PKU dietary handbook to accompany PKU guidelines

A. MacDonald; A. M. J. van Wegberg; K. Ahring; S. Beblo; A. Belanger-Quintana; A. Burlina; J. Campistol; T. Coskun; F. Feillet; M. Gizewska; S. C. Huijbregts; V. Leuzzi; F. Maillot; A. C. Muntau; J. C. Rocha; C. Romani; F. Trefz; F. J. van Spronsen

doi:10.1186/s13023-020-01391-y

PKU dietary handbook to accompany PKU guidelines

A. MacDonald, A. M. J. van Wegberg, K. Ahring, S. Beblo, A. Belanger-Quintana, A. Burlina, J. Campistol, T. Coskun, F. Feillet, M. Gizewska, S. C. Huijbregts, V. Leuzzi, F. Maillot, A. C. Muntau, J. C. Rocha, C. Romani, F. Trefz, F. J. van Spronsen^*

^*Corresponding author for this work

Research output: Contribution to journal › Review article › peer-review

137 Citations (Scopus)

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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.

Original language	English
Article number	171
Number of pages	21
Journal	Orphanet journal of rare diseases
Volume	15
Issue number	1
DOIs	https://doi.org/10.1186/s13023-020-01391-y
Publication status	Published - 4-Aug-2020

Keywords

Phenylketonuria
PKU
Diet
Treatment
Recommendations
Guidelines
PROTEIN SUBSTITUTE
FEEDING PRACTICES
EATING BEHAVIOR
PHENYLKETONURIA
NEOPHOBIA
EXERCISE
CHILDREN

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PKU dietary handbook to accompany PKU guidelinesFinal publisher's version, 1.08 MBLicence: CC BY

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MacDonald, A., van Wegberg, A. M. J., Ahring, K., Beblo, S., Belanger-Quintana, A., Burlina, A., Campistol, J., Coskun, T., Feillet, F., Gizewska, M., Huijbregts, S. C., Leuzzi, V., Maillot, F., Muntau, A. C., Rocha, J. C., Romani, C., Trefz, F., & van Spronsen, F. J. (2020). PKU dietary handbook to accompany PKU guidelines. Orphanet journal of rare diseases, 15(1), Article 171. https://doi.org/10.1186/s13023-020-01391-y

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title = "PKU dietary handbook to accompany PKU guidelines",

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 = "Phenylketonuria, PKU, Diet, Treatment, Recommendations, Guidelines, PROTEIN SUBSTITUTE, FEEDING PRACTICES, EATING BEHAVIOR, PHENYLKETONURIA, NEOPHOBIA, EXERCISE, CHILDREN",

author = "A. MacDonald and {van Wegberg}, {A. M. J.} and K. Ahring and S. Beblo and A. Belanger-Quintana and A. Burlina and J. Campistol and T. Coskun and F. Feillet and M. Gizewska and Huijbregts, {S. C.} and V. Leuzzi and F. Maillot and Muntau, {A. C.} and Rocha, {J. C.} and C. Romani and F. Trefz and {van Spronsen}, {F. J.}",

year = "2020",

month = aug,

day = "4",

doi = "10.1186/s13023-020-01391-y",

language = "English",

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MacDonald, A, van Wegberg, AMJ, Ahring, K, Beblo, S, Belanger-Quintana, A, Burlina, A, Campistol, J, Coskun, T, Feillet, F, Gizewska, M, Huijbregts, SC, Leuzzi, V, Maillot, F, Muntau, AC, Rocha, JC, Romani, C, Trefz, F & van Spronsen, FJ 2020, 'PKU dietary handbook to accompany PKU guidelines', Orphanet journal of rare diseases, vol. 15, no. 1, 171. https://doi.org/10.1186/s13023-020-01391-y

TY - JOUR

T1 - PKU dietary handbook to accompany PKU guidelines

AU - MacDonald, A.

AU - van Wegberg, A. M. J.

AU - Ahring, K.

AU - Beblo, S.

AU - Belanger-Quintana, A.

AU - Burlina, A.

AU - Campistol, J.

AU - Coskun, T.

AU - Feillet, F.

AU - Gizewska, M.

AU - Huijbregts, S. C.

AU - Leuzzi, V.

AU - Maillot, F.

AU - Muntau, A. C.

AU - Rocha, J. C.

AU - Romani, C.

AU - Trefz, F.

AU - van Spronsen, F. J.

PY - 2020/8/4

Y1 - 2020/8/4

N2 - 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.

AB - 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.

KW - Phenylketonuria

KW - PKU

KW - Diet

KW - Treatment

KW - Recommendations

KW - Guidelines

KW - PROTEIN SUBSTITUTE

KW - FEEDING PRACTICES

KW - EATING BEHAVIOR

KW - PHENYLKETONURIA

KW - NEOPHOBIA

KW - EXERCISE

KW - CHILDREN

U2 - 10.1186/s13023-020-01391-y

DO - 10.1186/s13023-020-01391-y

M3 - Review article

SN - 1750-1172

VL - 15

JO - Orphanet journal of rare diseases

JF - Orphanet journal of rare diseases

IS - 1

M1 - 171

ER -

PKU dietary handbook to accompany PKU guidelines

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