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Protein Requirements in Children With Phenylketonuria (PKU)

NCT01965691 · Status: COMPLETED · Phase: NA · Type: INTERVENTIONAL · Enrollment: 4

Last updated 2020-09-24

No results posted yet for this study

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

Interventions

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.

Sponsors & Collaborators

  • Rare Disease Foundation, Vancouver, Canada

    collaborator OTHER

  • Saudi Arabian Cultural Bureau, Ottawa

    collaborator UNKNOWN

  • University of British Columbia

    lead OTHER

Principal Investigators

  • Rajavel Elango, PhD · Child and Family Research Institute, University of British Columbia

  • Sylvia Stockler-Ipsiroglu, MD · University of British Columbia

  • Keiko Ueda, MPH, RD · Provincial Health Services Authority

Study Design

Allocation
NA
Purpose
SUPPORTIVE_CARE
Masking
NONE
Model
SINGLE_GROUP

Eligibility

Min Age
5 Years
Max Age
18 Years
Sex
ALL
Healthy Volunteers
No

Timeline & Regulatory

Start
2013-10-31
Primary Completion
2015-08-31
Completion
2015-12-31

Countries

  • Canada

Study Locations

More Related Trials

Entities

Diseases

Read the full study record

This page highlights key information. For complete eligibility criteria, study locations, investigator contacts, and the full protocol, visit the original record on ClinicalTrials.gov.

View NCT01965691 on ClinicalTrials.gov