Protein Requirements in Children With Phenylketonuria (PKU)
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.
|Study Design:||Intervention Model: Single Group Assignment
Masking: Single Blind (Subject)
Primary Purpose: Supportive Care
|Official Title:||Application of Stable Isotopes to Determine Protein Requirements in Children With Phenylketonuria (PKU)|
- 13 Co2 production [ Time Frame: 8 hours (1 study day) ] [ Designated as safety issue: No ]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.
|Study Start Date:||October 2013|
|Study Completion Date:||December 2015|
|Primary Completion Date:||August 2015 (Final data collection date for primary outcome measure)|
Experimental: Protein intake
Protein intake- Dietary supplement
Dietary Supplement: Protein intake
Oral consumption of eight hourly experimental meals-
Please refer to this study by its ClinicalTrials.gov identifier: NCT01965691
|Canada, British Columbia|
|Child and Family Research Institute|
|Vancouver, British Columbia, Canada, V5Z4H4|
|Principal Investigator:||Rajavel Elango, PhD||Child and Family Research Institute, University of British Columbia|
|Study Chair:||Sylvia Stockler-Ipsiroglu, MD||University of British Columbia|
|Study Chair:||Keiko Ueda, MPH, RD||Provincial Health Services Authority|