Murdoch Children's Research Institute, Parkville, VIC 3052, Australia.
The Royal Children's Hospital, Parkville, VIC 3052, Australia.
Nutrients. 2024 Jul 7;16(13):2161. doi: 10.3390/nu16132161.
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.
普拉德-威利综合征（PWS）是一种罕见的疾病，其特征是在整个生命周期中存在不同的营养阶段，从生长不良到暴食症。如果不加控制，能量摄入和消耗之间的不平衡会导致肥胖的发展，并增加发病率和死亡率的风险。尽管评估营养状况所需的能量需求测量方法至关重要，但证据似乎很少且不一致；因此，本综述的目的是检查使用各种方法预测或测量 PWS 个体能量消耗的现有文献。研究中应介绍有关静息能量消耗或基础代谢率的方法和结果。完成了叙述性综合，以介绍研究特点和结果。确定能量需求的方法包括预测方程和间接测热法。年龄、生长激素治疗、禁食状态以及结果呈现的方式的差异限制了对能量消耗趋势的适当总结和识别。间接测热法被认为是最准确的方法；然而，它在并非所有环境中都广泛可用。鼓励开展更多研究，以支持开发有效和可靠的预测方程，从而更好地为支持 PWS 患者的临床实践提供信息并提高其效率。
