Effect Of The System In The Enriched Fat Metabolism Of The Child Reached Mitochondrial Cytopathy (MITOX)

The aim of the present study is to compare Resting Energy Expenditure (REE) between normal fat diet (NFD) as a control vs high fat diet (HFD) as a treatment in Mitochondrial cytopathies disorders ( MID's ) patients. Secondary objectives is to compare diet induced thermogenesis (DIT) and body composition between NFD vs HFD.

This study is a randomized cross-over study. Normal fat diet (ie 10 % proteins, 45 % lipids, 45 % carbohydrates) is the imposed diet at baseline, high fat diet (ie 10 % proteins, 30 % lipids, 60 % carbohydrates is used according to the cross-over design trial. Main evaluation criteria is REE and second evaluation criteria is DIT both measured by indirect calorimetry ). 36 included MID subjects will be included in this study. Main evaluation criteria and second evaluation criteria will be measured at baseline, 1 month, 2 month and 3 month.

Study Overview

• Status: Completed
• Conditions: Mitochondrial Cytopathies Disorders
• Intervention / Treatment: Other: high fat diet; Other: normal fat diet

Detailed Description

REE and DIT are measured by indirect calorimetry . The device used is a using an open-circuit ventilated hood system (QUARK RMR®, Cosmed, Pavona; Italy). The respiration quo- tient (RQ) and flow settings are calibrated by reference to alcohol combustion every six months. Before each test, the calorimeter is calibrated with a reference gas mixture (5% CO2, 95% O2 v/v). Expired carbon di- oxide (VCO2) and inspired oxygen (VO2) -flows are recorded, as well as the RQ. EE is calculated every minute from oxygen consumption (VO2 in ml/min) and carbon dioxide production (VCO2 in ml/min) using the Weir formula without protein correction. DIT is measured during 6 hours after a calibrated breakfast (normal fat diet or high fat diet according to the cross-over design) as 30% of theoric energy intake.

• Study Type: Interventional
• Enrollment (Actual): 36
• Phase: Not Applicable

Contacts and Locations

Study Locations

• France
  • Lille, France, 59037
    • Centre d'Investigation Clinique
  • Paris, France, 75000
    • Centre d'Investigation Clinique

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 5 years to 21 years (Child, Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• 5 to 21 years-old children,

  • Children with a proven mitochondrial cytopathy documented on biopsies by histology, histoenzymology, ultrastructure and enzyme assay and/or on body fluids by laboratory markers including lactacidemia, metabolic acidosis, increased CSF lactate, lactate/pyruvate and beta-hydroxybutyrate/acetoacetate ratios, paradoxical postprandial ketonemia, alaninemia, citrullinemia, plasma acylcarnitines.
  • Children on the usual diet (30% lipids, 60% glucides and 10% proteins) for at least 1 month.
  • Informed consent of the 2 parents and from the child when in age to express a consent.
  • Child with a social security cover.

Exclusion Criteria:

• Acute infection (fever > 38.5°C for more than 6h) within 7 days prior to the study.
• Disability for understanding and following the protocol
• Rejection of the study by the patient or failure to comply to the protocol

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Treatment
• Allocation: Randomized
• Interventional Model: Crossover Assignment
• Masking: None (Open Label)

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: high fat diet; 10 % proteins, 30 % lipids, 60 % carbohydrates	Other: high fat diet; high fat diet as 10 % proteins, 30 % lipids, 60 % carbohydrates
Placebo Comparator: Normal fat diet; 10 % proteins, 45 % lipids, 45 % carbohydrates	Other: normal fat diet; high fat diet as 10 % proteins, 45 % lipids,45 % carbohydrates

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
resting energy expenditure	resting energy expenditure measured by indirect calorimetry	up to 3 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
diet induced thermogenesis	diet induced thermogenesis measured by indirect calorimetry	baseline, 1 month, 2 months, 3 months
body composition	body composition by DEXA	baseline, 1 month, 2 months, 3 months

Collaborators and Investigators

• Sponsor: University Hospital, Lille
• Collaborators: Ministry of Health, France
• Investigators: Principal Investigator: Dries DOBBELAERE, MD, University Hospital, Lille

Study record dates

Study Major Dates

• Study Start: February 1, 2004
• Primary Completion (Actual): December 1, 2008
• Study Completion (Actual): December 1, 2009

Study Registration Dates

• First Submitted: March 2, 2015
• First Submitted That Met QC Criteria: March 10, 2015
• First Posted (Estimate): March 11, 2015

Study Record Updates

• Last Update Posted (Estimate): March 11, 2015
• Last Update Submitted That Met QC Criteria: March 10, 2015
• Last Verified: March 1, 2015

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Heart Diseases; Cardiovascular Diseases; Metabolic Diseases; Nervous System Diseases; Eye Diseases; Neurologic Manifestations; Retinal Degeneration; Retinal Diseases; Musculoskeletal Diseases; Muscular Diseases; Neuromuscular Diseases; Retinal Dystrophies; Cranial Nerve Diseases; Cardiomyopathies; Ocular Motility Disorders; Mitochondrial Diseases; Paralysis; Ophthalmoplegia; Mitochondrial Myopathies; Retinitis Pigmentosa; Ophthalmoplegia, Chronic Progressive External; Kearns-Sayre Syndrome
• Other Study ID Numbers: 2004/0401; 2004/0138 (Other Identifier: DGS)