PKU dietary handbook to accompany PKU guidelines

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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.

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Most cited references 37

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The complete European guidelines on phenylketonuria: diagnosis and treatment

A. van Wegberg, A. MacDonald, K. Ahring … (2017)

Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism caused by deficiency in the enzyme phenylalanine hydroxylase that converts phenylalanine into tyrosine. If left untreated, PKU results in increased phenylalanine concentrations in blood and brain, which cause severe intellectual disability, epilepsy and behavioural problems. PKU management differs widely across Europe and therefore these guidelines have been developed aiming to optimize and standardize PKU care. Professionals from 10 different European countries developed the guidelines according to the AGREE (Appraisal of Guidelines for Research and Evaluation) method. Literature search, critical appraisal and evidence grading were conducted according to the SIGN (Scottish Intercollegiate Guidelines Network) method. The Delphi-method was used when there was no or little evidence available. External consultants reviewed the guidelines. Using these methods 70 statements were formulated based on the highest quality evidence available. The level of evidence of most recommendations is C or D. Although study designs and patient numbers are sub-optimal, many statements are convincing, important and relevant. In addition, knowledge gaps are identified which require further research in order to direct better care for the future.

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Nutrition Recommendations in Pregnancy and Lactation

Priya Rajan, Michelle Kominiarek (2016)

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Parental influences on children's eating behaviour and characteristics of successful parent-focussed interventions.

Gemma Mitchell, Claire V Farrow, Emma Haycraft … (2013)

Parental reports suggest that difficulties related to child-feeding and children's eating behaviour are extremely common. While 'fussy eating' does not pose an immediate threat to health, over the long-term, consumption of a poor diet can contribute to the development of a range of otherwise preventable diseases. In addition, the stress and anxiety that can surround difficult mealtimes can have a detrimental impact upon both child and parental psychological wellbeing. Since parents have a great influence over what, when, and how much food is offered, feeding difficulties may be preventable by better parental awareness. The aim of this review is to describe how parental factors contribute to the development of common feeding problems, and to discuss the merits of existing interventions aimed at parents/primary caregivers to improve child-feeding and children's eating behaviour. The potential for different technologies to be harnessed in order to deliver interventions in new ways will also be discussed. Copyright © 2012 Elsevier Ltd. All rights reserved.

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Author and article information

Contributors

F. J. van Spronsen: f.j.van.spronsen@umcg.nl

Journal

Journal ID (nlm-ta): Orphanet J Rare Dis

Journal ID (iso-abbrev): Orphanet J Rare Dis

Title: Orphanet Journal of Rare Diseases

Publisher: BioMed Central (London )

ISSN (Electronic): 1750-1172

Publication date (Electronic): 30 June 2020

Publication date PMC-release: 30 June 2020

Publication date Collection: 2020

Volume: 15

Electronic Location Identifier: 171

Affiliations

[1 ]GRID grid.415246.0, ISNI 0000 0004 0399 7272, Dietetic Department, , Birmingham Children’s Hospital, ; Birmingham, UK

[2 ]Division of Metabolic Diseases, Beatrix Children’s Hospital, University Medical Centre Groningen, University of Groningen, Hanzeplein 1, 9700 RB Groningen, The Netherlands

[3 ]Department of PKU, Kennedy Centre, Glostrup, Denmark

[4 ]Department of Women and Child Health, Center for Pediatric Research Leipzig, Hospital for Children and Adolescents, University Hospitals, Leipzig, Germany

[5 ]GRID grid.411347.4, ISNI 0000 0000 9248 5770, Metabolic Diseases Unit, Department of Paediatrics, , Hospital Ramon y Cajal Madrid, ; Madrid, Spain

[6 ]GRID grid.411474.3, ISNI 0000 0004 1760 2630, Division of Inherited Metabolic Diseases, Department of Paediatrics, , University Hospital of Padova, ; Padova, Italy

[7 ]Neuropaediatrics Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain

[8 ]GRID grid.14442.37, ISNI 0000 0001 2342 7339, Hacettepe University Faculty of Medicine, ; Ankara, Turkey

[9 ]Department of Paediatrics, Hôpital d’Enfants Brabois, CHU Nancy, Vandoeuvre les Nancy, France

[10 ]GRID grid.107950.a, ISNI 0000 0001 1411 4349, Department of Paediatrics, Endocrinology, Diabetology, Metabolic Diseases and Cardiology of the Developmental Age, , Pomeranian Medical University, ; Szczecin, Poland

[11 ]GRID grid.5132.5, ISNI 0000 0001 2312 1970, Department of Clinical Child and Adolescent Studies-Neurodevelopmental Disorders, Faculty of Social Sciences, , Leiden University, ; Leiden, The Netherlands

[12 ]GRID grid.7841.a, Department of Paediatrics, Child Neurology and Psychiatry, , Sapienza University of Rome, ; Via dei Sabelli 108, 00185 Rome, Italy

[13 ]CHRU de Tours, Université François Rabelais, INSERM U1069, Tours, France

[14 ]GRID grid.13648.38, ISNI 0000 0001 2180 3484, University Children’s Hospital, University Medical Centre Hamburg-Eppendorf, ; 20246 Hamburg, Germany

[15 ]GRID grid.10772.33, ISNI 0000000121511713, Nutrition & Metabolism, NOVA Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal. Centre for Health Technology and Services Research (CINTESIS), ; Porto, Portugal

[16 ]GRID grid.7273.1, ISNI 0000 0004 0376 4727, School of Life and Health Sciences, , Aston University, ; Birmingham, UK

[17 ]GRID grid.7700.0, ISNI 0000 0001 2190 4373, Department of Paediatrics, , University of Heidelberg, ; Heidelberg, Germany

Author information

A. M. J. van Wegberg http://orcid.org/0000-0002-9402-4833

Article

Publisher ID: 1391

DOI: 10.1186/s13023-020-01391-y

PMC ID: 7329487

PubMed ID: 32605583

SO-VID: 2ecaba62-4d6c-4aa6-b45d-0d8a6121bfa4

License:

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

History

Date received : 9 September 2019

Date accepted : 4 May 2020

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ScienceOpen disciplines: Infectious disease & Microbiology

Keywords: phenylketonuria ,pku ,diet ,treatment ,recommendations ,guidelines

Data availability:

ScienceOpen disciplines: Infectious disease & Microbiology

Keywords: phenylketonuria , pku , diet , treatment , recommendations , guidelines

PKU dietary handbook to accompany PKU guidelines

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Abstract

Background

Main body

Conclusion

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Future Science Group: Coronavirus

Most cited references 37

The complete European guidelines on phenylketonuria: diagnosis and treatment

Nutrition Recommendations in Pregnancy and Lactation

Parental influences on children's eating behaviour and characteristics of successful parent-focussed interventions.

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Contributors

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