Back to resultsProtein Requirements in Adults With Phenylketonuria (PKU)
Primary Purpose
Phenylketonuria
Status
Unknown status
Phase
Not Applicable
Locations
Canada
Study Type
Interventional
Intervention
Free amino acids intake
Sponsored by
About this trial
This is an interventional supportive care trial for Phenylketonuria focused on measuring Phenylketonuria, PKU, Phenylalanine metabolism, Protein requirements, Indicator Amino acid Oxidation, Stable isotopes, GMP, Glycomacropeptide
Eligibility Criteria
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
Sites / Locations
- BC Children's Hospital Research Institute, University of British ColumbiaRecruiting
Arms of the Study
Arm 1
Arm Type
Experimental
Arm Label
Protein intake
Arm Description
Free amino acids vs. Glycomacropeptide (GMP)
Outcomes
Primary Outcome Measures
13 Co2 production
Breath samples will be collected during the study to measure the rate of oxidation of tracer in the expired breath.
Lysine flux
Urine samples will be collected during the study to measure the flux enrichment in urine.
Secondary Outcome Measures
Phenylalanine concentrations
Phenylalanine concentrations will be measured in blood.
Tyrosine concentrations
Tyrosine concentrations will be measured in blood.
16 other amino acids
16 other amino acids will be measured in blood.
Full Information
NCT ID
NCT03939052
First Posted
May 1, 2019
Last Updated
August 20, 2019
Sponsor
University of British Columbia
Collaborators
Vitaflo International, Ltd
1. Study Identification
Unique Protocol Identification Number
NCT03939052
Brief Title
Protein Requirements in Adults With Phenylketonuria (PKU)
Official Title
Protein Requirements in Phenylketonuria (PKU) Patients Compared Using PKU Sphere™ | Glycomacropeptide (GMP) and an L-amino Acid-based Product
Study Type
Interventional
2. Study Status
Record Verification Date
August 2019
Overall Recruitment Status
Unknown status
Study Start Date
July 19, 2019 (Actual)
Primary Completion Date
May 2020 (Anticipated)
Study Completion Date
May 2020 (Anticipated)
3. Sponsor/Collaborators
Responsible Party, by Official Title
Principal Investigator
Name of the Sponsor
University of British Columbia
Collaborators
Vitaflo International, Ltd
4. Oversight
Studies a U.S. FDA-regulated Drug Product
No
Studies a U.S. FDA-regulated Device Product
No
5. Study Description
Brief 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.
6. Conditions and Keywords
Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Phenylketonuria
Keywords
Phenylketonuria, PKU, Phenylalanine metabolism, Protein requirements, Indicator Amino acid Oxidation, Stable isotopes, GMP, Glycomacropeptide
7. Study Design
Primary Purpose
Supportive Care
Study Phase
Not Applicable
Interventional Study Model
Single Group Assignment
Masking
None (Open Label)
Allocation
N/A
Enrollment
6 (Anticipated)
8. Arms, Groups, and Interventions
Arm Title
Protein intake
Arm Type
Experimental
Arm Description
Free amino acids vs. Glycomacropeptide (GMP)
Intervention Type
Dietary Supplement
Intervention Name(s)
Free amino acids intake
Other Intervention Name(s)
glycomacropeptide (GMP) intake
Intervention Description
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).
Primary Outcome Measure Information:
Title
13 Co2 production
Description
Breath samples will be collected during the study to measure the rate of oxidation of tracer in the expired breath.
Time Frame
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.
Title
Lysine flux
Description
Urine samples will be collected during the study to measure the flux enrichment in urine.
Time Frame
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 Outcome Measure Information:
Title
Phenylalanine concentrations
Description
Phenylalanine concentrations will be measured in blood.
Time Frame
One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.
Title
Tyrosine concentrations
Description
Tyrosine concentrations will be measured in blood.
Time Frame
One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.
Title
16 other amino acids
Description
16 other amino acids will be measured in blood.
Time Frame
One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.
10. Eligibility
Sex
All
Minimum Age & Unit of Time
19 Years
Maximum Age & Unit of Time
50 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
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
Central Contact Person:
First Name & Middle Initial & Last Name or Official Title & Degree
Rajavel Elango, PhD
Phone
604-875-2000
Ext
4911
Email
relango@bcchr.ubc.ca
Overall Study Officials:
First Name & Middle Initial & Last Name & Degree
Rajavel Elango, PhD
Organizational Affiliation
BC Children's Hospital Research Institute, University of British Columbia
Official's Role
Principal Investigator
First Name & Middle Initial & Last Name & Degree
Sandra Sirrs, MD
Organizational Affiliation
University of British Columbia
Official's Role
Study Chair
First Name & Middle Initial & Last Name & Degree
Sylvia Stockler, MD
Organizational Affiliation
University of British Columbia
Official's Role
Study Chair
Facility Information:
Facility Name
BC Children's Hospital Research Institute, University of British Columbia
City
Vancouver
State/Province
British Columbia
ZIP/Postal Code
V5Z4H4
Country
Canada
Individual Site Status
Recruiting
Facility Contact:
First Name & Middle Initial & Last Name & Degree
Rajavel Elango, Ph.D
12. IPD Sharing Statement
Learn more about this trial
Protein Requirements in Adults With Phenylketonuria (PKU)
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