Protein Requirements in Children With Phenylketonuria (PKU)

Phenylketonuria Clinical Trial

Official title:

Application of Stable Isotopes to Determine Protein Requirements in Children With Phenylketonuria (PKU)

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT01965691
• Other study ID #: H13-00220
• Status: Completed
• Phase: N/A
• Start date: October 2013
• Est. completion date: December 2015

Study information

• Verified date: September 2020
• Source: University of British Columbia
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 4
• Est. completion date: December 2015
• Est. primary completion date: August 2015
• Accepts healthy volunteers: No
• Gender: All
• Age group: 5 Years to 18 Years

Eligibility

Inclusion Criteria:

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

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.

Related Conditions & MeSH terms

• Phenylketonuria
• Phenylketonurias

Intervention

Dietary Supplement:
• Protein 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 flavored liquid, protein free cookies and corn oil 4 isotopically labeled experimental meals.

Locations

Country	Name	City	State
Canada	Child and Family Research Institute	Vancouver	British Columbia

Sponsors (3)

Lead Sponsor	Collaborator
University of British Columbia	Rare Disease Foundation, Vancouver, Canada, Saudi Arabian Cultural Bureau, Ottawa

Country where clinical trial is conducted

Canada, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	13 Co2 production	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.	8 hours (1 study day)