Evaluation of PKU Explore (PKU Explore)
Phenylketonuria, Inborn Errors of Metabolism
About this trial
This is an interventional treatment trial for Phenylketonuria focused on measuring Phenylketonuria, PKU, Metabolism, Metabolic, Control, Acceptability
Eligibility Criteria
Inclusion Criteria:
i. A diagnosis of PKU on new-born screening requiring a low protein diet and Phe-free L-amino acid supplements.
ii. Aged between 6 months and 5 years.
iii. Already taking a second stage concentrated protein substitute as part of their PKU management OR is at the stage in their PKU management when a second stage concentrated protein substitute is recommended to commence.
iv. Willingly given, written, informed consent from parent/guardian.
v. Willingly given, written assent (if appropriate).
Exclusion Criteria:
i. Diagnosis of persistent hyperphenylalaninaemia, or mild PKU not requiring dietary treatment with a low protein diet and Phe-free L-amino acid supplements.
ii. Diagnosis of a concurrent condition which may adversely affect developmental progression and feeding ability.
iii. Participation in any other clinical trial/acceptability study.
iv. Any serious medical precluding the study intervention.
v. Use of additional macro/micronutrient supplements during the study period, unless clinically indicated and prescribed by the investigator (must be recorded in patient case record file).
Sites / Locations
- NHS Greater Glasgow and Clyde
- Birmingham Women's and Children's NHS Foundation Trust
- Bradford Teaching Hospitals NHS Foundation Trust
Arms of the Study
Arm 1
Experimental
Consumption of PKU Explore
Daily feed, substituting the participant's normal phe-free protein substitute for PKU Explore.