Protein Requirements in Adults With Phenylketonuria (PKU)

August 20, 2019 updated by: Rajavel Elango, PhD, University of British Columbia

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

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.

Study Overview

Status

Unknown

Conditions

Intervention / Treatment

Study Type

Interventional

Enrollment (Anticipated)

6

Phase

  • Not Applicable

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Locations

  • Canada
    • British Columbia
      • Vancouver, British Columbia, Canada, V5Z4H4
        • Recruiting
        • BC Children's Hospital Research Institute, University of British Columbia
        • Contact:
          • Rajavel Elango, Ph.D

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

19 years to 50 years (Adult)

Accepts Healthy Volunteers

No

Genders Eligible for Study

All

Description

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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Primary Purpose: Supportive Care
  • Allocation: N/A
  • Interventional Model: Single Group Assignment
  • Masking: None (Open Label)

Arms and Interventions

Participant Group / Arm
Intervention / Treatment
Experimental: Protein intake
Free amino acids vs. Glycomacropeptide (GMP)
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).
Other Names:
  • glycomacropeptide (GMP) intake

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
13 Co2 production
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.
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.
Lysine flux
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.
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 Outcome Measures

Outcome Measure
Measure Description
Time Frame
Phenylalanine concentrations
Time Frame: One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.
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.
Tyrosine concentrations
Time Frame: One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.
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.
16 other amino acids
Time Frame: One sample (at 6th meal) after starting of the tracer protocol. Data will be reported an average of 2 years.
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.

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

University of British Columbia

Collaborators

Vitaflo International, Ltd

Investigators

  • Principal Investigator: Rajavel Elango, PhD, BC Children's Hospital Research Institute, University of British Columbia
  • Study Chair: Sandra Sirrs, MD, University of British Columbia
  • Study Chair: Sylvia Stockler, MD, University of British Columbia

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

July 19, 2019

Primary Completion (Anticipated)

May 1, 2020

Study Completion (Anticipated)

May 1, 2020

Study Registration Dates

First Submitted

May 1, 2019

First Submitted That Met QC Criteria

May 2, 2019

First Posted (Actual)

May 6, 2019

Study Record Updates

Last Update Posted (Actual)

August 22, 2019

Last Update Submitted That Met QC Criteria

August 20, 2019

Last Verified

August 1, 2019

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • H18-03464

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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