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Energy Requirements in Mitochondrial Disease

NCT01776918 · Status: COMPLETED · Type: OBSERVATIONAL · Enrollment: 23

Last updated 2017-07-18

No results posted yet for this study

Summary

Metabolic diseases and mitochondrial disorders are caused by genetic mutation which lead to disruptions in energy producing pathways in our body. Enough energy or calories must be given in the diet to ensure normal growth and development. Currently, energy needs for patients with metabolic and mitochondrial diseases are not measured, but is estimated using a mathematical equation based on healthy children. This may lead to under feeding or overfeeding of calories, and has negative nutritional implications.

The clinical standard for measuring energy needs is the use of indirect calorimeter.The indirect calorimeter takes individualized measurements for each patient and therefore will enable dietitians and clinicians to provide sufficient calories in the diet to better manage the disease and promote normal growth and development.

We believe daily energy requirements will vary within metabolic diseases (Phenylketonuria) and mitochondrial disorders (mitochondrial fatty acid oxidation defect, POLG1 mutation etc.).

The objective of this preliminary study is to measure resting energy expenditure in children living with metabolic and mitochondrial conditions and data obtained will be used to generate future hypothesis and will form a basis for future studies.

Conditions

Sponsors & Collaborators

  • Rare Disease Foundation, Vancouver, Canada

    collaborator OTHER

  • University of British Columbia

    lead OTHER

Principal Investigators

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

  • Sylvia Stockler-Ipsiroglu · University of British Columbia/Faculty of Pediatrics

  • Ramona Meni Salvarinova Zivkovic · University of Bristish Columbia/Faculty of Pediatrics

  • Howard Parsons · University of British Columbia/Faculty of Pediatrics

Eligibility

Min Age
1 Year
Max Age
18 Years
Sex
ALL
Healthy Volunteers
No

Timeline & Regulatory

Start
2012-02-29
Primary Completion
2014-06-30
Completion
2014-09-30

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 NCT01776918 on ClinicalTrials.gov