Evidence-Based Approach to Dietary Management of Prader-Willi Syndrome (PWS)
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 10
- 试验地点
- 4
- 主要终点
- Suppression of ghrelin
研究概览
简要总结
The overall objective is to explore the mechanisms by which macronutrients regulate food intake and weight gain in Prader Willi Syndrome (PWS).
Previous studies from the investigators' labs suggest that the increased appetite of PWS may be triggered or maintained by an increase in the levels of ghrelin, an appetite-stimulating hormone produced primarily by the stomach. This study will compare the effects of low carbohydrate diet versus low fat diet on levels of ghrelin, appetite suppressing hormones and markers of insulin sensitivity in patients with PWS.
The investigators hypothesize that the low carbohydrate diet will suppress plasma active ghrelin and increase appetite-suppressing hormones to a greater degree and for longer duration than the low fat diet and will thereby reduce hyperphagia and increase satiety. The investigators also hypothesize that the low carb diet will improve hormonal and metabolic markers (fatty acids, amino acids and organic acids) of insulin sensitivity and inflammatory cytokine profiles of children with PWS.
研究设计
- 研究类型
- Interventional
- 分配方式
- Non Randomized
- 干预模型
- Crossover
- 盲法
- None
入排标准
- 年龄范围
- 5 Years 至 17 Years（Child）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •diagnosis of Prader Willi syndrome confirmed by chromosome analysis (ie. interstitial deletion of paternally-derived chromosome 15q, uniparental maternal disomy or other chromosome 15 abnormalities)
- •age 5 years to 17 years
- •written informed consent and assent obtained and willingness to comply with the study schedule and procedures
- •free T4, TSH values in the normal range (either endogenous or with thyroxine replacement)
- •weight stable (BMI percentile fluctuation of <5 percentiles) over the preceding 2 months prior to the study
排除标准
- •presence of other clinically significant disease that would impact body composition including diabetes mellitus, chronic inflammatory bowel disease, chronic severe liver or kidney disease or neurologic disorders
- •concomitant use of medications known to affect body weight or investigational drug in the past year
结局指标
主要结局
Suppression of ghrelin
时间窗: 10 hour fast following completion of dietary intervention
Fasting labs will be obtained immediately following dietary intervention. These labs will include total and active ghrelin.
次要结局
- Changes in satiety(During the 72 hour dietary intervention)
- Improvements in insulin sensitivity(During the 72 hour dietary intervention.)