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N/A N=16 Randomized Treatment

Evaluation of Phe Fluctuation in PKU Pts Treated With PKU GOLIKE Versus Standard Amino Acid Protein Substitute.

Phenylketonurias

Enrolled (actual)
16
Serious AEs
0.0%
Results posted
Jun 2025
Primary outcome: Primary: Blood Phe After 7 Days of Each Treatment — 357.5; 294.0; 346.8; 442.4 µmol/L

Study Design & Population

Study type
Interventional
Phase
N/A
Interventions
PKU GOLIKE (Dietary_supplement)
Age
Pediatric · 5+ yrs
Sex
All
Sponsor
APR Applied Pharma Research s.a.
Primary completion
May 2024

Outcome Measures

OutcomeResultp-value
PRIMARY
Blood Phe After 7 Days of Each Treatment
357.5; 294.0; 346.8; 442.4
SECONDARY
Dosage of Tyr in Blood (Umol/L) With Dried Blood Spots Before Breakfast
46.4; 62.1; 49.5; 51.6

Summary

This is a 2 arm, randomised, controlled, cross-over study in 16 children with PKU. Subjects who are currently taking a Phe free/low Phe protein substitute will be recruited for a 31-day trial. Patients will be randomised to receive: 1. The study product for 7 days as their last dose of protein substitute for the day (at least one sachet with 15g PE) in an amount equivalent to their usual protein substitute PE; or 2. An amino acid protein substitute for all daily doses for 7 days; followed by a 2-week washout period on their usual protein substitute, and then 7 days of the other study arm. During this time, patients/caregivers will be asked to: * Collect 3 finger prick blood spots on days -1, 0, 6, 7, 20, 21, 27 and 28. * Collect urine sample, second void of the day on days 0, 7, 21 and 28. * Complete a questionnaire on sleep quality on day 0, 7, 21 and 28. * Complete a 24 hour food diary on days -1, 0, 6, 7, 20, 21, 27 and 28. APR will supply the study product for participants free of charge.

Eligibility Criteria

Inclusion Criteria

  • Male and female PKU patients ≥5 years and ≤16 years of age.
  • Patients diagnosed with PKU via new born screening.
  • Taking a Phe free/low Phe protein substitute
  • On a low phenylalanine diet .
  • Absence of neurological deficiencies.
  • Adherence with dietary management and protein substitute.
  • Able to understand and comply with the requirements of the investigation and sign the Informed Consent Form/Assent form.

Exclusion Criteria

  • Age 16 years old.
  • Patients with mild PKU or HPA.
  • On sapropterin therapy.
  • Patients with late diagnosis of PKU and neurological problems.
  • History of hypersensitivity to any excipients/components of the investigational product.
  • Pregnancy or breastfeeding during the study.
  • Any moderate to severe acute illness which in the opinion of the Investigator would interfere with the study procedures or study outcome.
  • History of poor co-operation, non-adherence with dietary management, or poor adherence to investigation procedures.
  • Participation in any other studies involving investigational or marketed products concomitantly or within two weeks prior to entry into the study.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT05487378). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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