Nutrients (Jul 2020)
Uniformity of Food Protein Interpretation Amongst Dietitians for Patients with Phenylketonuria (PKU): 2020 UK National Consensus Statements
- Sharon Evans,
- Sarah Adam,
- Sandra Adams,
- Heather Allen,
- Catherine Ashmore,
- Sarah Bailey,
- Janette Banks,
- Harriet Churchill,
- Barbara Cochrane,
- Jennifer Cook,
- Clare Dale,
- Anne Daly,
- Marjorie Dixon,
- Carolyn Dunlop,
- Charlotte Ellerton,
- Anita Emm,
- Sarah Firman,
- Suzanne Ford,
- Moira French,
- Joanna Gribben,
- Anne Grimsley,
- Ide Herlihy,
- Melanie Hill,
- Shirley Judd,
- Karen Lang,
- Jo Males,
- Joy McDonald,
- Nicola McStravick,
- Chloe Millington,
- Camille Newby,
- Catharine Noble,
- Rachel Pereira,
- Alex Pinto,
- Louise Robertson,
- Abigail Robotham,
- Kathleen Ross,
- Kath Singleton,
- Rachel Skeath,
- Allyson Terry,
- Karen Van Wyk,
- Fiona White,
- Lucy White,
- Jo Wildgoose,
- Alison Woodall,
- Anita MacDonald
Affiliations
- Sharon Evans
- Birmingham Women’s & Children’s NHS Foundation Trust, Birmingham B4 6NH, UK
- Sarah Adam
- Royal Hospital for Children, Glasgow G51 4TF, UK
- Sandra Adams
- Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK
- Heather Allen
- Sheffield Children’s NHS Foundation Trust, Sheffield S10 2TH, UK
- Catherine Ashmore
- Birmingham Women’s & Children’s NHS Foundation Trust, Birmingham B4 6NH, UK
- Sarah Bailey
- Cardiff and Vale UHB, Cardiff CF14 4XW, UK
- Janette Banks
- Wrightington, Wigan and Leigh NHS Foundation Trust, Wigan WN1 2NN, UK
- Harriet Churchill
- University College London Hospitals NHS Foundation Trust, London WC1N 3BG, UK
- Barbara Cochrane
- Royal Hospital for Children, Glasgow G51 4TF, UK
- Jennifer Cook
- Guy’s and St Thomas’ NHS Foundation Trust, London SE1 7EU, UK
- Clare Dale
- University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, UK
- Anne Daly
- Birmingham Women’s & Children’s NHS Foundation Trust, Birmingham B4 6NH, UK
- Marjorie Dixon
- Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK
- Carolyn Dunlop
- Royal Hospital for Sick Children, Edinburgh EH9 1LF, UK
- Charlotte Ellerton
- University College London Hospitals NHS Foundation Trust, London WC1N 3BG, UK
- Anita Emm
- University Hospital Southampton NHS Foundation Trust, Southampton SO16 6YD, UK
- Sarah Firman
- Guy’s and St Thomas’ NHS Foundation Trust, London SE1 7EU, UK
- Suzanne Ford
- The National Society for Phenylketonuria, Purley CR8 9DD, UK
- Moira French
- University Hospitals of Leicester NHS Trust, Leicester LE1 5WW, UK
- Joanna Gribben
- Evelina London Children’s Hospital, London SE1 7EH, UK
- Anne Grimsley
- Belfast Health & Social Care Trust, Belfast BT9 7AB, UK
- Ide Herlihy
- Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK
- Melanie Hill
- Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S5 7AU, UK
- Shirley Judd
- Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool L7 8XP, UK
- Karen Lang
- Ninewells Hospital, Dundee DD1 9SY, UK
- Jo Males
- Aneurin Bevan University Health Board Wales, Newport NP20 2UB, UK
- Joy McDonald
- Belfast Health & Social Care Trust, Belfast BT9 7AB, UK
- Nicola McStravick
- Belfast Health & Social Care Trust, Belfast BT9 7AB, UK
- Chloe Millington
- Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK
- Camille Newby
- Bristol Royal Hospital for Children, Bristol BS2 8BJ, UK
- Catharine Noble
- Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S5 7AU, UK
- Rachel Pereira
- Norfolk and Norwich University Hospital, Norwich NR4 7UY, UK
- Alex Pinto
- Birmingham Women’s & Children’s NHS Foundation Trust, Birmingham B4 6NH, UK
- Louise Robertson
- University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, UK
- Abigail Robotham
- Bristol Royal Hospital for Children, Bristol BS2 8BJ, UK
- Kathleen Ross
- Royal Aberdeen Children’s Hospital, Aberdeen AB25 2ZG, UK
- Kath Singleton
- University Hospital of Wales, Cardiff CF14 4XW, UK
- Rachel Skeath
- Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK
- Allyson Terry
- Alder Hey Children’s NHS Foundation Trust, Liverpool L12 2AP, UK
- Karen Van Wyk
- Royal Manchester Children’s Hospital, Manchester M13 9WL, UK
- Fiona White
- Royal Manchester Children’s Hospital, Manchester M13 9WL, UK
- Lucy White
- Sheffield Children’s NHS Foundation Trust, Sheffield S10 2TH, UK
- Jo Wildgoose
- Bradford Teaching Hospitals NHS Foundation Trust, Bradford BD5 0NA, UK
- Alison Woodall
- Salford Royal NHS Foundation Trust, Salford M6 8HD, UK
- Anita MacDonald
- Birmingham Women’s & Children’s NHS Foundation Trust, Birmingham B4 6NH, UK
- DOI
- https://doi.org/10.3390/nu12082205
- Journal volume & issue
-
Vol. 12,
no. 8
p. 2205
Abstract
In phenylketonuria (PKU), variable dietary advice provided by health professionals and social media leads to uncertainty for patients/caregivers reliant on accurate, evidence based dietary information. Over four years, 112 consensus statements concerning the allocation of foods in a low phenylalanine diet for PKU were developed by the British Inherited Metabolic Disease Dietitians Group (BIMDG-DG) from 34 PKU treatment centres, utilising 10 rounds of Delphi consultation to gain a majority (≥75%) decision. A mean of 29 UK dietitians (range: 18–40) and 18 treatment centres (range: 13–23) contributed in each round. Statements encompassed all foods/food groups divided into four categories based on defined protein/phenylalanine content: (1) foods high in protein/phenylalanine (best avoided); (2) foods allowed without restriction including fruit/vegetables containing phenylalanine ≤75 mg/100 g and most foods containing protein ≤0.5 g/100 g; (3) foods that should be calculated/weighed as an exchange food if they contain protein exchange ingredients (categorized into foods with a protein content of: >0.1 g/100 g (milk/plant milks only), >0.5 g/100 g (bread/pasta/cereal/flours), >1 g/100 g (cook-in/table-top sauces/dressings), >1.5 g/100 g (soya sauces)); and (4) fruit/vegetables containing phenylalanine >75 mg/100 g allocated as part of the protein/phenylalanine exchange system. These statements have been endorsed and translated into practical dietary management advice by the medical advisory dietitians for the National Society for PKU (NSPKU).
Keywords