Protein Requirements in Adults With Phenylketonuria (PKU)

Not Applicable

Completed

• Conditions: Phenylketonuria
• Interventions: Dietary Supplement: Free amino acids intake

• Registration Number: NCT03939052
• Lead Sponsor: University of British Columbia

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.

Detailed Description

Not available

Basic Information
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Recruitment & Eligibility
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Study & Design
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Trial Locations

Study Timeline

• Study First Submitted: 2019-05-01
• Study First Submitted QC: 2019-05-02
• Study First Posted: 2019-05-06
• Study Start: 2019-07-19
• Primary Completion: 2021-12-01
• Study Completion: 2023-01-13
• Status Verified: 2024-07
• Last Update Submitted: 2024-07-24
• Last Update Posted: 2024-07-25

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 6

Inclusion Criteria

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

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Exclusion Criteria

• Adults with PKU under age 19 year
• Adults diagnosed with PKU but are currently ill with a fever or cold

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Study & Design

• Study Type: INTERVENTIONAL
• Study Design: SINGLE_GROUP

Arm && Interventions

Group	Intervention	Description
Protein intake	Free amino acids intake	Free amino acids vs. Glycomacropeptide (GMP)

Primary Outcome Measures

Name	Time	Method
13 Co2 production	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.
Lysine flux	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.

Secondary Outcome Measures

Name	Time	Method
16 other amino acids	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.
Phenylalanine concentrations	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.
Tyrosine concentrations	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.

Trial Locations

Locations (1)

BC Children's Hospital Research Institute, University of British Columbia; 🇨🇦 Vancouver, British Columbia, Canada