Protein Requirements in Adults With Phenylketonuria (PKU)

Phenylketonuria Clinical Trial

Official title:

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

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT03939052
• Other study ID #: H18-03464
• Status: Recruiting
• Phase: N/A
• Start date: July 19, 2019
• Est. completion date: May 2020

Study information

• Verified date: August 2019
• Source: University of British Columbia
• Contact: Rajavel Elango, PhD
• Phone: 604-875-2000
• Email: relango[@]bcchr.ubc.ca
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 6
• Est. completion date: May 2020
• Est. primary completion date: May 2020
• Accepts healthy volunteers: No
• Gender: All
• Age group: 19 Years to 50 Years

Eligibility

Inclusion Criteria:

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

Exclusion Criteria:

• Adults with PKU under age 19 year

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

Related Conditions & MeSH terms

• Phenylketonuria
• Phenylketonurias

Intervention

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).

Locations

Country	Name	City	State
Canada	BC Children's Hospital Research Institute, University of British Columbia	Vancouver	British Columbia

Sponsors (2)

Lead Sponsor	Collaborator
University of British Columbia	Vitaflo International, Ltd

Country where clinical trial is conducted

Canada, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	13 Co2 production	Breath samples will be collected during the study to measure the rate of oxidation of tracer in the expired breath.	8 hours (1 study day). 3 samples will collected as a baseline prior to isotope protocol and 6 samples after 2 hours and 30 minutes of starting the tracer protocol. Data will be reported an average of 2 years.
Primary	Lysine flux	Urine samples will be collected during the study to measure the flux enrichment in urine.	8 hours (1 study day). 1 sample will collected as a baseline prior to isotope protocol and 2 samples after 2 hours and 30 minutes of starting the tracer protocol. Data will be reported an average of 2 years.
Secondary	Phenylalanine concentrations	Phenylalanine concentrations will be measured in blood.	One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.
Secondary	Tyrosine concentrations	Tyrosine concentrations will be measured in blood.	One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.
Secondary	16 other amino acids	16 other amino acids will be measured in blood.	One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.