NCT03939052

Protein Requirements in Adults With Phenylketonuria (PKU)

Protein Requirements in Phenylketonuria (PKU) Patients Compared Using PKU Sphere™ | Glycomacropeptide (GMP) and an L-amino Acid-based Product

• Status: Completed
• Phase: N/A
• Study type: Interventional
• Enrollment: 6 (actual)
• Sponsor: University of British Columbia · Academic / Other
• Sex: All
• Age: 19 Years – 50 Years
• Healthy volunteers: Not accepted

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.

Conditions

• Phenylketonuria

Interventions

Type	Name	Description
DIETARY_SUPPLEMENT	Free amino acids intake	Oral consumption of eight hourly experimental meals- -4 tracer free experimental meals containing a mixture of free amino acids and calories from protein free flavoured liquid, protein free cookies and corn oil -4 isotopically labeled experimental meals. The same protocol will be repeated with glycomacropeptide (GMP).

Timeline

• Start date: 2019-07-19
• Primary completion: 2021-12-01
• Completion: 2023-01-13
• First posted: 2019-05-06
• Last updated: 2024-07-25

Locations

1 site across 1 country: Canada