Protein Requirements in Adults With Phenylketonuria (PKU)

Last updated: July 24, 2024
Sponsor: University of British Columbia
Overall Status: Completed

Phase

N/A

Condition

Phenylketonuria

Treatment

Free amino acids intake

Clinical Study ID

NCT03939052
H18-03464
  • Ages 19-50
  • All Genders

Study Summary

Phenylketonuria (PKU) is an inherited inborn error of phenylalanine (PHE) metabolism caused by decreased activity of phenylalanine hydroxylase (PAH) enzyme. Therefore, PHE accumulates in plasma leading to mental problems. Treatment is a phenylalanine-restricted diet with sufficient protein. However, the optimum protein requirements are still unknown and compliance with diet is not satisfactory in PKU adults. A Previously established technique called indicator amino acid oxidation (IAAO) will be used to determine protein requirements from amino acid based formula vs. glycomacropeptide (GMP) in adults with PKU (≥ 19y). This study will help treat adults with enough protein ensuring maintenance of health.

Eligibility Criteria

Inclusion

Inclusion Criteria:

-Adults more than 19 years of age who are diagnosed with PKU and clinically stable with no acute illness

Exclusion

Exclusion Criteria:

  • Adults with PKU under age 19 year

  • Adults diagnosed with PKU but are currently ill with a fever or cold

Study Design

Total Participants: 6
Treatment Group(s): 1
Primary Treatment: Free amino acids intake
Phase:
Study Start date:
July 19, 2019
Estimated Completion Date:
January 13, 2023

Connect with a study center

  • BC Children's Hospital Research Institute, University of British Columbia

    Vancouver, British Columbia V5Z4H4
    Canada

    Site Not Available

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