Suitability and Allocation of Protein-Containing Foods According to Protein Tolerance in PKU: A 2022 UK National Consensus

Maria Inês Gama, Sarah Adam, Sandra Adams, Heather Allen, Catherine Ashmore, Sarah Bailey, Barbara Cochrane, Clare Dale, Anne Daly, Giana De Sousa, Sarah Donald, Carolyn Dunlop, Charlotte Ellerton, Sharon Evans, Sarah Firman, Suzanne Ford, Francine Freedman, Moira French, Lisa Gaff, Joanna GribbenAnne Grimsley, Ide Herlihy, Melanie Hill, Farzana Khan, Nicola McStravick, Chloe Millington, Nicola Moran, Camille Newby, Patty Nguyen, Janet Purves, Alex Pinto, Júlio César Rocha, Rachel Skeath, Amy Skelton, Simon Tapley, Alison Woodall, Carla Young, Anita MacDonald

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Introduction: There is little practical guidance about suitable food choices for higher natural protein tolerances in patients with phenylketonuria (PKU). This is particularly important to consider with the introduction of adjunct pharmaceutical treatments that may improve protein tolerance. Aim: To develop a set of guidelines for the introduction of higher protein foods into the diets of patients with PKU who tolerate >10 g/day of protein. Methods: In January 2022, a 26-item food group questionnaire, listing a range of foods containing protein from 5 to >20 g/100 g, was sent to all British Inherited Metabolic Disease Group (BIMDG) dietitians (n = 80; 26 Inherited Metabolic Disease [IMD] centres). They were asked to consider within their IMD dietetic team when they would recommend introducing each of the 26 protein-containing food groups into a patient’s diet who tolerated >10 g to 60 g/day of protein. The patient protein tolerance for each food group that received the majority vote from IMD dietetic teams was chosen as its tolerance threshold for introduction. A virtual meeting was held using Delphi methodology in March 2022 to discuss and agree final consensus. Results: Responses were received from dietitians from 22/26 IMD centres (85%) (11 paediatric, 11 adult). For patients tolerating protein ≥15 g/day, the following foods were agreed for inclusion: gluten-free pastas, gluten-free flours, regular bread, cheese spreads, soft cheese, and lentils in brine; for protein tolerance ≥20 g/day: nuts, hard cheeses, regular flours, meat/fish, and plant-based alternative products (containing 5–10 g/100 g protein), regular pasta, seeds, eggs, dried legumes, and yeast extract spreads were added; for protein tolerance ≥30 g/day: meat/fish and plant-based alternative products (containing >10–20 g/100 g protein) were added; and for protein tolerance ≥40 g/day: meat/fish and plant-based alternatives (containing >20 g/100 g protein) were added. Conclusion: This UK consensus by IMD dietitians from 22 UK centres describes for the first time the suitability and allocation of higher protein foods according to individual patient protein tolerance. It provides valuable guidance for health professionals to enable them to standardize practice and give rational advice to patients.

Original languageEnglish
Article number4987
Issue number23
Publication statusPublished - Dec 2022


  • national consensus
  • phenylalanine
  • Phenylketonuria
  • protein
  • sapropterin


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