THE ASSESSMENT OF CALORIC NEEDS IN AMYOTROPHIC LATERAL SCLEROSIS

这个子项目是利用由NIH/NCRR资助的中心拨款提供的资源的许多研究子项目之一。子项目和调查员(PI)可能从另一个NIH来源获得了主要资金，因此可能会出现在其他CRISE条目中。列出的机构是针对中心的，而不一定是针对调查员的机构。在肌萎缩侧索硬化症(ALS)的病程中，由于热量缺乏导致的营养不良是非常普遍的。严重的体重减轻、肌肉无力和肌肉萎缩是导致进行性残疾和过早死亡的显著特征。营养方面的妥协使胃造口术成为必要，并对生存带来不利影响。由于肌萎缩侧索硬化症营养不良的确切原因(S)尚未用现代技术充分研究，尚不清楚卡路里缺乏是单纯由于摄入不足，还是由于卡路里消耗增加，或两者兼而有之。此外，尽管胃造口术经常被推荐给被认为营养不良的ALS患者，但关于需要胃造口术的热量不足的程度或纠正胃造口术后营养不良的热量补充量的指南即将出台。这项建议的总体目标是检查ALS患者的卡路里需求，以确定他们病程早期的卡路里缺乏，计划及时的营养干预，并根据客观的营养标准而不是简单地根据吞咽困难的存在来预测胃造口术的需要。这项建议中的假设是，ALS的相对热量缺乏在疾病的症状阶段几乎是普遍的，这是由于每日摄入减少和每日卡路里消耗增加的共同原因，通过比较患者和匹配的正常对照组的数据将会很明显。为了验证这一假设，我们建议采取以下具体目标：1)估计ALS患者的每日热量摄入和消耗，并与我们数据库中年龄和性别匹配的健康对照组的值进行比较(横断面分析)；2)在12个月内定期测量ALS患者的每日热量摄入和消耗，以检查疾病严重程度的逐渐增加以及身体成分和体力活动水平的变化对热量需求的影响(纵向分析)。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Malnutrition due to caloric deficiency is very prevalent in amyotrophic lateral sclerosis (ALS) during the course of the disease. Profound weight loss, muscle weakness and muscle atrophy are prominent features which produce progressive disability and early death. Nutritional compromise necessitates gastrostomy and carries with it an adverse influence on survival. Since the precise cause(s) of malnutrition in ALS has not been adequately studied using modern technology, it is unknown whether caloric deficiency is due solely to inadequate intake, or could be due to increased expenditure of calories, or both of these mechanisms. Further, although gastrostomy is frequently recommended to ALS patients who are believed to be malnourished, guidelines on neither the degree of caloric deficiency requiring gastrostomy nor the amount of caloric replacement to correct the malnutrition after gastrostomy are forthcoming. The overall goal of this proposal is to examine caloric requirements in patients with ALS to identify caloric deficiency early in the course of their disease, plan for timely nutritional intervention, and predict the need for gastrostomy by objective nutritional criteria rather than simply by the presence of dysphagia. The hypothesis in this proposal is that relative caloric deficiency in ALS is almost universal in the symptomatic stages of the disease, is due to a combination of decreased daily intake and increased daily expenditure of calories, and will be evident by comparing data from patients and matched normal controls. To test this hypothesis we propose to undertake the following Specific Aims: 1) Estimate daily caloric intake and expenditure in ALS patients and compare values with those in age- and gender-matched healthy controls from our database (cross-sectional analysis), and 2) Measure daily caloric intake and expenditure in ALS patients periodically over 12 months to examine the effects of progressively increasing disease severity and changes in body composition and physical activity level on caloric requirements (longitudinal analysis).