Breath and urine organic compounds in GMP and Phe-free L-AA
Research type
Research Study
Full title
A PILOT RANDOMISED, CROSSOVER STUDY IN PKU INVESTIGATING THE EXHALED BREATH AND URINE PROFILE OF ORGANIC COMPOUNDS, COMPARING GLYCOMACROPEPTIDE WITH PHENYLALANINE-FREE L-AMINO ACID SUPPLEMENTS
IRAS ID
211894
Contact name
Anne Daly
Contact email
Sponsor organisation
Research and Development Department Birmingham Children's Hospital
Duration of Study in the UK
0 years, 1 months, 1 days
Research summary
PKU is treated with a low protein diet. This is very restrictive and equivalent to to only 1 slice/day of normal bread. To prevent nutritional deficiency, a special amino acid supplement (Phe-free L-AA) is taken 3 times daily. The Phe-free L-AA contains 100% free amino acids. These are unpleasant to take and rich in cysteine- an amino acid that smells like bad eggs. Caregivers complain their children have bad breath, which may be linked to their protein substitute. An alternative protein substitute called cGMP is available. It contains less free cysteine and may cause less bad breath. We aim to study by questionnaire and chemical analysis (by collecting breath and urine samples) the effect of a Phe-free L-AA vs. cGMP on bad breath. We also will collect information in control children.
REC name
West Midlands - Coventry & Warwickshire Research Ethics Committee
REC reference
17/WM/0032
Date of REC Opinion
10 Apr 2017
REC opinion
Further Information Favourable Opinion