ENERGY METABOLISM IN PATIENTS WITH HUNTINGTON'S DISEASE

亨廷顿病患者的能量代谢

• 批准号: 5202088
• 负责人: R PRATLEY
• 金额: --
• 依托单位: NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES
• 依托单位国家: 美国
• 资助国家: 美国
• 起止时间: 至
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/5202088
• 关键词: Huntington's disease; basal metabolism; bioenergetics; body composition; body physical activity; human subject; noninsulin dependent diabetes mellitus; weight loss

Huntington's Disease is an autosomal dominant neurologic disorder characterized by chorea, eye movement abnormalities, and dementia. Significant weight loss is a common finding early in the course of the disease despite reportedly normal or increased caloric intakes. The cause of this apparent increase in energy expenditure is not known. We hypothesize that energy expenditure is increased in patients with Huntigton's Disease as a consequence of the movement disorder. To test this, basal metabolic rate, 24-hour energy expenditure, and non-resting energy expenditure (all measured using the human respiratory chamber on the Research Ward of the Clinical Diabetes and Nutrition Section), and 7-day free-living energy expenditure (measured using the doubly-labeled water technique) will be compared in three groups of patients with Huntington's Disease with varying degrees of chorea (asymptomatic, mild, and moderate) and age, sex, and weight matched controls. To correct energy expenditure for differences in metabolically active (primarily lean) body mass among subjects, all individuals will have body composition measured by anthropometry, bioelectrical impedance analysis, and dual energy X-ray absorptiometry scanning. In addition, since the reported prevalence of non-insulin dependent diabetes is 7 times higher in patients with Huntington's Disease than in the general population, all patients will also undergo an oral glucose tolerance test with measurement of plasma insulin levels. Comparison of asymptomatic patients with Huntington's Disease and controls will address whether an inherent abnormality in energy metabolism is present in Huntington's Disease, whereas comparison of patients with varying degrees of chorea will address the contribution of the movement disorder to the apparent increase in energy expenditure. In addition to providing insight into the pathophysiologic abnormalities in Huntington's Disease, this study may also improve the care of patients with Huntington's Disease by providing more precise estimates of their caloric needs.

亨廷顿病是一种常染色体显性神经系统疾病 其特征是舞蹈症、眼球运动异常和痴呆。 体重明显减轻是早期的常见现象 据报道，尽管热量摄入正常或增加，但仍患有疾病。 这 能量消耗明显增加的原因尚不清楚。 我们 假设患者的能量消耗增加 亨廷顿病是运动障碍的结果。 测试 这个，基础代谢率，24小时能量消耗，以及非休息状态 能量消耗（全部使用人体呼吸室测量 临床糖尿病和营养科研究室），以及 7 天自由生活能量消耗（使用双标签测量 水技术）将在三组患者中进行比较 亨廷顿舞蹈病伴不同程度的舞蹈病（无症状、轻度、 和中等）和年龄、性别和体重匹配的对照。 纠正 代谢活性差异的能量消耗（主要是 瘦）受试者之间的体重，所有个体都会有身体 通过人体测量学、生物电阻抗分析测量的成分， 和双能X射线吸收扫描。 此外，由于 据报道，非胰岛素依赖型糖尿病的患病率高出 7 倍 与普通人群相比，亨廷顿病患者的所有 患者还将接受口服葡萄糖耐量测试 血浆胰岛素水平的测量。 无症状比较 亨廷顿病患者和对照将解决是否 亨廷顿舞蹈病存在固有的能量代谢异常 疾病，而不同程度舞蹈病患者的比较 将解决运动障碍对明显症状的影响 能量消耗增加。 除了提供洞察 亨廷顿病的病理生理学异常，这项研究 还可以通过以下方式改善亨廷顿病患者的护理 更准确地估计他们的热量需求。