GMP Powdered Protein Substitutes in PKU and TYR Case Studies
Research type
Research Study
Full title
Evaluating the adherence, tolerance, acceptability and safety of new GMP-based powdered protein substitutes in PKU and TYR: a case study series
IRAS ID
349251
Contact name
Gary Hubbard
Contact email
Sponsor organisation
Nutricia
Duration of Study in the UK
1 years, 11 months, 26 days
Research summary
Phenylketonuria (PKU) and tyrosinaemia (TYR) are rare inherited disorders of amino acid metabolism which require lifelong management to avoid severe cognitive impairment and disability. The dietary management of PKU and TYR is complex and burdensome for patients and their families. It involves the restriction of dietary protein intake, supplementation with phenylalanine-free and phenylalanine and tyrosine-free protein substitutes, respectively, to ensure optimal metabolic control, whilst meeting nutrient requirements for growth and normal functioning.
Four new GMP-based protein substitutes have been developed to help with compliance in the dietary management of PKU and TYR: 1) PKU GMPro Delight 20, 2) PKU GMPro Delight 15, 3) TYR GMPro Delight 20, 4) TYR GMPro Delight 15, are powdered protein substitutes, low in phenylalanine and low in phenylalanine and tyrosine respectively, with a mix of glycomacropeptide (GMP), essential and non-essential amino acids, carbohydrates, fibres, fats (including DHA) and micronutrients. The proteins in these new protein substitutes are based on GMP, a peptide isolated from milk during cheese manufacturing and the only known naturally derived protein source low in phenylalanine and tyrosine. Studies to date have illustrated that PKU patients who replace their regular phenylalanine-free amino acid-based formula with GMP-based foods may have better diet compliance and prefer the taste whilst maintaining metabolic control.
This series of case-studies aims to evaluate the gastrointestinal tolerance, acceptability, compliance, and safety of these four GMPro Delight protein substitutes in both adults and children over 3 years. These case studies will last 29 days in total, including a 1-day baseline period followed by a 28-day intervention period. The case studies will be conducted across multiple specialist metabolic centres in the UK
REC name
East of England - Cambridge South Research Ethics Committee
REC reference
24/EE/0262
Date of REC Opinion
23 Jan 2025
REC opinion
Further Information Favourable Opinion