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Clinical Nutrition

Protein substitutes for phenylketonuria in Europe: access and nutritional composition

European Journal of Clinical Nutrition volume 70, pages 785–789 (2016)Cite this article

Subjects

Abstract

Background/Objectives:

Protein substitutes (PS) are an essential component in the dietary management of phenylketonuria (PKU). PS are available as phenylalanine-free amino-acid mixtures (AAM), glycomacropeptide-based PS (GMP) and large neutral amino acids (LNAA). There is a lack of information regarding their availability in different countries and comparison of their nutritional composition is limited. The objectives of this study were to identify the number of PS available in different European countries and Turkey and to compare their nutritional composition.

Subjects/Methods:

Members of the European Nutritionist Expert Panel on PKU (ENEP) (Portugal, Spain, Belgium, Italy, Germany, Netherlands, United Kingdom, Denmark and Turkey) provided data on PS available in each country. The nutritional composition of PS available in Portugal was analyzed.

Results:

The number of PS available in each country varied from 30 (Turkey) to 105 (Germany), with a median of 64. GMP was available only in Portugal, whereas LNAA was an option in Portugal, Italy, Turkey and Denmark. Some PS were designed for weaning. Many PS did not contain added fat and fiber. GMP contained the highest carbohydrate (CHO) and energy content as well as higher LNAA content compared with AAM. Only one AAM contained added fructo-oligosaccharides and galacto-oligosaccharides. AAM designed for the first year of life had the highest CHO, fat and LNAA contribution. Liquid AAM had lower CHO and fat contents compared with powdered AAM, but contained higher LNAA.

Conclusions:

There was widely dissimilar numbers of PS available in different countries. Nutritional composition of different PS was variable and should be considered before prescription.

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Author information

Authors and Affiliations

  1. Centro de Genética Médica, Centro Hospitalar do Porto (CHP), Porto, Portugal

    M J Pena, M F de Almeida & J C Rocha

  2. Unit for Multidisciplinary Research in Biomedicine (UMIB), Instituto de Ciências Biomédicas Abel Salazar (ICBAS), University of Porto, Porto, Portugal

    M F de Almeida

  3. University of Groningen, University Medical Center Groningen, Beatrix Children’s Hospital, Groningen, The Netherlands

    E van Dam

  4. Department of PKU, Kennedy Centre, Copenhagen University Hospital, Glostrup, Denmark

    K Ahring

  5. Unidad Enfermedades Metabolicas Servicio de Pediatria Hospital Ramon y Cajal, Madrid, Spain

    A Bélanger-Quintana

  6. Department of Metabolism and Nutrition, Dr von Hauner Children's Hospital, University of Munich, Munich, Germany

    K Dokoupil

  7. Department of Nutrition and Dietetics, Hacettepe University, Ankara, Turkey

    H Gokmen-Ozel

  8. Department of Pediatrics, San Paolo Hospital University of Milan, Milan, Italy

    A M Lammardo

  9. The Children’s Hospital, Birmingham, UK

    A MacDonald

  10. Nutrition and Metabolism Unit, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium

    M Robert

  11. Faculdade de Ciências da Saúde, Universidade Fernando Pessoa, Porto, Portugal

    J C Rocha

  12. Center for Health Technology and Services Research (CINTESIS), Porto, Portugal

    J C Rocha

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  1. M J Pena
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  2. M F de Almeida
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  3. E van Dam
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  4. K Ahring
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  6. K Dokoupil
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  7. H Gokmen-Ozel
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  8. A M Lammardo
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  9. A MacDonald
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  10. M Robert
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Corresponding author

Correspondence to J C Rocha.

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Competing interests

All authors have received compensation from Merck Serono as members of the European Nutritionist Expert Panel in PKU. AM has received research funding and honoraria from Nutricia, Vitaflo International, Arla, Sobi and Merck Serono. She is a member of the European Nutrition Expert Panel (Merck Serono international), a member of Sapropterin Advisory Board (Merck Serono international) and a member of the Advisory Board ELEMENT (Danone-Nutricia). MJP and MFdA declare no conflict of interest.

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Pena, M., de Almeida, M., van Dam, E. et al. Protein substitutes for phenylketonuria in Europe: access and nutritional composition. Eur J Clin Nutr 70, 785–789 (2016). https://doi.org/10.1038/ejcn.2016.54

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  • DOI: https://doi.org/10.1038/ejcn.2016.54

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