Effect Of The System In The Enriched Fat Metabolism Of The Child Reached Mitochondrial Cytopathy
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 发起方
- 入组人数
- 36
- 试验地点
- 3
- 主要终点
- resting energy expenditure
研究概览
简要总结
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.
详细描述
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.
研究设计
- 研究类型
- Interventional
- 分配方式
- Randomized
- 干预模型
- Crossover
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 年龄范围
- 5 Years 至 21 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •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.
排除标准
- •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
研究组 & 干预措施
high fat diet
10 % proteins, 30 % lipids, 60 % carbohydrates
干预措施: high fat diet (Other)
Normal fat diet
10 % proteins, 45 % lipids, 45 % carbohydrates
干预措施: normal fat diet (Other)
结局指标
主要结局
resting energy expenditure
时间窗: up to 3 months
resting energy expenditure measured by indirect calorimetry
次要结局
- body composition(baseline, 1 month, 2 months, 3 months)
- diet induced thermogenesis(baseline, 1 month, 2 months, 3 months)