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Review
. 2020 Jun 30;15(1):171.
doi: 10.1186/s13023-020-01391-y.

PKU dietary handbook to accompany PKU guidelines

A MacDonald  1 , A M J van Wegberg  2 , K Ahring  3 , S Beblo  4 , A Bélanger-Quintana  5 , A Burlina  6 , J Campistol  7 , T Coşkun  8 , F Feillet  9 , M Giżewska  10 , S C Huijbregts  11 , V Leuzzi  12 , F Maillot  13 , A C Muntau  14 , J C Rocha  15 , C Romani  16 , F Trefz  17 , F J van Spronsen  18
Affiliations
Review

PKU dietary handbook to accompany PKU guidelines

A MacDonald et al. Orphanet J Rare Dis. .

Erratum in

  • Correction to: PKU dietary handbook to accompany PKU guidelines.
    MacDonald A, van Wegberg AMJ, Ahring K, Beblo S, Bélanger-Quintana A, Burlina A, Campistol J, Coşkun T, Feillet F, Giżewska M, Huijbregts SC, Leuzzi V, Maillot F, Muntau AC, Rocha JC, Romani C, Trefz F, van Spronsen FJ. MacDonald A, et al. Orphanet J Rare Dis. 2020 Sep 1;15(1):230. doi: 10.1186/s13023-020-01486-6. Orphanet J Rare Dis. 2020. PMID: 32873338 Free PMC article.

Abstract

Background: Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism caused by deficiency in the enzyme phenylalanine hydroxylase that converts phenylalanine into tyrosine.

Main body: In 2017 the first European PKU Guidelines were published. These guidelines contained evidence based and/or expert opinion recommendations regarding diagnosis, treatment and care for patients with PKU of all ages. This manuscript is a supplement containing the practical application of the dietary treatment.

Conclusion: This handbook can support dietitians, nutritionists and physicians in starting, adjusting and maintaining dietary treatment.

Keywords: Diet; Guidelines; PKU; Phenylketonuria; Recommendations; Treatment.

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Conflict of interest statement

KA has been a member of the European Nutrition Expert Panel (Merck Serono international, Biomarin).

ABQ has been a board member and consultant for BioMarin and Merck Serono, received grants from BioMarin and received honoraria as a consultant and/or speaker and travel support from BioMarin, Merck, Nutricia, Vitaflo, and Mead-Johnson.

SB has received research grants from Nutricia Metabolics, Nutricia GmbH Germany and Merck Serono GmbH.

AB has been a member of Scientific Advisory Boards of Merck-Serono SA, Biomarin, Nutricia and has received honoraria from Merck-Serono SA, Biomarin, Nutricia/Danone.

JC has received honoraria as a consultant and/or speaker from Merck-Serono SA and Nutricia/Danone.

TC has been a consultant for Biomarin and has received honoraria as a consultant and/or speaker and travel support from BioMarin.

MG has been a member of the Scientific Advisory Board of Merck-Serono SA, and Biomarin and received honoraria as a consultant and/or speaker for Merck Serono SA and Nutricia.

FF has been a board member for BioMarin and received honoraria as a consultant and/or speaker and travel support from BioMarin, Genzyme, Merck-Serono, Nutricia and Vitaflo.

CEMH’s institution has received financial support for registries for Gaucher disease and Fabry disease and for participation in premarketing studies from Genzyme and Protalix.

SCJH has participated in strategic advisory boards and received honoraria as a consultant and/or speaker for Merck Serono SA, Biomarin and Nutricia.

AM has been a member of the European Nutritionist Expert Panel Biomarin, Sapropterin Advisory Board Biomarin, the advisory board ELEMENT Danone-Nutricia, the advisory board for Arla and Applied Pharma Research, and received research funding and honoraria from Nutricia, Vitaflo International and Merck Serono.

FM has received honoraria as a consultant and/or speaker from Merck Serono SA, Nutricia and Arla Foods.

ACM has been a board member and consultant for BioMarin, Merck, Arla Food, Applied Pharma Research, and Nestlé, received grants from Nutricia and Vitaflo, and received honoraria as a consultant and/or speaker from BioMarin, Merck, Nestlé, and Actelion.

JCR has been a member of the European Nutritionist Expert Panel [Biomarin], the Advisory Board for Applied Pharma Research s.a. and Nutricia, and received honoraria as a speaker from APR, Merck Serono, Biomarin, Nutricia, Vitaflo and Cambrooke.

FJvS has been a member of scientific advisory boards for defects in amino acid metabolism of APR, Arla Food International, BioMarin, Eurocept Int, Lucana, Moderna TX, Nutricia, Rivium and SoBi, has received research grants from Alexion, Biomarin, Codexis, Nutricia, SoBi, and Vitaflo, has received grants from patient organizations ESPKU, Metakids, NPKUA, Stofwisselkracht, Stichting PKU research and Tyrosinemia Foundation, and has received honoraria as consultant and speaker from APR, Biomarin, MendeliKABS and Nutricia.

FT received grants from Vitaflo Germany and honoraria as a speaker from Merck-Serono SA.

AMJvW has received a research grant from Nutricia, honoraria from Biomarin as speaker, and travel support from Nutricia and Vitaflo.

The other authors declare no conflict of interest.

Figures

Fig. 1
Fig. 1
Food pyramid for PKU
Fig. 2
Fig. 2
Protein exchange calculator (NSPKU website 2019, download)
See this image and copyright information in PMC

References

    1. van Wegberg AMJ, MacDonald A, Ahring K, Belanger-Quintana A, Blau N, Bosch AM, et al. The complete European guidelines on phenylketonuria: diagnosis and treatment. Orphanet J Rare Dis. 2017;12(1):162. doi: 10.1186/s13023-017-0685-2. - DOI - PMC - PubMed
    1. Araujo A, Araujo WMC, Marquez UML, Akutsu R, Nakano EY. Table of phenylalanine content of foods: comparative analysis of data compiled in food composition tables. JIMD Rep. 2017;34:87–96. doi: 10.1007/8904_2016_12. - DOI - PMC - PubMed
    1. Kim J, Boutin M. A list of phenylalanine to protein ratios for common foods. 2014.
    1. Weetch E, Macdonald A. The determination of phenylalanine content of foods suitable for phenylketonuria. J Hum Nutr Diet. 2006;19(3):229–236. doi: 10.1111/j.1365-277X.2006.00696.x. - DOI - PubMed
    1. MacDonald A, White F. Amino acid disorders. In: Shaw V, editor. Clinical paediatrics dietetics. Chichester: Wiley Blackwell; 2015. pp. 391–456.

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