PDF(Pubmed)
Abstract：
Prader-Willi syndrome (PWS) is a rare disorder characterised by varying nutritional phases that occur throughout the lifespan, ranging from failure to thrive to hyperphagia. If uncontrolled, the imbalance between energy intake and expenditure results in obesity development and increased morbidity and mortality risk. Although measures of energy requirements for accurate nutrition assessment are vital, the evidence appears sparse and heterogeneous; hence, the aim of this review was to examine the available literature on energy expenditure predicted or measured using various methods in individuals with PWS. Studies were sought that presented methods and results on resting energy expenditure or basal metabolic rate. A narrative synthesis was completed to present the study characteristics and results. Methods of determining energy requirements included predictive equations and indirect calorimetry. Differences amongst ages, growth hormone therapy, fasting status, and measures in which results were presented were limitations to appropriately summarising and identifying trends in energy expenditure. Indirect calorimetry was identified as the most accurate method; however, it is not widely available in all settings. Further research is encouraged to support the development of valid and reliable predictive equations that will better inform and improve the efficiency of clinical practice in supporting people with PWS.
摘要：
Prader-Willi综合征（PWS）是一种罕见的疾病，其特征是在整个生命周期中发生不同的营养阶段，从失败到茁壮成长到饮食过度。如果不受控制，能量摄入和消耗之间的不平衡导致肥胖的发展以及发病率和死亡率的增加.尽管准确的营养评估所需能量的措施至关重要，证据显得稀疏和异质；因此，这篇综述的目的是研究可用的文献，在PWS患者中使用各种方法预测或测量能量消耗。寻求提供有关静息能量消耗或基础代谢率的方法和结果的研究。完成了叙述性综合，以介绍研究特征和结果。确定能量需求的方法包括预测方程和间接量热法。年龄之间的差异，生长激素治疗,空腹状态,以及提出结果的措施限制了适当总结和确定能源支出趋势。间接量热法被认为是最准确的方法；然而，它并非在所有设置中广泛可用。鼓励进一步的研究，以支持有效和可靠的预测方程的发展，这将更好地告知和提高临床实践的效率，支持PWS的人。
