THE ASSESSMENT OF CALORIC NEEDS IN AMYOTROPHIC LATERAL SCLEROSIS

该子项目是利用NIH/NCRR资助的中心赠款提供的资源的许多研究子项目之一。子项目和研究者（PI）可能从另一个NIH来源获得主要资金，因此可以在其他CRISP条目中表示。所列机构为中心机构，不一定为研究者机构。肌萎缩侧索硬化症（ALS）患者在病程中由于热量缺乏而导致的营养不良非常普遍。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早期死亡的突出特征。营养不良需要胃造口术，并对生存产生不利影响。由于ALS患者营养不良的确切原因尚未使用现代技术进行充分研究，因此尚不清楚热量缺乏是否仅是由于摄入不足，或可能是由于热量消耗增加，或这两种机制。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缺乏程度和胃造口术后纠正营养不良的热量替代量的指南都没有出现。该提案的总体目标是检查ALS患者的热量需求，以在疾病早期发现热量缺乏，计划及时的营养干预，并通过客观的营养标准预测胃造口术的需要，而不仅仅是吞咽困难的存在。该提案中的假设是，ALS中的相对热量缺乏在疾病的症状阶段几乎是普遍的，是由于每日热量摄入减少和每日热量消耗增加的组合，并且通过比较患者和匹配的正常对照的数据将是显而易见的。为了检验这一假设，我们提出以下具体目标：1）估计ALS患者的每日热量摄入和支出，并与我们数据库中年龄和性别匹配的健康对照者的值进行比较。（横截面分析），和2）在12个月内定期测量ALS患者的每日热量摄入和支出，以检查疾病严重程度逐渐增加的影响以及身体成分和体力活动水平对热量需求的影响（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).