Alsaif Maha, Elliot Sarah A, MacKenzie Michelle L, Prado Carla M, Field Catherine J, Haqq Andrea M
Department of Agricultural, Food and Nutritional Science, Faculty of Agriculture, Life and Environmental Sciences, Li Ka Shing Centre for Health Research Innovation, University of Alberta, Edmonton, Alberta, Canada.
Department of Pediatrics, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada.
Adv Nutr. 2017 Nov 15;8(6):905-915. doi: 10.3945/an.117.016253. Print 2017 Nov.
Prader-Willi syndrome (PWS) is a rare genetic disorder associated with excessive weight gain. Hyperphagia associated with PWS may result in higher energy intake, but alterations in energy expenditure may also contribute to energy imbalance. The purpose of this critical literature review is to determine the presence of alterations in energy expenditure in individuals with PWS. Ten studies that measured total energy expenditure (TEE), resting energy expenditure (REE), sleep energy expenditure (SEE), activity energy expenditure (AEE), and diet induced thermogenesis (DIT) were included in this review. The studies provided evidence that absolute TEE, REE, SEE, and AEE are lower in individuals with PWS than in age-, sex-, and body mass index-matched individuals without the syndrome. Alterations in lean body mass and lower physical activity amounts appear to be responsible for the lower energy expenditure in PWS rather than metabolic differences. Regardless of the underlying mechanism for lower TEE, the estimation of energy requirements with the use of equations derived for the general population would result in weight gain in individuals with PWS. The determination of energy requirements for weight management in individuals with PWS requires a more comprehensive understanding of energy metabolism. Future studies should aim to comprehensively profile all specific components of energy expenditure in individuals with PWS with the use of appropriately matched controls and gold standard methods to measure energy metabolism and body composition. One component of energy expenditure that is yet to be explored in detail in PWS is DIT. A reduced DIT (despite differences in fat free mass), secondary to hormonal dysregulation, may be present in PWS individuals, leading to a reduced overall energy expenditure. Further research exploring DIT in PWS needs to be conducted. Dietary energy recommendations for weight management in PWS have not yet been clearly established.
普拉德-威利综合征（PWS）是一种与体重过度增加相关的罕见遗传疾病。PWS相关的食欲亢进可能导致能量摄入增加，但能量消耗的改变也可能导致能量失衡。这篇重要文献综述的目的是确定PWS患者能量消耗是否存在改变。本综述纳入了十项测量总能量消耗（TEE）、静息能量消耗（REE）、睡眠能量消耗（SEE）、活动能量消耗（AEE）和饮食诱导产热（DIT）的研究。这些研究提供的证据表明，PWS患者的绝对TEE、REE、SEE和AEE低于年龄、性别和体重指数匹配的非综合征个体。瘦体重的改变和较低的身体活动量似乎是PWS患者能量消耗较低的原因，而非代谢差异。无论TEE降低的潜在机制如何，使用针对一般人群推导的公式来估计能量需求会导致PWS患者体重增加。确定PWS患者体重管理的能量需求需要对能量代谢有更全面的了解。未来的研究应旨在通过使用适当匹配的对照和测量能量代谢及身体成分的金标准方法，全面描述PWS患者能量消耗的所有特定组成部分。PWS中尚未详细探讨的能量消耗组成部分之一是DIT。PWS患者可能存在由于激素失调导致的DIT降低（尽管去脂体重存在差异），从而导致总体能量消耗减少。需要对PWS中的DIT进行进一步研究。PWS体重管理的饮食能量建议尚未明确确立。
