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High Protein Diet in Patients With Long-chain Fatty Acid Oxidation Disorders

Primary Purpose

Very Long-chain Acyl-CoA Dehydrogenase Deficiency, Trifunctional Protein Deficiency, Carnitine Palmitoyltransferase 2 Deficiency

Status
Completed
Phase
Phase 1
Locations
Study Type
Interventional
Intervention
Diet counseling
Sponsored by
Oregon Health and Science University
About
Eligibility
Locations
Arms
Outcomes
Full info

About this trial

This is an interventional treatment trial for Very Long-chain Acyl-CoA Dehydrogenase Deficiency focused on measuring fatty acid oxidation disorders

Eligibility Criteria

7 Years - 45 Years (Child, Adult)All SexesDoes not accept healthy volunteers

Inclusion Criteria:

  • diagnosis of a long-chain fatty acid oxidation disorder including VLCAD, CPT2, LCHAD or TFP deficiency
  • 7 years old or greater
  • able to comply with diet guidelines

Exclusion Criteria:

  • pregnant
  • enrolled in another study that alters diet composition
  • cannot complete treadmill exercise study

Sites / Locations

    Arms of the Study

    Arm 1

    Arm 2

    Arm Type

    Active Comparator

    Experimental

    Arm Label

    High carbohydrate diet

    High protein diet

    Arm Description

    Diet composition of 10% long-chain fatty acids, 20% medium-chain triglycerides, 12% protein and 68% carbohydrate is the current standard of care in long-chain fatty acid oxidation disorders.

    Diet composition of 10% long-chain fatty acids, 20% medium chain triglycerides, 25% protein and 45% carbohydrate is the comparison diet. Fat content is the same between treatments; only the carbohydrate to protein ratio varies.

    Outcomes

    Primary Outcome Measures

    Energy Expenditure
    does energy expenditure and substrate oxidation differ between subjects randomized to the high carbohydrate versus the high protein diet? We measured resting energy expenditure with indirect calorimetry and estimated substrate oxidation with indirect calorimetry results and urine urea nitrogen excretion. We also measured total energy expenditure with doubly labeled water.

    Secondary Outcome Measures

    Body Composition
    does body composition change more among subjects randomized to the high protein diet compared to the high carbohydrate diet? We measured body composition at baseline and again at the end of the study by DEXA - dual energy x-ray absorptiometry.

    Full Information

    First Posted
    December 14, 2011
    Last Updated
    April 20, 2020
    Sponsor
    Oregon Health and Science University
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    1. Study Identification

    Unique Protocol Identification Number
    NCT01494051
    Brief Title
    High Protein Diet in Patients With Long-chain Fatty Acid Oxidation Disorders
    Official Title
    Fatty Acid Oxidation and Body Weight Regulation in Long-chain Fatty Acid Oxidation Disorders.
    Study Type
    Interventional

    2. Study Status

    Record Verification Date
    April 2020
    Overall Recruitment Status
    Completed
    Study Start Date
    January 2006 (undefined)
    Primary Completion Date
    September 2009 (Actual)
    Study Completion Date
    January 2010 (Actual)

    3. Sponsor/Collaborators

    Responsible Party, by Official Title
    Principal Investigator
    Name of the Sponsor
    Oregon Health and Science University

    4. Oversight

    Data Monitoring Committee
    No

    5. Study Description

    Brief Summary
    The study also determines if eating a diet higher in protein alters body composition, energy balance and metabolic control among patients with a long-chain fatty acid oxidation disorder.
    Detailed Description
    At baseline, the amount of muscle and fat in the whole body and inside the liver and muscle will be measured. All subjects will complete a moderate treadmill exercise test and a test to determine how they use sugar (oral glucose tolerance test). The amount and type of energy burned by each subject will be measured. Subjects will be randomly assigned to follow either a high carbohydrate diet or a reduced carbohydrate, high protein diet for 4 months. At the end of 4 months, the tests will be repeated.

    6. Conditions and Keywords

    Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
    Very Long-chain Acyl-CoA Dehydrogenase Deficiency, Trifunctional Protein Deficiency, Carnitine Palmitoyltransferase 2 Deficiency, Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency
    Keywords
    fatty acid oxidation disorders

    7. Study Design

    Primary Purpose
    Treatment
    Study Phase
    Phase 1, Phase 2
    Interventional Study Model
    Parallel Assignment
    Masking
    None (Open Label)
    Allocation
    Randomized
    Enrollment
    13 (Actual)

    8. Arms, Groups, and Interventions

    Arm Title
    High carbohydrate diet
    Arm Type
    Active Comparator
    Arm Description
    Diet composition of 10% long-chain fatty acids, 20% medium-chain triglycerides, 12% protein and 68% carbohydrate is the current standard of care in long-chain fatty acid oxidation disorders.
    Arm Title
    High protein diet
    Arm Type
    Experimental
    Arm Description
    Diet composition of 10% long-chain fatty acids, 20% medium chain triglycerides, 25% protein and 45% carbohydrate is the comparison diet. Fat content is the same between treatments; only the carbohydrate to protein ratio varies.
    Intervention Type
    Behavioral
    Intervention Name(s)
    Diet counseling
    Other Intervention Name(s)
    low fat diet, high carbohydrate diet, high protein diet
    Intervention Description
    Subjects counseled how to follow either the high carbohydrate diet or the high protein diet for 4 months at home.
    Primary Outcome Measure Information:
    Title
    Energy Expenditure
    Description
    does energy expenditure and substrate oxidation differ between subjects randomized to the high carbohydrate versus the high protein diet? We measured resting energy expenditure with indirect calorimetry and estimated substrate oxidation with indirect calorimetry results and urine urea nitrogen excretion. We also measured total energy expenditure with doubly labeled water.
    Time Frame
    change from baseline after 4 months of treatment
    Secondary Outcome Measure Information:
    Title
    Body Composition
    Description
    does body composition change more among subjects randomized to the high protein diet compared to the high carbohydrate diet? We measured body composition at baseline and again at the end of the study by DEXA - dual energy x-ray absorptiometry.
    Time Frame
    change from baseline to 4 months of treatment

    10. Eligibility

    Sex
    All
    Minimum Age & Unit of Time
    7 Years
    Maximum Age & Unit of Time
    45 Years
    Accepts Healthy Volunteers
    No
    Eligibility Criteria
    Inclusion Criteria: diagnosis of a long-chain fatty acid oxidation disorder including VLCAD, CPT2, LCHAD or TFP deficiency 7 years old or greater able to comply with diet guidelines Exclusion Criteria: pregnant enrolled in another study that alters diet composition cannot complete treadmill exercise study

    12. IPD Sharing Statement

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