NCT01965691

Protein Requirements in Children With Phenylketonuria (PKU)

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

• Status: Completed
• Phase: N/A
• Study type: Interventional
• Enrollment: 4 (actual)
• Sponsor: University of British Columbia · Academic / Other
• Sex: All
• Age: 5 Years – 18 Years
• Healthy volunteers: Not accepted

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.

Conditions

• Phenylketonuria

Interventions

Type	Name	Description
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.

Timeline

• Start date: 2013-10-01
• Primary completion: 2015-08-01
• Completion: 2015-12-01
• First posted: 2013-10-18
• Last updated: 2020-09-24

Locations

1 site across 1 country: Canada