analyses were performed by CR and SB, with valuable advice from JG; results were discussed and conclusions drawn by all contributing authors, especially CR, SB, AGT, AS, M, CR and SB drafted the manuscript and incorporated comments by all co-authors.

This study was supported by unrestricted research grant from Nutricia GmbH, Heilbronn, Germany

Author information

Authors and Affiliations

  1. Department of Women and Child Health, Hospital for Children and Adolescents, Centre of Paediatric Research (CPL), University Hospitals, University of Leipzig, Leipzig, Germany

    C Rohde, A G Thiele, M Arelin, U Mütze, W Kiess & S Beblo

  2. Department of Pediatrics, University Hospital, University of Münster, Münster, Germany

    A von Teeffelen-Heithoff

  3. Nutricia GmbH, Heilbronn, Germany

    C Kiener & J Gerloff

  4. Department of Internal Medicine, University Hospitals, University of Leipzig, Leipzig, Germany

    C Baerwald

  5. Center for Obstetrics and Paediatrics, University Medical Center Hamburg-Eppendorf, Hamburg-Eppendorf, Germany

    S Schultz

  6. Department of Paediatrics and Adolescent Medicine, University Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany

    C Heller

  7. Institute of Agricultural and Nutritional Sciences, Martin-Luther-University, Halle-Wittenberg, Germany

    A S Müller

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  1. C Rohde
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Corresponding author

Correspondence to S Beblo.

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Rohde, C., von Teeffelen-Heithoff, A., Thiele, A. et al. PKU patients on a relaxed diet may be at risk for micronutrient deficiencies. Eur J Clin Nutr 68, 119–124 (2014). https://doi.org/10.1038/ejcn.2013.218

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  • DOI: https://doi.org/10.1038/ejcn.2013.218

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