Protein Requirements in Children With Phenylketonuria (PKU)

Not Applicable

Completed

• Conditions: Phenylketonuria
• Interventions: Dietary Supplement: Protein intake

• Registration Number: NCT01965691
• Lead Sponsor: University of British Columbia

Brief Summary

Phenylketonuria (PKU) is an inherited inborn error of an amino acid phenylalanine (PHE) metabolism affecting 1:15,000 births. It is caused by a decreased activity of an enzyme in the liver called phenylalanine hydroxylase (PAH) which is important to convert PHE into tyrosine, another amino acid. Consequently, PHE accumulates in the blood leading to mental and developmental delays. Nutritional management is the primary choice of treatment that includes providing sufficient protein in the diet and at the same time restricting PHE. However the amount of protein to be given is unknown. A new technique called Indicator Amino Acid Oxidation (IAAO) will be used to determine the protein requirements in children with PKU (5-18y). The study will help treat and manage these children with sufficient protein to ensure proper growth and development. Current dietary recommendations range from 35-65 g/day and is based on factorial calculations.

The investigators hypothesize that the protein requirement in children with PKU will be higher than the current mathematically calculated recommended intake of 35-65 g/day for the 5-18y children.

Detailed Description

Not available

Study Timeline

• Study Start: 2013-10
• Study First Submitted: 2013-10-16
• Study First Submitted QC: 2013-10-17
• Study First Posted: 2013-10-18
• Primary Completion: 2015-08
• Study Completion: 2015-12
• Status Verified: 2020-09
• Last Update Submitted: 2020-09-22
• Last Update Posted: 2020-09-24

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 4

Inclusion Criteria

• Children (5-18y) who are diagnosed with Phenylketonuria (PKU), and clinically stable with no acute illness

Read More

Exclusion Criteria

• Children less than 5y of age and who are diagnosed with PKU, as it may be difficult to take breath samples and perform indirect calorimetry in very young children.
• Children diagnosed with PKU, but are currently ill, with a fever, cold, vomiting or diarrhea.

Read More

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: SINGLE_GROUP

Arm && Interventions

Group	Intervention	Description
Protein intake	Protein intake	Protein intake- Dietary supplement

Primary Outcome Measures

Name	Time	Method
13 Co2 production	8 hours (1 study day)	Urine and breath samples will be collected during the study to measure the rate of oxidation of tracer in the expired breath and flux enrichment in urine.

Trial Locations

Locations (1)

Child and Family Research Institute; 🇨🇦 Vancouver, British Columbia, Canada